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SERIES
PEDIATRIC
BUSTER
with High-Yield Facts
PG
SERIES
PEDIATRIC
BUSTER
with High-Yield Facts
(Based on latest revised edition of OP Ghai: Textbook of Pediatrics, 5th edition
and Nelson: Textbook of Pediatrics, 17th edition)
Compiled by
Dharmendra Sharma
Rinju Sharma
MBBS, MD
MBBS, MD
Consultant Cardiologist
Life Line Heart Centre and Hospital
Kamla Nagar, Bypass Road
Agra
Consultant Gynaecologist
Life Line Heart Centre and Hospital
Kamla Nagar, Bypass Road
Agra
JAYPEE BROTHERS
MEDICAL PUBLISHERS (P) LTD
New Delhi
Published by
Jitendar P Vij
Jaypee Brothers Medical Publishers (P) Ltd
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PG SeriesPediatric Buster
2005, Dharmendra Sharma, Rinju Sharma
All rights reserved. No part of this publication should be reproduced, stored in a retrieval system,
or transmitted in any form or by any means: electronic, mechanical, photocopying, recording,
or otherwise, without the prior written permission of the editors and the publisher.
This book has been published in good faith that the material provided by editors is
original. Every effort is made to ensure accuracy of material, but the publisher, printer
and editors will not be held responsible for any inadvertent error(s). In case of any
dispute, all legal matters to be settled under Delhi jurisdiction only.
81-8061-430-1
To
Our Parents
and
Our Patients
Preface
Its never too late to start. Adding to the PG series a new subject pediatrics, again
conceptualized and arranged chapterwise with references based on latest revised 5th edition
of OP Ghai and 17th edition of Nelson. Little text has been put in proximity only for revision
not for the sake to skip the textbooks. Always remember this is our way to make you revise
the subject and a testimony about how much you have grabbed after going through text.
Questions are all recent ones. So, start at the very earliest as every minute counts and
be systematic in your approach as it is what that matters.
Special thanks to Mr. JP Vij, CMD, Jaypee Brothers Medical Publishers (P) Ltd for his
personal interest. We wish to express our thanks to all our associates and colleagues.
Suggestion and criticism are always welcome and sorry for any typographical error.
Best of luck,
Dharmendra Sharma
Rinju Sharma
Contents
1. Growth and Development .................................................................................................................... 1
2. Disorders of Growth and Development ........................................................................................... 6
3. Adolescent Health and Development ................................................................................................ 9
4. Nutrition and Nutritional Disorders .............................................................................................. 11
5. Newborn Infants ................................................................................................................................... 21
6. Immunity and Infectious Diseases .................................................................................................. 39
7. Gastrointestinal System ..................................................................................................................... 50
8. Hematological Diseases ...................................................................................................................... 55
9. Diseases of Ear, Nose, Throat and Respiratory System ........................................................... 61
10. Cardiovascular System ....................................................................................................................... 66
11. Kidney and Urinary Tract ................................................................................................................. 75
12. Endocrine and Metabolic Disorders ............................................................................................... 81
13. Central Nervous System ..................................................................................................................... 86
14. Malignancies in Childhood ................................................................................................................ 97
15. Collagen Vascular Disorders and Genetics ................................................................................. 103
16. Inborn Errors of Metabolism .......................................................................................................... 105
17. Miscellaneous ...................................................................................................................................... 108
18. Self-assessment ................................................................................................................................... 115
1. Consider the following statements with reference to the normal child development:
(OPG5th/1)(UPSC/04)
(1) The sequence and time of attainment of milestones may vary among individual children, (2) Development
always proceeds in a cephalocaudal sequence,
(3) Presence of primitive reflexes is essential for attaining
voluntary actions, (4) Development depends on the myelination and integrity of central nervous system.
Which of these statements is/are true interpretation of the laws of normal child development?
(a) 1 only
(b) 1 and 3
(c) 2 and 4
(d) 1, 2, 3 and 4
2. A newborn baby has a head circumference of 35 cm at birth. His optimal head circumference will be 43 cm at:
(OPG5th/4) (UPSC/99)
(a) 4 months of age
(b) 6 months of age
(c) 8 months of age
(d) 12 months of age
3. Increase in height in first year is by:
(OPG5th/4)
(DNB 2001)
(a) 40%
(b) 50%
(c) 60%
(d) 75%
4. Birth weight of a child doubles at five months of age while the birth length doubles at the age of:
(a) 1 year
(b) 2 years
(c) 3 years
(d) 4 years
(OPG5th/4) (UPSC 98)
5. Increase is length in the first year of life is:
(OPG5th/4) (AIIMS 81)
(a) 12 cm
(b) 15 cm
(c) 25 cm
(d) 35 cm
1 D
2 D
3 B
4 D
5 C
2 / Pediatric Buster
6. The optimum body weight of a baby by the end of one year is:
(a) Addition of three kg to birth weight
(b) Double the birth weight
(d) Four times the birth weight
7. Height of children 2-10 years increased by:
(a) 2 cm/year
(b) 3 cm/year
(c) 6 cm/year
(d) 10 cm/year
8. The infant triples weight at the age of:
(a) 5 months
(b) 11 months
(c) 18 months
(d) 2 years
(OPG5th/4)(UPSC/03)
(c) Three times the birth weight
(OPG5th/4) (AI 97)
(OPG5th/4) (AIIMS-96)
Weechs formula for estimating weight and height for age of normal children
Weight
Kilograms
Pounds
At birth
3.25
7
3 to 12 months
age in months + 9
Age in months + 11
2
1 to 6 years
(age in years 2) + 8
(age in years 5) + 17
7 to 12 years
(age in years 7) + 5
(age in years 7) - 5
2
Height
Centimetres
Inches
At birth
50
20
At 1 year
75
30
2 to 12 years
(age in years 6) + 77
(age in years 2) + 30
9. Two carpal bones are radiologically seen in the wrist X-ray of most of the children by the end of:
(a) 1 year
(b) 2 years
(c) 3 years
(d) 4 years
(OPG5th/6) (UPSC 96)
An average full-term newborn has the following 5 radiologically demonstrable ossification centers:
Distal end of femur
Proximal end of tibia
Talus
Calcaneus
Cuboid
By the age of 6 months, ossification centers for two carpal bones, i.e. capitate and hamate, appear
that number of centers at wrist is equal to age in years plus one. Thus, a child of 2 years should have
3 centers.
10. First permanent tooth to erupt is:
(a) 1st premolar
(b) 1st molar
7 C
8 B
9 A
10 A
months
2.0
16.0
3.0
12.0
3.0
6.5
6.0
4.0
Cognitive
Stares momentarily at spot where object disappeared
(e.g., yarn ball dropped)
Pretend play with doll (gives doll bottle)
Stares at own hand
Bangs two cubes
Uncovers to (after seeing it hidden)
Egocentric pretend play
(e.g. pretends to drink from cup)
Uses stick to reach toy
17.0
4.0
8.0
8.0
12.0
17.0
7.0
10.0
1.5
6.0
15.0
18.0
19.0
7.0
10.0
12.0
Fine Motor
Palmar grasp gone
Grasps rattle
Reaches for objects
Transfers object hand to hand
Scribbles
Builds tower of two cubes
Builds tower of six cubes
Thumb-finger grasp
Turns pages of book
4.0
3.5
4.0
5.5
13.0
15.0
22.0
8.0
12.0
11 C
12 B
13 C
2.0
4 / Pediatric Buster
14. Pincer grasp develops in an infant at:
(OPG5th/44) (PGI 88)
(a) 8 months
(b) one year
(c) 18 months
(d) 2 years
15. Milestones at 10 months of age are:
(OPG5th/44) (PGI 88)
(a) Pincer grasp
(b) Arranging 3 cubes
(c) Walks few steps without support
(d) Says mama dada
16. In a child, one should be worried if:
(OPG5th/44) (PAL 93)
(a) Stammering occurs at 3 years
(b) Lack of toilet control at 2.5 years
(c) Teeth do not erupt by 11 months
(d) Social smile absent by 10 weeks
17. Child recognizes and smiles at:
(OPG5th/44) (AIIMS 98)
(a) 12 months
(b) 6 months
(c) 4 months
(d) 2 months
18. Which about development is incorrect:
(OPG5th/44) (AIIMS 95)
(a) 2 years old can use pleurals
(b) Social smile at 3 months
(c) Pincer grasp at 3 months
(d) Sitting at 6 months
19. An infant is able to grasp a rattle and transfer it from one hand to the other by the age of: (OPG5th/44) (AI 88)
(a) 3 months
(b) 6 months
(c) One year
(d) 2 years
20. Head control is possible in an infant by...months:
(OPG5th/44) (JIPMER 87)
(a) 1
(b) 2
(c) 3
(d) 6
21. A six-week-old infant cannot:
(OPG5th/44) (AIIMS 92)
(a) Grasp dangling objects
(b) Fix gaze
(c) Lift and hold head
(d) Turn head towards sound
22. A 3-year-old can do all except:
(OPG5th/44) (AIIMS 92)
(a) Walk properly
(b) Eat with a spoon
(c) Vacobulary of 20-100 words
(d) Climb stairs running
23. Consider the following factors :
(OPG5th/44)(UPSC/04)
(1) Head control (2) Social smile (3) Crawls (4) Sits up
The correct sequence in the developmental milestones durin the first year of life is:
(a) 1, 2, 3, 4
(b) 2, 1, 4, 3
(c) 1, 4, 3, 2
(d) 2, 1, 3, 4
24. Absence of following sign will be considered normal is 3 months old child:
(OPG5th/44) (AIIMS 2000)
(a) Babbling
(b) Head raising upto 90
(c) Shift red ring from one hand to other
(d) Smile to familiar
faces
25. Regarding the developmental stage of a newborn, which of the following is/are true: (OPG5th/44) (PGI 2000)
(a) He can fix his eyes on a bright object
(b) Can focus on an object 8-12inches away
(c) Turns to one side
(d) Grasps a rattle
(e) Elevates his head on the neck developmental delay
Motor Development. Developmental delay should be suspected if the child is not able to
(i) Pull up to sit by 4th months;
(ii) Roll over by 5 months;
(iii) Sit without support by 7-8 months;
(iv) Stand holding on by 9-10 months;
(v) Walk by 15 months;
(vi) Climb up or down the stairs by 2 years;
(vii) Jump with both feet by 2 1/2 years;
(viii) Stand momentarily on one foot by 3 years;
(viii) Hop by 4 years and walk in a straight line back and forth or balance on one foot for 5-10 seconds
by 5 years.
26. A 3-year-child is expected to do all except:
(OPG5th/46) (AIIMS 94)
(a) Copy a circle
(b) Speak in sentences
(c) Climb up stairs and down stairs
(d) Hobble five steps
27. A normal infant sits briefly leaning forward on her hands, reaches for and grasps a cube and transfers it from
hand to hand. She babbles but cannot wave bye-bye nor can she grasp objects with the finger and thumb. Her
age is:
(OPG5th/46) (UPSC/2K)
(a) 4 months
(b) 7 months
(c) 10 months
(d) 14 months
28. A child climbs with alternate steps, builds a tower of 8-9 cubes, tellsI but not his name and cannot say his
age and sex. The probabale age is:
(OPG/ 6th/46) (AIIMS /MAY/01)
(a) 24 Months
(b) 36 Months
(c) 30 Months
(d) 48 Months
14 A
15 D
16 D
17 D
18 C
19 B
20 C
21 A
22 D
23 B
24 C
25 A
26 D
27 B
28 A
29 C
30 D
31 C
32 C
33 C
34 A
35 B
36 C
37 D
38 C
39 A
40 D
2 A
3 D
4 B
5 B
6 D
Probable disorder
Delayed adolescence, Hypopituitarism, Constitutional
dwarfism, Nutritional dwarf
Hypothy, Achondroplasia, Ellis-van Creveld syndrome,
Turner syndrome
Hurlers syndrome, Morquios
syndrome, hypogonadism
(b) Hypopituitarism
(c) Hyperpituitarism
Breath holding spells are common b/w 6 mon - 5 yr, but if hypoxia continues for 10-15 sec convulsion
may occur. Kindness and understanding is helpful.
8 D
9 D
10 B
11 B
12 A
13 C
14 B
15 A
16 B
8 / Pediatric Buster
Enuresis (Bed wetting): Diagnosis is made when urine is voided twice a week for at least 3
consecutive months.
Management of enuretic child should begin with behavioral treatment.
17. All of the following are essential features of attention deficit hyperactivity disease (ADHD) except:
(OPG5th/64) (AI/04)
(a) Lack of concentration
(b) Impulsivity
(c) Mental retardation
(d) Hyperactivity
The important clues to dyslexia in school age children include delayed language, trouble rhyming words,
mispronunciations, hesitations and word-finding difficulties.
Attention-Deficit/Hyperactivity Disorder: Characterized by inattention, including increased distractibility and difficulty sustaining attention; poor impulse control and decreased self inhibitory
capacity and motor overactivity and motor restlessness.
ADHD commonly occurs following:
1. Damage to the CNS (prematurity or traumatic brain injury)
2. Toxic exposure (e.g. foctal alcohol syndrome or lead poisoning)
3. Mal development (e.g. Mental retardation syndromes)
4. Abnormalities in the dopamine transporter gene, the D 4 receptor gene and human thyroid
receptor beta gene.
Behaviors must begin before the age of 7 year and must be present for at least 6 months and
impair the childs ability to function normally.
In at least 80% of affected children symptoms of ADHD persist into adolescence and adulthood.
18. One of the following statements regarding childhood autism is false:
(OPG5th/65) (KERALA/94)
(a) It represents a type of childhood neurotic disorder (b) Emergence of speech and social smile is often delayed
(c) Parental attitude and behaviour do not attribute to it
(d) Isolated area of remarkably talent may be
developed
19. Autism refers to:
(OPG5th/65) (PGI 2000)
(a) Neurodevelopmental delay (b) Condition seen following head injury (c) Deafness (d) Impaired reciprocal
social and interpersonal interaction
Autism: Characterized by1. Severe deficiency in social knowledge and reciprocral social interaction
2. Abnormality in language and nonverbal communication.
3. Activities and interests of patients are limited.
4. Patient may develop generalized tonic clonic or psychomotor seizures.
5. 70% patients are retarded.
17 C
18 A
19 A
population
10-19 years
15-24 years
10-24 years
Early
Middle
Late
Age (years)
SMR stages
Changes
9 to 13
1 to 2
(i) Beginning of rapid growth
(ii) Appearance of secondary
sexual characterstics.
14 to 16
3 to 4
(i) Peak height growth
(ii) Menarche/
spermarche
17 to 20
5
Slower growth
1 A
2 B
3 B
4 C
5 A
6 A
7 A
10 / Pediatric Buster
Sequence of puberty:
In girls:
1. Breast development (Thelarche)
2. Public hair (Pubarche)
3. Peak growth velocity
4. Menarche (2 yr after pubic hair start)
In boys: Testis Penis Public hair Axillary hair appearing
Peak stage in growth corresponds to 2nd stage in pubic hair in girls and to 3rd stage in boys.
In first two yr after menarche; menstruation is irregular; cycles may be anovulatory.
8 C
(OPG5th/69)(UPSC/03)
(d) Sexual maturity
Energy requirement
Age
0-2 mon
116 kcal/kg
2-3 mon
109 kcal/kg
3-4 mon
103 kcal/kg
4-5 mon
99 kcal/kg
5-9 mon
95 kcal/kg
9-12 mon
112 (470 kJ/kg) kcal/kg
1-3 yr
1200 kcal/day
4-6 yr
1700 kcal/day
7-9 yr
1900 kcal/day
10-12 yr
2100 kcal/day
13-15 yr
2400 kcal/day
16- 18 yr
2600 kcal/day
2. The normal calorie requirement for a 5-year old child is:
(OPG5th/92) (PGI 93)
(a) 800 calories
(b) 1000 calories
(c) 1500 calories
(d) 2000 calories
3. The average calorie requirement for infants during the first year of life is:
(OPG5th/92) (PGI 81)
(a) 80 to 90 kcal/kg
(b) 100 to 120 kcal/kg
(c) 120 to 130 kcal/kg
(d) Any of the above depending upon
weight
4. The amount of calories required at 1 year of age are:
(OPG5th/92) (AI 96)
(a) 900 kcal/day
(b) 1000 kcal/day
(c) 1200 kcal/day
(d) 1400 kcal/day
5. Protein efficiency ratio is:
(OPG5th/93) (AI 94)
(a) Gain in weight of young animals per unit of protein consumed
(b) Product of digestibility coefficient and
biological value divided by 100 (c) Percentage of nitorgen retained out of the nitrogen absorbed (d) Percentage
of protein absorbed after digestion
Protein quality
A. Protein efficiency ratio (PER)
B. Bilogical value =
1 A
2 C
3 B
4 C
5 A
100
12 / Pediatric Buster
C. True digestibility =
(KARN 94)
COLOSTRUM
The secretion started in mid pregnancy and for the first two days following delivery is called
colostrum.
- It is deep yellow serous fluid with alkaline reaction
- 10-40 ml is daily secretion
- It contains high protein, vitamin A, sodium and chloride and high specific gravity
- The carbohydrate, fat and potassium are less than breast milk
- It contains antibodies (lgA, lgG, lgM) and humoral factors
- It contains fat globules which gives laxative effect to baby
- Assumes the characteristics of mature breast milk by 3-4 weeks
Selective contraindication for breastmilk are galactosemia and phenylketonuria
9. Human colostrum has a relatively higher concentration of all of the following except: (OPG5th/97) (UPSC/99)
(a) Protein
(b) Fat
(c) IgA
(d) Sodium
10. Breastfeeding is contraindicated if the mother is receiving:
(OPG5th/98) (AIIMS 83)
(a) Streptomycin
(b) Insulin
(c) Antithyroid drugs
(d) Broad spectrum antibiotics
11. Which of the following is the reference standard on which classification of protein energy malnutrition is based:
(OPG5th/102) (AIIMS 83)
(a) Gomez classification
(b) 10th percentile of weight for age of ICMR
(c) 80% of the 50th percentile of
weight of ICMR
(d) 80% of the 50th percentile of weight for age of American standard
6 A
7 A
8 A
9 B
10 C
11 C
A. Usual age
B. Essential features
1. Oedema
2. Wasting
13 C
14 B
15 A
Kwashiorkor
0-3 yr
1-3 yr
None
Lower legs,
sometimes face or generalized
Sometimes hidden, sometimes fat
blubbery
Gross loss of
subcutaneous fat
All skin and bone
Obvious
Obvious
Usually apathetic quiet
3. Muscle wasting
4. Growth retardation
5. Mental changes
12 C
Marasmus
16 D
17 D
18 B
Sometimes hidden
Sometimes hidden
Usually irritable moaning, also
apathetic
14 / Pediatric Buster
C. Variable features
1. Appetite
2. Diarrhea
3. Skin changes
Usually good
Often (past or present)
Seldom
4. Hair changes
Seldom
D. Biochemistry/pathology
1. Serum albumin
2. Urinary urea per g
creatinine
3. Urinary hydroxyproline
per g creatinine
4. Serum essential
amino acid index
5. Anemia
6. Liver biopsy
Usually poor
Often (past or present)
Often-diffuse depigmentation
occasional flaky paint or enamel
dermatosis
Often sparse, straight silky,
dyspigmentation, grey or reddish
Low
Low
Low
Low
Low
Low
Uncommon
Normal or atrophic
Common
Fatty changes
19 C
20 A
21 A
22 B
23 A
COMPLICATION OF PEM
S Sugar deficiency i.e., hypoglycemia
H Hypothermia
I Infection and septic shock
El Electrolyte imbalance
DE DEhydration
D Deficiencies of iron, vitamins, and other micronutrients.
27. Moderate to severe malnutrition is indicated by:
(a) Albumin less than 2.1
(b) Body mass index less than 19
(d) Hair changes
(OPG5th/109) (PGI/99)
(c) Serum transferrin less than 160
28. A 10-month-old child weighing 8 kg has Bitots spots in both eyes. Which of the following the most appropriate
schedule to prescribe vitamin A to this child?
(OPG5th/121)(AI/04)
(a) 2 lakh units intramuscular (IM) on day 0,14.
(b) 1 lakh units IM on day 0,14.
(c) 2 lakh units IM on
day 0,1 and 14.
(d) 1 lakh units IM on day 0, 1 and 14.
DOSES OF VITAMIN A
Prevention of vitamin A deficiency: Infants who are not breastfed should receive a 50,000 IU
supplement of vitamin A by 2 months of age (or two doses of 25,000 IU each with 1 month interval
in between) in areas of endemic vitamin A deficiency. Every infant should be administered one dose
of 1 lac units of vitamin A along with measles vaccine at 9 months followed by four more doses of 2
lakh IU each at 18, 24, 30 and 36 months.
24 A
25 B
26 C
27 A
28 D
16 / Pediatric Buster
WHO/UNICEF treatment schedule of xerophthalmia
Children 1 to 6 years and above
Immediately on diagnosis
200,000 IU vitamin A (O)
The following day
200,000 IU vitamin A (O)
Fours weeks later
200,000 IU vitamin A (O)
Children under 1 year and under 8 kg weight at any age
Half the doses as indicated for children 1 to 6 years and above
For night blindness or Bitots Spot
Treat with a daily dose of
10,000 IU vitamin A (O) for 2 weeks
29. All of the following statements are true regarding vitamin A deficiency except:
(NEL17th/180) (KARNAT 96)
(a) Growth retardation is common
(b) Frequent infections can occur
(c) Hydrocephalus is infrequent
(d) Posterior segment of the eye is initially involved
C/F of vitamin A deficiency:
Initially involves posterior segment of eye.
Defective dark adaptation (Night blindness)
XeropthalmiaBitots spot on the temporal side of corneoseleral junction (reversibel)
Keratomalaica
Skin becomes scaly and toad like (hyperkeratosis).
Toad skin is now believed to be due to deficiency of essential fatty acid.
Squamous metaplasia of respiratory mucosa
formation of renal and vesical stones.
Interference with reproduction.
Raised intracranial pressureseparation of cranial bones at sutures may occur (hydrocephalus).
Hypervitaminosis A: Headache; vomiting; raised ICT anorexia; dry skin; painful extremities sparse
hair; hepatosplenomegaly, hypoplastic anemia and benign inracranial hypertension (pseudotumour
cerebri).
30. The most common cause of anemia during childhood is:
(OPG5th/122) (AIIMS 83)
(b) Folic acid deficiency
(c) Pyridoxine deficiency
(d) Iron deficiency
(a) Vitamin B12 deficiency
31. One of the following is not reported to be a clinical manifestation of zinc deficiency state in children:
(a) Dwarfism and hypogonadism
(b) Lever and spleen enlargement
(c) Impaired cell mediated immunity
(d) Macrocytic anemia
(OPG5th/123) (KARN 94)
32. Growth retardation, taste alteration, hepatosplenomegaly, hypochromic microcytic anemia, loss of hair is seen
in defeciency of:
(OPG5th/123) (KARN 95)
(a) Selenium
(b) Copper
(c) Zinc
(d) Iron
Zinc deficiency: Taste alteration and loss of hair; acrodermatitis enteropathica; Night blindness, A
syndrome of growth failure, anaemia and hepatomegaly, and hypogonadism.
33. Perioral rash, pigmentation of skin creases and neuritis is seen in deficiency of:
(OPG5th/123) (AIIMS 89)
(a) Zinc
(b) Copper
(c) Magnesium
(d) Biotin
34. A child with alopecia, psoriatic dermatitis in genitals and mouth and hypogonadism suffering for:
(a) Cu deficiency
(b) Iron deficiency
(c) Zn deficiency
(d) Mg deficiency
(OPG5th/123) (AI 98)
35. Riboflavin deficiency is characterised by:
(OPG5th/125) (PGI 89)
(a) Angular stomatitis
(b) Cheiolosis
(c) Glossitis
(d) All
Riboflavine (B2): Involved in oxidation-reduction mechanism in cellular respiration
C/F of deficiency - Glossitis
- Cheilosis
- Scaly dermatitis
- Circumcorneal vascularization and dermatitis, erythroid hypoplasia.
29 C
30 D
31 D
32 C
33 A
34 C
35 D
36 D
37 A
38 C
39 D
40 A
18 / Pediatric Buster
41. A child who bleeds from gums and has swollen knee probably due to:
(a) Haemophilia
(b) ITP
(c) Scurvy
(d) Trauma
Aminoaciduria
Rickets (Inadequate mineralization and hence excess of unmineralized matrix) The changes are
complicated by inadequate provisional calcification of epiphyseal cartilage deranging enchondral
bone growth.
41 C
42 A
43 C
44 B
45 C
46 D
Type I
(Calcium deficient)
(1)
(2)
(3)
(4)
(5)
Normal or low
Normal
Usually low
Low
Elevated
Elevated
Present
Absent
(a) Deficiency of vit D
(a) Primary
(b) Liver disease
hypophosphatemia
(c) Anticonvulsant therapy
(d) Deficiency of phosphate
Serum calcium
Serum phosphorus
Alkaline phosphatase
Secondary
Cause
Type II
(Phosphate deficient)
47 C
48 D
49 C
50 B
51 C
Type III
(End organ resistance)
Low
Normal
Elevated
20 / Pediatric Buster
Disorders of trace elements
Trace element
Function
Deficiency
Excess
Transferrin
Hemoglobin
Absorption of iron
Oxidative enzyme
activation-tyrosinase,
catalase, uricase,
cytochrome oxidase,
lysyl oxidase, etc
Indian childhood
cirrhosis
Wilsons disease
Hemolytic anemia
(Fanconi-like anemia)
Acute copper poisioningvomiting, hemolytic anemia
and gastric hemorrage
(2) Fluoride
(E)
Constituent of bone
and tooth
Refractory anemia
Osteoporosis
Neutropenia
Delayed bone age
Pseudoparalysis
Hypercholesterolemia
Menkes disease
Defective hair
pigmentation
Hypothermia
Degenerative changes
inelastin of aorta
Mental deterioration
Retarded growth
Dental caries
Osteoporosis
(3) Iodide
(E)
Part of T3 and T4
Endemic cretinism
Simple goiter
Fluorosis
Vomiting, diarrhea,
Abdominal pain
Tetany
Cardiovascular collapse,
Goiter
Tetany
Anemia, abdominal
colic, muscular weakness
low IQ low attention
encephalopathy
Unknown
Keshan
cardiomyopathy
Kaschin Beck disease
Decreased fertility
Cancer of esophagus
Alopecia,
nail deformity
lassitude,
garlic odor to breath
Hyperuricemia
(5) Magnesium
(E)
(6) Selenium
(SE)
(7) Molybdenum
(E)
(8) Mercury
(9) Nickel
(E)-
EEssential
SEsemiessential
Constituent of bone
Enzyme activator
Muscle and nerve
irritability cation
Cofactor of
glutathione peroxidase
Constituent of
xanthine oxidases,
Toxic for many enzymes
and RNA
Stabilization of DNA
and RNA
Urease-matalloprotein
contains nickel as
essential constituent
Constituent of
mitochondrial
superoxide dismutase
Structure of bone
Antioxidant
Not known
Increased
prothrombin
and bleeding time
Minimatta disease
Acrodynia
Dermatitis,
nasal and pulmonary
carcinoma,
liver necrosis
Encephalopathy
pneumoconiosis,
syndrome like
Parkinsons disease
Newborn Infants
In India, the average weight of a normal newborn after 40 wk of gestation is around 2.8 kg.
Low Birth weight baby is of 2500 gm or less irrespective of the gestational age or less than 10th
percentile of the weight.
Very low birth weight baby is weighing 1500 gm or less irrespective of gestational age.
Algorithm regarding the brief outline of steps of action as recommended by NRP for the neonatal resuscitation
22 / Pediatric Buster
Certain vital donts In neonatal resuscitation
Dont administer heavy sedation to the mother
Dont do heavy and continuous suction.
Dont let the neonate develop hypothermia.
Dont carry on with tactile stimulation beyond 2 and never beyond 4 flicks.
Dont delay endotracheal intubation in an apneic neonate.
Dont below your lungs into neonates mouth.
Dont use full palmar grasp for giving bag and mask ventilation.
Dont give respiratory stimulants.
Dont suck the nose first and the mouth later. The breathing effort that follows sucking the nose
first may allow secretions in the mouth to be suddenly aspirated into the lower airway
Dont slap the back
Dont squeeze the rib cage
Dont force thighs on the abdomen
Dont dilate the anal sphincters.
1. The primary aim of neonatal resuscitation:
(OPG5th/137) (UP 94)
(a) To maintain breathing
(b) To restore heart rate
(c) To retain colour
(d) To induce spontaneous cry
2. Neonatal period extends up to:
(OPG5th/137) (JIPMER 81)
(a) 21 days of life
(b) 30 days of life
(c) 28 days of life
(d) 35 days of life
3. You are part of a neonatal resuscitation team attending a delivery. A baby weighing 3 kg is born and has not
cried immediately. What sequence of resuscitation steps will you carry out for this baby?
(OPG5th/139)(UPSC/04)
(a) Dry the baby; position the baby; tactile stimulation; suction of mouth and nose; free flow oxygen if required
(b) Dry the baby; free flow oxygen; position the baby; suction of mouth and nose
(c) Position the baby; suction
of mouth and nose; free flow oxygen; tactile stmulation; dry the baby (d) Dry the baby; position the baby; suction
of mouth and nose; tactile stimulation; free flow oxygen if required.
4. Meconium aspiration is done for 3 times but no breathing occurs next step is:
(OPG5th/140) (AI 97)
(a) Chest compression
(b) O2 inhalation
(c) Bag and mask incubation
(d) Trickling
5. A mother is preparing to breastfeed her infant, it would be of value of her, if she were acquainted with:
(a) Moro reflex
(b) Rooting reflex
(c) Grasp reflex
(d) Cough reflex
(OPG5th/145) (AIIMS 830)
Neonatal reflexes: The neonatal reflexes can be best elicited during quite awake state.
Sucking and rooting reflexstrong and well-cordinated at 32 wk
Grasp reflexusually disappears by 12 wk. Persistence beyond 12 wk is indication of brain
damage.
Moros reflexIt is a vestibular reflex elicited after 28 wk of gestation even in premature baby
but the abduction is weak. The reflex is complete after 32 wk of gestation. Infants with cerebral
damage have exaggerated or absent response. An asymmetric response is seen in Erbs palsy; spastic
hemiplegia and fracture of the humerus or clavicle. Response is poor in Down syndrome and
hypothyroidism. Absent in kernicterus and depressed in opioid withdrawal syndrome. The reflex
disappears after the age of 12 wk so persistence is considered pathological.
Glabellar tap reflex.
Cross extensionappears at 32 wk but at 32 wk, there is extension but no adduction.
Automatic walking32 wk.
Tonic neck reflex(Fencing posture)Prominent during 2-4 mon persistence post 6-9 mon.
Persistence past 6-9 mon indicates poor control of motor function or cerebral pasly.
Landau reflex
Parachute reflex
Babinskis sign and Chvosteks sign may be present normally in term neonate.
1 A
2 C
3 D
4 D
5 B
Newborn Infants / 23
PRIMITIVE REFLEXES
Reflex
Age of appearance
Age of disappearance
Stepping
Placing
Moro
Sucking and
rooting
Palmar grasp
Plantar grasp
Tonic neck
Landau
Neck righting
Parachute
Birth
Birth
Birth
Birth
6 weeks
6 weeks
3 months
4 months while awake.
7 months while asleep
6 months
10 months
4 to 6 months
24 months
24 months
Persists throughout life
Birth
Birth
2 months
3 months
4 months
9 months
(OPG5th/145)(UPSC/03)
(d) Parachute reflex
7 A
8 B
9 D
10 A
11 D
12 D
13 A
24 / Pediatric Buster
14. Persistent Moros reflex at 12 weeks indicates:
(a) Normal child
(b) Brain damage
(c) Hungry
15. Moros reflex disappears by:
(a) 1 month
(b) 3 months
(c) 5 months
(d)
16. Which is not a normal finding in the newborn:
(a) Systolic murmur
(b) Pulse rate of 100/min
17. A hymenal tag in a newborn is best treated by:
(a) Steroids
(b) Surgery
(c) Leaving it alone
18. The following are normal features in a neonate:
(a) A vaginal bleeding
(b) Breast engorgement
19. Breast milk at room temperature stored for:
(a) 4 hr
(b) 8 hr
(c) 12 hr
(d) 24 hr
7 months
(OPG5th/147) (JIPMER 93)
(c) Peripheral cyanosis
(d) Central cyanosis
(OPG5th/147) (AIIMS 80 PGI 81)
(d) None
(OPG5th/147) (PGI 89)
(c) Palpable liver and kidney
(d) Phimosis
(e) All
(OPG5th/151) (PGI/98)
Expressed break milk can be stored at room temperature for 10 hours; in a refrigerator for 24 hours
and in a freezer at 20C for 3 month
The first 2 weeks of the neonatal period are crucial for establishing breastfeeding, lactogenic
hormones are ineffective in stimulating human breast secretion.
Birth asphyxia
Hypothermia
Feeding difficulties
Infections
Respiratory distress due to
hyaline membrane disease
Apneic spells
Intraventricular hemorrhage
Necrotising enterocolitis
Metabolic acidosis
Hyperbilirubinemia
Birth asphyxia
Meconium aspiration
syndrome
Hypothermia
Hypothermia
Infections
Polycythemia
14 B
15 B
16 D
17 C
18 E
19 B
20 D
21 A
22 A
Newborn Infants / 25
23. All of following are true about neonatal sepsis except:
(OPG5th/161) (AI 96)
(a) Preterm babies are predisposed to sepsis
(b) Late initiation of breastfeeding is a predisposition
(c) Premature rupture of membranes predisposes to sepsis
(d) None
24. Laboratory findings in neonatal septicaemia are A/E:
(OPG5th/161) (AI 96)
(a) C-reactive protein
(b) Leucocytosis
(c) ESR increased
(d) Toxic granulated multilobulated nuclei
25. Most common cause of neonatal septicaemia:
(OPG5th/161) (AI 98)
(a) Local nusery environment
(b) Infection through umblical cord
(c) Exclusively breastfed baby
(d) Infection by GIT bacteria
Septicemia in the neonatal period:
Preterm and low birth weight infants are susceptible to infection via the umblical cord.
Predisposing factors.
- Poor hygiene of nursery personnel
- Premature rupture of membrane for 24 hr or more before labor
- Maternal infection fornight prior to delivery
- Repeated vaginal examination during labor.
Host risk factors- Male sex; galactosemia (E.coli); I/M iron, congenital anomalies, immune defects,
second twin and omphalitis.
C/F:
Baby becomes lethargic and unresponsive if sucking normally.
Apneic episodes may be only manifestation in preterm babies.
There may be hepatosplenomegaly; jaundice and abdominal distension.
Marked pallor; ashen grey color; cold extremities and absent pulse with fall in BP indicates shock
There is neutropenia; immature forms of neurophils exceed 20% of the total neutrophils; C reactive
protein is elevated and ESR and haptoglobin also raised.
Limulus amebocyte lysate detects endotoxins of gram-negative bacteria.
Bactericial drugs cefotaxime and ceftriaxone are the antibiotics of choice if organism is not known.
Therapy continues for 3-7 days beyond apparent cure for ordinary case; 3 wk of meningitis and
osteomyelitis and 6 wk for UTI.
26. Neonatal septicaemia is most commonly caused by:
(OPG5th/161) (AIIMS 92)
(a) E. coli
(b) Grp. B Streptococcus
(c) Staph. aureus
(d) Streptococcus viridans
27. Neonatal sepsis and meningitis most common cause:
(OPG5th/161) (PGI/98)
(a) Streptococcus pyogenes
(b) Streptococcus algaetaciae
(c) Enterococcus fecalis
(d) Staphylococcus aureus
DEFINITION OF SIRS AND SEPSIS
SIRS: The systemic inflammatory response to a variety of clinical insults, manifested by 2 or more of
the following conditions:
Temperature instability < 35 C or > 38.5C
Respiratory dysfunction
Tachypnea > 2 SD above the mean for age
Hypoxemia (PaO2 < 70 mm Hg on room air)
Cardiac dysfunction
Tachycardia > 2 SD above the mean for age
Delayed capillary refill > 3 sec
Hypotension > 2 SD below the mean for age
Perfusion abnormalities
Oliguria (urine output < 0.5 ml/kg/hr)
Lactic acidosis (elevated plasma lactate and/or arterial pH < 7.25)
Altered mental status
Sepsis: The systemic inflammatory response to an infectionus process
23 B
24 B
25 A
26 A
27 B
26 / Pediatric Buster
28. In neonatal necrotising enterocolitis all of the following are found except:
(OPG5th/164) (AI 96)
(a) Bradycardia
(b) Blood in stools
(c) Abdominal distention
(d) Bowel sounds increased
Neonatal necrotizing enterocolitis:
More prone to occur in premature infants.
There occurs apnea; bradycardia and cyanosis, hypotension, polycythemia and leucocytosis.
Bowel shows edema aned vascular engorgment. Gas collects in the submucosa and the intestinal
wall becomes necrotic.
Ileum is the most common site and clostridia is suspected to be the cause.
Illness develops in 1 or 2 days with mild abdominal distension; blood in stools, bowel sounds are
diminished, mild acidosis, and mild thrombocytopenia
DIC may occur in last stage and pneumoperitoneum is present on X-ray.
Parenteral alimentation is done with amino acid mixture and 25% glucose.
29. Which of the following infections has the most neurological complications:
(OPG5th/165t) (UPSC 86)
(a) Rubella
(b) Measles
(c) Smallpox
(d) Scarlet fever
30. Rubella maximally infects the foetus if the mother contracts the disease during....of pregnancy:
(a) 5-6 weeks
(b) 9-12 weeks
(c) 12-13 weeks
(d) 20 weeks
(OPG5th/165t) (AIIMS 87)
Congenital Rubella:
Rubella is also called 3 days measles
Risk of fetal infection is 50% or high if mother acquires infection during the first four weeks of
gestation.
But the greatest risk to foetus is when infection develops 3-6 wk post-conception
Most florid fetal illness is manifested in first 8-10 wk of gestation.
Foetus infected after 20 weeks may have isolated deafness as only symptom
31. The following are seen in congenital rubella except:
(OPG5th/165) (PGI 89)
(a) Cataract
(b) Pulmonary stenosis
(c) Salt and pepper retinitis
(d) Bullae and vesicles on skin
32. Heart disease common with rubella infection:
(OPG5th/165) (AI 96)
(a) PDA
(b) VSD
(c) ASD
(d) Essenmenger syndrome
33. Purpura is seen in:
(OPG5th/165) (AI 89)
(a) Miliary tuberculosis
(b) Niemann-Picks diesease
(c) Congenital rubella
(d) CMV infection
34. Rubella syndrome is characterised by A/E:
(OPG5th/165) (AIIMS/99)
(a) Microcephaly with growth retardation (b) Deafness (c) Blindness (cataract) (d) Renal tract anomalies
35. Defect most commonly occurring in congenital rubella is:
(OPG5th/165) (AIIMS/85)
(a) Cataract
(b) Deafness
(c) Microcephaly
(d) Blindness
Defects in congenital Rubella:
M.C. defect is cataract
Unilateral or bilateral sensorineural deafness
CVS malformations such as PDA, pulmonary artery branch stenosis, VSD
Interstitial pneumonia
Increased bone densities of metaphysis of long bones without periosteal reaction
Salt and pepper retinitis; nuclear cataract, glaucoma and microphthalmia
Polycystic kidneys
Esophageal; jejunal atresia and hepatitis
Microcephaly and mental retardation
IUGR.
(OPG5th/165) (AIIMS 91)
29 A
30 A
31 D
32 A
33 C
34 D
35 B
36 A
Newborn Infants / 27
37. Rubella infection in child is best diagnosed by:
(OPG5th/165) (AIIMS 95)
(a) IgM Ab in fetal blood
(b) IgA Ab in fetal blood
(c) T4 cell count in fetal blood
(d) Fetal haemoglobin
38. Cataract in newborn is seen in:
(OPG5th/165) (UPSC/2001)
(a) Congenital rubella
(b) Human papilloma virus infection
(c) Herpes simplex virus infection
(d) Toxoplasmosis
Congenital Rubella
Late complication
Diabetes
Thyroid disorders
Rubella encephalitis
Psychomotor defects
Dental abnormalities (Enamel hypoplasia)
Active Infection at birth
Hemolytic anemia
Thrombocytopenic purpura
Hepatitis
Encephalitis
Petechial blue bery muffin lesion
Or maculopapular skin lesion
Myocarditis
Pneumonitis
CSF protein raised without pleocytosis
Baby may shed virus upto 18 months
Prevention can be done by immunization of all boys and girls before puberty
39. An 8-year-old female child following URTI developed maculopapular rash on the face spreading onto the trunk
which cleared on the 3rd day without desquamation and tender post auricular and suboccpital lymphadenopathy.
The diagnosis is:
(OPG/ 5th/165) (AIIMS /MAY/01)
(a) Measles
(b) Rubella
(c) Erythema infectiosum
(d) Kawasaki disease (Mucocutaneous LN Syndrome)
40. A child born with microcephaly, chorioretinitis and intracranial calcification. Most likely diagnosis is:
(OPG5th/165t) (JIPMER 90)
(a) Congenital syphilis
(b) Rubella
(c) Toxoplasmosis
(d) Trypanosomiasis
Toxoplasmosis: Infection is transmitted from mother to foetus duing pregnancy in only 30% cases.
Women seropositive before pregnancy usually are protected against acute infection and do not give
birth to congenitally infected babies.
Chances of infection transmitted transplacental is less when acquired 6 months before conception
and increases as interval b/w infection and conception decreases.
Incidence of transplacental infection is lowest during first trimester (15%) but the disease in the
neonate is most severe. While incidence during third trimester is greatest (65%) but the infant is
usually asymptomatic at birth.
Infected infants who are asymptomatic at birth; develop learning disabilites and chronic neurologic
sequelae later in life.
C/F:
Cerebro-occular lesions are the most common; fever; refusal to feed; maculopapular rash;
hepatosplenomgaly and jaundice; meningoencephalitis chorioretinitis, convulsions; hydrocephalus,
microcephay and cerebral calcification.
Thrombocytopenia and IUGR
CSF shows xanthochromia
Tt: Pyrimethamine and sulfadiazine to child Spiramycin has been used for treating maternal
infection to prevent fetal Toxoplasma infection.
37 A
38 A
39 B
40 C
28 / Pediatric Buster
41. Which is not a manifestation of congenital toxoplasmosis:
(OPG5th/165t) (AIIMS 95)
(a) Hydrocephalus
(b) Chorioretinitis
(c) Cerebellar atrophy
(d) Intracranial calcification
42. Most common lesion in fetal toxoplasmosis:
(OPG5th/165t) (AIIMS 95)
(a) Chorioretinitis
(b) Pulmonary HT
(c) Encephalitis
(d) GIT involvement
43. STD which causes foetal abnormality is:
(OPG5th/165t) (AI 94)
(a) Herpes simplex
(b) Hepatitis
(c) Group B Streptococcus
(d) Toxoplasmosis
Herpes Simplex:
Neonatal HSV is almost always acquired during vaginal delivery or by ascending infection by early
rupture of membranes.
Lesion appears on 4-10th day after birth vesicular skin lesions may appear as inital sign.
Infant may have fever, hypothermia; dyspnoea and myocarditis, liver is enlarged; convulsions are
also seen.
CSF shows moderate pleocytosis and disproportionate elevation of proteins.
Breastfeeding is safe.
Vidarabine and acyclovir may help.
Cesarean section is indicated in genital herpes.
44. 6 weeks child presents with B/L cataract with seizure the probable diagnosis is:
(OPG5th/1 65) (AIIMS 96)
(a) Congenital toxoplasmosis
(b) Galactosaemia
(c) Fructosaemia
(d) Gauchers disease
Congenital Syphilis:
Rarely the infection may be contracted
The treponemes do not invade the placental tissue untill the fifth month of gestation.
Early manifestation usually appear within first year of life.
M.C. is a bullous rash; rhinitis snuffles
Hepatospleenomegaly; genealized lymphadenopathy
Coombs negative hemolytic anemia; bleeding diathesis thrombocytopenia; nephrosis or nephritis;
osteochondritis and syphilitic metaphysitis (within one month) are also seen.
Patient may have acute syphilitic leptomeningitis progressive hydrocephalus and cranial nerve palsies.
Patient may have congenital glaucoma and chorioretinitis.
Late manifestations (after 2 yr) are triad of Hutchinsons teeth, interstitial keratitis and nerve
deafness. There may be rhagades; saber-shaped tibia and mulberry molars.
Cluttons joints; painless synovitis of the knee joint is most common lesion around the age of
puberty.
There may be pseudoparalysis of Parrot; olympian brow and Higounenakis sign, Congenital syphilis
is strongly suscepted when VDRL and FTA-ABS lg M are positive in the infant.
Specific Treponema pallidum lgM antibodies and IGM antibodies against 47 kD antigen are usuful
for diagnosis of congenital syphilis
Treatment: if CSF
abnormalpenicillin G
normalprocain Penicillin
Interstitial keratitis is treated with corticosteroid
Nerve deafness is treated with oral steroid and penicillin
Erythromycin is indicated if VDRL test remains positive.
45. In early congenital syphilis which one is not seen:
(OPG5th/165) (UP 96)
(a) Keratitis
(b) Vesicular rash
(c) Chorioretinitis
(d) Rhinitis
46. A neonate suspected to have congenital syphilis, diagnostic test to be done is:
(OPG5th/165) (AI/2001)
(a) FTA ABS IgM
(b) FTA-ABS IgG
(c) TPI
(d) VDRL
47. The clinical sign of hyaline membrane disease generally first appears:
(OPG5th/166) (AMU 88 PGI 81)
(a) In the first 6 hours of life
(b) Between 6 and 12 hours of life
(c) Between 36 and 48 hours of life
(d) After 48 hours of life
41 C
42 C
43 D
44 A
45 A
46 A
47 B
Newborn Infants / 29
48. Hyaline membrane in the lung is seen in:
(OPG5th/166) (PGI 87)
(a) Respiratory distress syndrome
(b) Pulmonary oedema
(c) Pneumococcal pneumonia
(d) Acute viral hepatitis
49. Respiratory distress syndrome in infants occur in:
(OPG5th/166) (PGI 89)
(a) Babies of diabetic mother
(b) Premature rupture of membrane
(c) Birth weight more than 2500 gm
(d) Even in term baby
50. Surfactant production in lungs starts at:
(OPG5th/166) (AI/2001)
(a) 28 weeks
(b) 32 weeks
(c) 34 weeks
(d) 36 weeks
HMD (Hyaline membrane disease or idiopathic respiratory distress syndrome):
Occurs commonly in preterm neonates; babies of diabetic mother and infants delivered by cesarean
section or breech.
Ischemia of the alveolar cells results in effusion of albuminous fluid.
Clinical manifestations usually appear 4-6 hr after birh.
2 or more of the following features if found at examination 1 hour apart are enough to diagnose RDSRespiratory rate > 60 enough xiphoid retraction.
Expiratory grunt
Cyanosis
Biochemical changes show metabolic and respiratory acidosis; pCO2 may rise to even 80 mmHg;
Increased excretion of sodium may cause hyponatrenia.
X-ray show ground glass motting.
1/3 babies die within first 3-days
Glucocorticoid administration to the mother two days before delivery in 32 wk of gestation reduces
the risk.
Lacithin: sphingomyelin ratio less than one is always associated with severe RDS.
Maturation of surfactant can be detected by shake test or bubble stability test.
51. Respiratory distress syndrome in the newborn is commonly associated with all except: (OPG5th/166) (UPSC/01)
(a) Diabetic pregnancy (b) Prematurity (c) Following an elective caesarean section (d) Intrauterine growth
retardation
52. Which of the following is false about hyaline membrane disease:
(OPG5th/166) (ALL INDIA/02)
(a) It occurs when delivery occurs before 34 weeks (b) Diabetes mellitus is a predisposing factor (c) Cyanosis
(d) 100% oxygen is the treatment
SCORING SYSTEM FOR RESPIRATORY DISTRESS SYNDROME
Score
0
1
Respiratory rate
<60
60 to 80
Cyanosis
None in
in 40% oxygen
room air
Retraction
None
Mild
Grunting
None
Audible with
stetho
Air entry (crying)
Clear
Decreased
Note: Score
0 to 4
5 to 7
Over 7
Action needed
less than 40$ oxygen
CPAP
assisted ventilation
48 A
49 A
2
80 or apnea
in more than 40%
oxygen
Moderate or severe
Audible without
stetho
Barely audible
50 A
51 D
52 D
53 B
(c) Acidosis
30 / Pediatric Buster
54. Foetal lung maturity is assessed by:
(OPG5th/167) (KERALA 94)
(a) L/S ratio
(b) Bilirubin content of amniotic fluid
(c) Ultrasound
(d) Amniocentesis
55. A child delivered at 31st week of gestation has ground glass appearance of lungs and got a cyanotic attack on
4th day, the features point towards:
(OPG5th/167) (AIIMS/99)
(a) ARDS
(b) Meconium aspiration
(c) Hyaline membrane disease
(d) None of the above
56. Which one of the following is true of hyaline membrane disease of the newborn?
(OPG5th/167) (PGI/98)
(a) Prematurity provides relative protection to the occurrence
(b) Maternal steroid exposure increases severity
of the disease
(c) Phosphatidyl glycerol estimation is a reliable method of diagnosis
(d) Surfactant increases
the surface tension of alveoli
57. Most common cause of respiratory distress after birth in first 24 hours is:
(OPG5th/168) (JIPMER 95)
(a) Neonatal sepsis
(b) Meconium aspiration
(c) Bacterial pneumonia
(d) Air embolism
MECONIUM ASPIRATION
About 13% of all deliveries have meconium staining of amniotic fluid (MSAF). Around 6% of such
neonates aspirate meconium into the lungs in utero.
Complications
Airleak syndromes: Pneumothorax, interstitial emphysema, pneumomediastinum, pneumopericardium, pneumoperitoneum, subcutaneous emphysema.
Persistent pulmonary hypertension of the newborn (vide infra)
Pulmonary/cerebral hemorrhage.
Superadded bacterial sepsis.
Subglottic stenosis (due to endotracheal intubation)
Chest radiograph shows overinflated lungs, flat diaphragm, retrosternal lucency, bilateral
pneumonia and signs of airleak syndromes. Cardiomegaly may also be present.
58. In a child cessetion of breathing for 20 sec with bradycardia is:
(a) Apnoea
(b) Dyspnoea
(c) Cheyne-Stokes respiration
(d) None
54 A
55 C
56 C
57 B
58 A
59 C
60 C
61 B
Newborn Infants / 31
62 D
63 B
64 A
65 B
66 B
67 A
68 D
32 / Pediatric Buster
69. Physiological icterus usually appears on the ....and regress by the...:
(OPG5th/170) (AIIMS 83)
(a) First day and third day
(b) Third day and seventh day
(c) Third day and ninth day
(d) Ninth day and eleventh day
Physiological Jaundice of the newborn
Usually appears after 30 hours
Peak level (12 mg/dl) is reached on 4th or 5th day and disappears by 7-14 days; rate of increase is
1 mg/dl/day.
In premature babies; maximum bilirubin level reached 12 to 15 mg/dl on 5th-7th days and
disappears by 14 days to a month.
Physiological jaundice is exaggerated in prematurity
- Hypoxia and circulatory insufficiency
- DrugsNovabiocin, salicylate, Vit K
- Cephalohaematoma (1 gm Hb = 34 mg of billirubin on hemolysis)
- Role of infections
Breast milk jaundice (develops gradually on 2nd or 3rd wk) when conjugation of bilirubin is
interfered by pregnanediol) temporary interruption of nurshing for 3-4 days help.
70. Which one of the following is not associated with jaundice in infancy?
(a) Hereditary fructose intolerance
(b) Maple syrup urine disease
syndrome
(OPG5th/170) (UPSC/04)
(c) Galactosemia
(d) Crigler-Najjar
69 B
70 B
Newborn Infants / 33
Cystic fibrosis
Galactosemia
Alpha1-antitrypsin deficiency
Tyrosinemia
71. Which of the following factor is responsible for sporadic cases of jaundice in a breastfed infant?
(OPG5th/171) (AIIMS 81, DNB 90)
(a) Excessive iron
(b) Pregnanediole
(c) Vitamin K1 deficiency
(d) None of the above
72. Commonest cause of pathological hyperbilirubinaemia in India is:
(OPG5th/171) (PGI 89)
(a) ABO incompatibility
(b) Rh incompatibility
(c) Septicaemia
(d) G-6-PD deficiency
73. Unconjugated hyperbilirubinaemia in newborn is caused by all except:
(OPG5th/171) (AIIMS 92)
(a) Galactosaemia
(b) Gilberts syndrome
(c) Spherocytosis
(d) Breast milk jaundice
Pathological jaundiace:
Appears within 24 hr of birth.
Increase in bilirubin level at the rate of 5 mg/100ml per 24 hrs.
Absolute bilirubin level more than 15 mg/100 ml.
Jaundice persists more than one week in mature and two week in premature neonates.
74. All of the following factors may contribute to the development of indirect hyperbilirubinaemia in neonates except:
(a) Drugs
(b) Cephalhaematoma
(c) Hypothyroidism
(d) Choledochal cyst (OPG5th/171) (KARN 94)
75. Administration of large doses of vitamin K to a newborn may result in:
(OPG5th/171) (AI 88)
(a) Hyperbilirubinaemia (b) Bulging of the fontanelle (c) Hypoprothrombinaemia (d) Renal vein thrombosis
76. Neonatal jaundice persisting 2 weeks after birth is not seen in:
(OPG5th/171) (AI 92)
(a) Galactosaemia
(b) Rh incompatibility
(c) Hypothyroidism
(d) Breast milk jaundice
CAUSES OF JAUNDICE BASED ON TIME OF ONSET
First day
Rh and ABO incompatibilities (hemolytic disease of the newborn)
Intrauterine infections like toxoplasmosis and cytomegalic inclusion disease
G-6-PD deficiency
Hereditary spherocytosis
Drug administration to mother (vitamin K, sulfisoxazole, salicylates)
Homozygous alpha-thalassemia
Second and Third Day
Physiologic
Hyperbilirubinemia of newborn
Birth asphyxia
Cephalhematoma
Acidosis
Hypothermia
Hypoglycemia
Drugs
Familial nonhemolytic icterus as in Crigler-Najjar disease, Gilberts disease, Dubin-Johnson syndrome.
Fourth to Seventh Day
Septicemia
Syphilis
Toxoplasmosis
Cytomegalic inclusion disease
Extrahepatic atresia of bile duct
Breast milk jaundice
71 B
72 B
73 A
74 D
75 A
76 B
34 / Pediatric Buster
After First Week
Septicemia
Extrahepatic atresia of bile duct
Hereditary spherocytosis
Neonatal hepatitis
Drug-induced hemolytic anemia
Galactosemia
Persistent Jaundice during First Month
Inspissated bile syndrome
Cretinism
Congenital hypertrophic pyloric stenosis
77. Cirrhosis occurs in A/E
(NEL17th/1129) (UP 96)
(a) Galactosaemia
(b) Wilsons disease
(c) -1 antitrypsin deficiency
(d) Cystinuria
78. Long-standing unconjugated jaundice of newborn is caused by following except:
(NEL17th/1131) (AIIMS 90)
(a) Crigler-Najjar syndrome
(b) Breast milk jaundice
(c) Cretinism
(d) Septicemia
79. Neonatal cholestatis is associated with:
(NEL17th/1314) (AIIMS 98)
(a) Glycogen-storage disease
(b) MPS-1H (Hurlers) syndrome
(c) Alpha-hydroxylase deficiency
(d) Niemann-Picks disease
80. A 2-month-old baby presented with jaundice, there is no urobilinogen in urine, but dark urine stools are pale,
the diagnosis goes in favour of:
(NEL 17th/1317)(AIIMS/99)
(a) Hereditary spherocytois (b) Breast milk jaundice (c) Hypothyroidism (d) Congenital biliary duct atresia
81. Gilberts syndrome are true A/E:
(NEL 17th/1320) (UP 97)
(a) Mild conjugated bilirubinaemia
(b) Normal LFT
(c) Normal liver biopsy
(d) Unconjugated
bilirubinaemia
82. Risk of kernicterus is increased in all except:
(OPG5th/172) (AIIMS 84)
(a) Low level of serum albumin
(b) Prematurity
(c) Acidosis
(d) High level of serum albumin
Kernicterus:
Unconjugated hyperbilirubinemia usually > 20 mg% with necrosis of neurons in basal ganglia,
hippocampus and subthalamic nuclei.
There is lethargy and setting sun sign. Convlusion and rigidity usually develop between 3rd and
7th day.
Spastic or athetoid type of cerebral palsy, brownish staining of teeth; deafness, intellectual
retardation and learning disabilities.
Bilirubin reversibly inhibits phosphorylation of synapsin.
The capacity of serum albumin to bind billirubin is low in newborns.
There is ketoacidosis
Risk increases with acidotic; hypothermic hypoglycemic and preterm babies.
CLINICAL FEATURES OF KERNICTERUS
Acute form
Phase 1 (1st 1-2 days): poor sucking, stupor, hypotonia, seizures
Phase 2 (middle of 1st wk): hypertonia of extensor muscles, opisthotonos, retrocollis, fever
Phase 3 (after the 1st wk): hypertonia
Chronic form
First year: hypotonia, active deep tendon reflexes, obligatory tonic neck reflexes, delayed motor skills
After 1st yr: movement disorders
(choreoathetosis, ballismus, tremor), upward gaze, sensorineural
hearing loss.
83. In kernicterus which is increased:
(OPG5th/172) (TN 89)
(a) Conjugated bilirubin
(b) Unconjugated bilirubin
(c) Both
(d) None
84. Which of the following antimicrobial agents increase the risk of kernicterus in newborn?
(a) Gentamicin
(b) Novobiocin
(c) Cloxacillin
(d) None
(OPG5th/172) (PGI 78, 82)
77 B
78 D
79 A
80 D
81 A
82 D
83 B
84 B
Newborn Infants / 35
85. In kernicterus staining of brain is more intense in the:
(OPG5th/172) (PGI 79, AIIMS 81)
(a) Spinal cord
(b) Corpus callosum
(c) Basal ganglion
(d) Cerebral cortex
86. Chances of developing kernicterus appears to be significant when serum level of unconjugated bilirubin reaches:
(a) 50 mg%
(b) 20% mg
(c) 10 mg%
(d) 5 mg%
(OPG5th/172) (PGI 80, AIIMS 86)
87. In a newborn brain damage may be associated with serum bilirubin protein ratio of:
(a) More than 1
(b) More than 2
(c) More than 3 (d) More than 3.5 (OPG5th/172) (AIIMS 81, AMU 89)
88. In Rh isoimmunisation, exchange transfusion is indicated if:
(OPG5th/173) (AIIMS 89)
(a) Cord blood haemoglobin is less than 10 mg/dl
(b) Cord bilirubin is more than 10 mg/dl
(c) History of previous sibling affected
(d) Hydrops fetalis
89. In infants receiving phototherapy all are seen except:
(OPG5th/173) (AIIMS 80, PGI 83)
(a) Have increased insensible water loss
(b) Generally develop of skin bronzing
(c) Should have their eyes
patched during therapy
(d) Often became constipated
Phototherapy
C/l in conjugated hyperbilirubin
Predominant from of bilirubin IX and isomer
Works upto depth of 2 mm takes 1-3 hours for fresh bilirubin from blood to re-equilibrate to tissues,
turn the baby every 2nd hour.
Bilirubin absorb light b/w 400 and 500 nm isomeration and oxidation is maximal between 450460 nm, cool white light (380-700 nm) with peak at 550 nm are most effective.
Green light least effective (form lumi bilirubin)
Light source Blue light
Cool day light fluorescent lamp
Halogen bulb and now fibroptic system under blanket
Level of bilirubin: Normal is 1% of body weight, i.e. 10 mg/dl in 1000 gm baby and 20 mg/dl in
2000 gm baby
Hemolysis lowers the threshold to start phototherapy
Blood bain barrier matures beyond 7 days of life
For Rh immunized babies, begin phototherapy at birth
ADR of phototherapyrash, dehydration, diarrhoea, acute hemolysis, hypocalcemia, bronzing of
skin and retinal image
Control of severe hyperbilirubinemia in full term newborn is with inhibition of bilirubin production
by sn mesoporphyrin, concentration 15 mg/dl 18mg/dl reached in 48-96 hours.
90. Most common cause of cholestatic jaundice in newborn:
(OPG5th/174) (AI 97)
(a) Hypoplasia of biliary tract
(b) Neonatal hepatitis
(c) Choledochal cyst
(d) Physiological cyst
91. A clue to the diagnosis of the following disorder is obtained from simple chemical tests on urine:
(a) Galactosaemia
(b) Cretinism
(c) Neonatal tetany
(d) Mucoviscidosis
(OPG5th/172) (AIIMS 83)
92. Bronze baby syndrome is a complication of:
(OPG5th/173) (JIPMER 89)
(a) Exchange transfusion
(b) Chloramphenicol toxicity
(c) Phototherapy
(d) 100% oxygen therapy
93. Hazards of exchange transfusion include all except:
(OPG5th/174) (PGI 81, AIIMS 83)
(a) Oligaemic shock
(b) Citrate tetany
(c) Cardiac arrest
(d) Hypokalaemia
85 C
86 B
87 D
88 A
89 D
90 A
91 A
92 C
93 D
36 / Pediatric Buster
94. A neonate is presented to the hospital with hepatitis and clay coloured stools. Biopsy of the liver shows giant
cells. The diagnosis is:
(OPG5th/174) (AIIMS/NOV/01)
(a) Neonatal hepatitis with extrabiliary atresia
(b) Physiological jaundice
(c) Extrabiliary atresia
(d) Neonatal hepatitis with physiological jaudice
95. In neonatal cholestasis, if the serum gammaglutamyl transpeptidase is more than 600 IU/L the most likely
diagnosis is:
(OPG5th/174) (AIIMS/02)
(a) Neonatal hepatitis
(b) Choledochal cyst
(c) Hypothyroidism
(d) Biliary atresia
96. Phototherapy in non haemolytic hyperbilirubinemia is indicated when the unconjugated bilirubin level is above:
(a) 12 mg%
(b) 15 mg%
(c) 9 mg%
(d) 18 mg%
(OPG5th/174) (AIIMS 99)
Approach to Indirect Hyperbilirubinemia in Healthy Term Infants without Hemolysis
Treatment Strategies
Age (hr)
<24
24-48
49-72
>72
Phototherapy
Intensive
Phototherapy and
Preparation for
Exchange Transfusion
Exchange
Transfusion if
Phototherapy
Fails
15-18
18-20
20
25
30
30
20
25
25
INDICATORS OF PHOTOTHERAPY
Birth weight
Serum bilirubin at which
phototherapy is indicated
2500 g
15 mg/dl
2000-2500 g
12 mg/dl
1500-2000 g
10 mg/dl
1000-1500 g
7 mg/dl
< 1000 g
5 mg/dl
97. A 2 months baby presents with history of jaundice, turmeric coloured urine, and pale stools since birth.
Examination reveals liver span of 10 cm, firm in consistency and spleen of 3 cm. The most specific investigation
for establishing the diagnosis would be:
(OPG5th/174) (AI/03)
(a) Liver function tests
(b) Ultrasound abdomen
(c) Preoperative cholangiogram
(d) Liver biopsy
98. 8 days old neonate with extensor posture suggests:
(OPG5th/175) (TN/99)
(a) Cerebral palsy
(b) Hypoxic-ischemic encephalopathy
(c) Malnutrition
(d) Infection
Neuropathological Lesions in Hypoxic-ischemic Encephalopathy
Infants
Lesions
Term
Preterm
Long-term sequalae
99. Neonatal seizures in the following disorders is associated with poor prognosis except:
(OPG5th/176) (AI 91)
(a) Hypoglycaemia
(b) Intraventricular haemorrhage
(c) Hypocalcaemia
(d) Meningitis
100. CSF examination in a one day old term male infant with convulsions reveals cell count-10, RBCs/HPF-50 cells,
protein-70 mg/dl, sugar-40 mg/dl. The child is likely to be suffering from:
(OPG5th/177) (KARNATAKA/98)
(a) Meningitis
(b) Intracranial bleed
(c) Hypoglycemia
(d) None of the above
94 A
95 D
96 B
97 C
98 B
99 C
100 C
Newborn Infants / 37
101. Treatment of choice for neonatal seizures:
(a) Phenytoin
(b) Valproate
(c) Diazepam
(d) Barbiturates
102. A newborn is screened for hypoglycaemia in following except:
(a) Large for date
(b) Birth asphygia
(c) Rh incompatability
CAUSES OF HYPOGLYCEMIA
Common
Inadequate substrate, especially if feeding is delayed or is sub-optimal: small for dates and preterm
babies.
Relative hyperinsulinism in infants of diabetic mothers.
Secondary to polycythemia.
Secondary to stressful conditions such as hypothermia, sepsis, asphyxia and respiratory distress.
Rare
Hyperinsulin states: Beta cell hyperplasia (nesidioblastosis), adenoma of beta cells.
Deficiency of hormones such as glucagon, hGH, epinephrine, adrenal and ACTH.
Metabolic diseases such as glycogen storage disease, fructose intolerance, ketotic hypoglycemia,
maple syrup urine disease, etc.
103. Hypoglycemia in late infant and child occurs if blood glucose level is:
(OPG5th/177) (UPSC/01)
(a) 40 mg/dl
(b) 60 mg/dl
(c) 10 mg/dl
(d) 20 mg/dl
104. Absent stomach bubble on antenatal ultrasonography is an important finding for antenatal diagnosis of:
(OPG5th/178) (UPSC 96)
(a) Congenital heart disease in the foetus
(b) Oesophageal atresia in the foetus
(c) Omphalocele in the
foetus
(d) Spina bifida in the foetus
105. Consider the following statements: Tracheo-oesophageal fistula in the neonatal period is characterised by all
except:
(OPG5th/178) (UPSC 98)
(a) Associated hydramnios in the mother
(b) Associated pre-eclamptic toxaemia in the mother
(c) Pneumonia in the infant
(d) Excessive oro-pharayngeal secretions
106. In live births the incidence of congenital malformations of oesophagus is:
(OPG5th/178) (AIIMS 78, 81)
(a) 1 in 1000
(b) 1 in 50,000
(c) 1 in 4000
(d) 1 in 7,00,000
Incidence of oesphageal anomalies is 1 in 4000 live births, the commonest being where the upper part of
oesophagus ends blindly and lower part is connected to trachea by fistula.
1. There may be associated poly-hydramnios and single umbilical artery. The baby has excessive
drooling, saliva is frothy and there is chocking and cyanosis with the first feed.
2. On X-ray air bubble is seen in stomach and lung show atelectasis or pneumonia in the right upper
zone.
107. A newborn baby had normal Apgar score at birth and developed excessive frothing and choking on attempted
feeds. The investigaton of choice is:
(OPG5th/178) (AIIMS/03)
(a) Esophagoscopy
(b) Bronchoscopy
(c) MRI chest
(d) X-ray chest and abdomen with the red rubber
catheter passed per orally into esophagus.
108. A newborn has dribbling after feeds. He has respiratory distress and froths at the mouth. Diagnosis is:
(OPG5th/178)(AI/2001)
(a) Tracheoesophageal fistula
(b) Tetralogy of Fallot
(c) Oesophageal atresia
(d) Laryngeal webs
109. Neonatal hypocalcemia to produce seizures is below serum level ofmg%:
(OPG/178) (ORISSA/98)
(a) 7
(b) 8
(c) 9
(d) 10
110. A neonate presents with acute obstruction with multiple fluid levels. Diagnosis is:(OPG5th/178) (ALL INDIA/02)
(a) Pyloric stenosis
(b) Duodenal atresia
(c) Ileal atresia
(d) Lazzs band
111. Baby of a diabetic mother show all except:
(OPG5th/180) (PGI 85)
(a) Hyaline membrane disease
(b) Large baby
(c) Tremulous baby
(d) Hyperglycaemia
112. Child born to diabetic mother can have:
(OPG5th/180) (PGI 89)
(a) Hyperglycaemia
(b) Hypocalcaemia
(c) Growth retardation
(d) Mental retardation
101 D
102 D
103 C
104 B
105 B
106 C
107 D
108 A
109 A
110 C
111 D
112 B
38 / Pediatric Buster
113. Which of the following is not seen in infants of diabetic mother?
(NEL17th/614) (PGI 89)
(a) Hairy pinna
(b) Large tongue
(c) Hypoglycaemia
(d) Asymmetrical ventricular septal hypertrophy
114. Long-term complication of infants born to IDDM mother is:
(OPG5th/180) (AI 95)
(a) DM
(b) Ketotic hypoglycaemia
(c) Obesity
(d) Blindness
Infant of Diabetic Mother:
Complications
115. The most common cause of death in patient with congenital diaphragmatic hernia is: (OPG5th/168) (AIIMS 98)
(a) Pul. hypoplasia
(b) Intestinal obstruction
(c) Associated malformations
(d) Septicaemia
116. A newborn baby has been referred to the casualty as a case of congenital diaphragmatic hernia. The first clinical
intervention is to:
(NEL17th/1354)(OPG5th/168)(AIIMS/03)
(a) Insert a central venous pressure line
(b) Bag and mask ventilation
(c) Insert a nasogastric tube
(d) Ventilate with high frequency ventilator.
117. X-ray appearance in hyaline membrane disease in:
(OPG5TH/167) (KERALA 94)
(a) Homogenous ground glass appearance (b) Reticulonodular shadow (c) Normal X-ray (d) Air bronchogram
113 B
114 A
115 A
116 C
117 D
Immunity and
Infectious Diseases
1. Which one of the following immunoglobulins is transmitted to the fetus through placenta?
(a) lgG
(b) lgA
(c) lgM
(d) lgD
(OPG5th/184) (UPSC/02)
2. The most common anomaly seen in the foetus of a mother taking lithium carbonate is:
(UP 97)
(a) Cardiac deformities
(b) Neural tube defect
(c) Limb reduction
(d) Genitourinary deformities
AGENTS AFFECTING FOETUS
Drug
Effect on Fetus
Alcohol
1 A
2 A
40 / Pediatric Buster
3. True about measles vaccine is that it is:
(OPG5th/195) (AMU 95)
(a) Thermolabile
(b) Thermostable
(c) Both
(d) None
4. Which one of the following is a conjugated vaccine?
(OPG5th/193)(UPSC/04)
(a) Hepatitis B
(b) Rubella
(c) Haemophilus influenzae b
(d) Pertussis
5. Toxic shock syndrome is due to:
(OPG5th/195) (UP 98)
(a) BCG vaccine
(b) DPT vaccine
(c) Measles vaccine
(d) Hepatitis-B vaccine
DISEASES AGAINST WHICH VACCINES ARE AVAILABLE
(a) Diseases preventable by environmental intevention or by immunization: Japanese encephalitis,
tuberculosis, yellow fever, typhoid fever, rabies, rotavirus diarrhea, cholera, hepatitis A, malaria.
(b) Diseases preventable only by immunization: Poliomyelitis, diphtheria, measles, tetanus, rubella,
pertussis, mumps, meningococcal meningitis (A and C serotypes), influenza, H influenzae
meningitis, chickenpox, S. pneumoniae pneumonia, hepatitis B.
6. The diluent used for measles vaccine should be stored at:
(a) 20C temperature
(b) 20C to 8C temperature
temperature
7. Zero dose of OPV is given at:
(a) Birth
(b) 1 month
(c) 3 months
(d) 9 months
(OPG5th/195) (UPSC/2K)
(c) +2C to +8C temperature
(d) Room
(OPG5th/200t) (AMU 95)
6 weeks
10 weeks
14 weeks
9 months
18-24 months
5 years
10 yr and 16 yr
For pregnant women
:
:
:
:
:
:
:
:
8. The schedule universal programme of immunization in a child begins at birth and end at:(OPG5th/200) ((AI 88)
(a) 3rd year
(b) 4th year
(c) 5th year
(d) 10th year
Chickenpox: Caused by varicella zoster virus
Cowdry type A eosinophillic intranuclear inclusions are seen in cells
Induction period is usually between 14 and 16 days.
The rash is centripetal in distribution appearing on the first day of the illness.
Crusts do not contain viable virus and hence noninfective
9. Chickenpox in children is characterised by all of the following except:
(OPG5th/205) (AI 88)
(a) Presence of live virus in crusts
(b) Lesion appears in crops
(c) Centripetal distribution of rash
(d) Rash shows rapid progression from macules to vesicles
10. Pleomorphic rash is a feature of:
(OPG5th/205)
(DELHI 96)
(a) Chickenpox
(b) Smallpox
(c) Erythema infectosum
(d) Erythema subitum
3 A
4 D
5 C
6 D
7 A
8 C
9 A
10 A
Measles:
Caused by RNA paramyxovirus
There is generalized lymphoid hyperplasia.
There is intranuclear and intracytoplasmic inclusion and multinucleated giant cell are also seen.
On 1st day there is fever2-3 day - Kopliks spot on the inner side of cheek, opposite lower molar teeth.
A maculopapular rash is seen on 4th-5th day
Coombs sign is present
A line of conjunctival inflammation on upper margin
13. Which one of the following is the most complication of measles infection in children? (OPG5th/207)(UPSC/04)
(a) Otitis media
(b) Bronchopneumonia
(c) Encephalitis
(d) Diarrhoea
14. In which one of the following conditions is SSPE a late complication?
(OPG5th/207)(UPSC/03)
(a) Enteric fever
(b) German measles
(c) Herpes zoster
(d) Measles
15. Complication of measles are all except:
(OPG5th/207) (KERALA 95)
(a) Myocarditis
(b) Appendicitis
(c) SSPE
(d) Pancreatitis
Complication:
Most common complication is diarrhoea
Others are:
- Cervical lymphadenopathy
- Bronchopneumonia (young age)
- Encephalitis
- Otitis media
- Myocarditis
- Interstitial pneumonia
- Hepatitis
- Glomerulonephritis
- Achlorhydria
- Appendicitis
- Malnutrition
- Transverse myelitis
- Bleeding manifestation
11 C
12 A
13 B
14 D
15 D
42 / Pediatric Buster
-
17 B
18 A
19 A
20 B
21 C
22 C
23 D
24 B
Complications of Mumps
Edema over manubrium and upper chest due to lymphatic obstruction
Meningoencephalitis
Facial neuritis and auditory nerve damage laeading to deafness
Mumps is probably one of the most common causes of aseptic meningitis
Epididymitis and orchitis are unusual in children but are seen frequenctly in adolscent
Bilateral involvement of testes may rarely lead to ssterilit
Pancreatitis may rarely be followed by diabetes mellitus.
Oophoritis; nephritis; myocarditis, thyroiditis; mastitis; arthritis; dacryoadenitis; uveokeratitis
and thrombocytopenic purpura.
29. Ocular manifestation of mumps is:
(OPG5th/209) (AIIMS 97)
(a) Dacryoadenetis
(b) Chorioretinitis
(c) Anterior uveitis
(d) Memb. conjunctivitis
30. The most frequent complication of mumps infection in young children is:
(OPG5th/209)(UPSC/03)
(a) Meningoencephalitis
(b) Orchitis
(c) Oophoritis
(d) Migratory polyarthralgia
31. The most common form of extrasalivary gland involvement in mumps is:
(OPG5th/209) (UPSC/01)
(a) Encephalomyelitis
(b) Pancreatitis
(c) Aseptic meningitis
(d) Oophritis
32. Acute stage of poliomyelitis lasts for:
(OPG5th/211) (PGI 80, DNB 91)
(a) 10 to 15 days
(b) 1 to 5 days
(c) 1 to 4 weeks
(d) 1 to 2 years
33. All of the following are features of poliomyelitis except:
(OPG5th/211) (ICS/98)
(a) Acute onset
(b) Progressive course
(c) Intact sensation
(d) Febrile onset
34. Diagnosis of poliomyelitis is confirmed by:
(OPG5th/211) (AIIMS 99)
(a) Presence of organism in stool
(b) 4-fold rise in antibody titre
(c) More than 60 days of paralysis
(d) CSF showing organism
35. Death in poliomyelitis is usually due to:
(OPG5th/212) (AI 92)
(a) Hypertension
(b) Respiratory paralysis
(c) Aspiration
(d) Arrhythmias
ACUTE FLACCID PARALYSIS (AFP)
What is AFP?
Acute flaccid paralysis means that paralysis is of acute onset (<4 weeks) and the affected limb or
limbs are flaccid, i.e. floppy or limp. Tone is diminished and deep tendon reflexes are diminished
or absent.
Rapid onset of weakness/paralysis of one limb or limbs in a child < 15 years.
Weakness may include muscles of respiration/swallowing.
Affected limbs are flaccid, i.e. tone is diminished.
There is a reflexia or diminished deep tendon reflexes.
Paralysis may be sudden in all limbs or may extend from one limb and progress to maximum
severity.
Sensory loss may/may not be there.
Common causes of AFP:
Poliomyelitis
Polio like illness caused by other neurotropic enteroviruses.
25 A
26 B
27 C
28 B
29 A
30 B
31 C
32 A
33 B
34 A
35 B
44 / Pediatric Buster
Other causes:
Post-diarrhoeal hypokalemia
Acute stage of hemiplegia
Tick paralysis
Botulism
Cases of AFP are confirmed as polio if:
They are associated with isolation of wild polio viruses from the stools of the case; or
They have residual neurologic sequalae at 60 days after the onset of paralysis; or
They died before FU could determine whether residual neurologic sequalae was present at 60 days
They were lost before FU could determine whether compatible neurologic sequalae was present at
60 days
Why AFP (not polio) must be reported?
A clinical diagnosis of polio may not be immediately apparent or may be inaccurate
Virus shedding is brief and a virological culture report takes 4-6 weeks to come
The sensitivity of surveillance can be measured only by the AFP rate (1 per 100,000)
Eradication (the ultimate goal) requires sensitive surveillance even in the absence of polio.
Each AFP case:
Case investigation to be done within 48 hours with all demographic and clinical information
2 stool samples to ref. lab collected 24 hrs apart within 14 days of onset of paralysis to be sent
to the lab on ice to be cultured to isolate the polio virus
Community investigation to identify additional cases
Outbreak control additional dose of OPV to children < 5yr of age
Entire village rural area
Municipal ward urban area
Follow up examination 60 days after onset
Purpose of AFP surveillance:
To identify high risk areas or groups where child polio virus continues to spread
To target areas for supplemental immunisation
To monitor progress to assess whether strategies are implemented effectively or not
To certify a country polio free
To detect importation of wild polio virus
Role of laboratory in AFP surveillance:
To confirm polio by virus isolation
To trace the origin of a case by maintaining a reference bank of molecular structure of known viruses
from different geographical areas.
To certify that polio has been eradicated
To assess vaccine potency
Standard performance indicators for AFP surveillance:
AFP rate
1. Target>1/00,00 children < 15 yrs of age. This is an indicator of surveillance sensitivity thus a rate
of < 1/00,000 means surveillance is not complete and cases are probably being missed
36 C
37 A
38 A
39 C
40 A
41 B
46 / Pediatric Buster
C/F- Fever; malaise; sore throat, loss of apetite; lymphadenopathy and maculopapular rash
Hepatosplenomegaly with elevated SGOT and SGPT levels
Frank jaundice is rare.
Rash usually develops on ampicillin intake
Patient may have aseptic meningitis or meningoencephalitis.
The disease is self limiting (2 mon)
Complications
Splenic rupture
CNSconvulsions, GB syndrome; peripheral neuropathy
Immune hemolytic anemia with thrombocytopenia
Myocarditis and hepatitis
Diagnosis is accomplished by absolute increase in lymphocytes which are large and atypical
Heterophile antibodies are tested by positive Paul Bunnel test (often negative in under 4 yr of child)
A screening test (monospot) has been developed for rapid diagnosis.
No specific treatment but acyclovir may be used in chronic infectious mononucleosis and EBV
associated polyclonal lymphoproliferation.
42. All of the following statements regarding infectious mononucleosis are true except:
(OPG5th/219) (AI 90)
(a) The onset is acute with fever, sore throat and lymphadenopathy
(b) Atypical lymphocytes are seen in
peripheral blood smear
(c) Acute lymphoblastic leucaemia is a common sequel
(d) Heterophile antibody
test is useful in diagnosis.
HIV
Perinatal HIV-1 transmission in the leading cause of pediatric AIDS
Most children are diagnosed b/w 2 months and 3 year of age.
Clinically present as prolonged diarrhoea
Failure to thrive
Oral candidiasis
Hepatosplenomegaly
Septicemia with Strepto Strep Pneumoneae or Salmonella
Chronic sinusitis may also be the major sign.
LIP is a distinctive presentation of HIV-1 infection and is probably the most common clinical
presentation in children.
LIP is defined as reticulonodular pulmonary infiltrates composed or CD8 + lymphocyte persisting
for 2 month or more with or without higher adenopathy that donot respond to antimicrobials.
The most common and highly lethal opportunistic infection is P. carinii pneumonia.
M. tuberculosis and malaria are also major opportunistic pathogens in AIDS other are Candida and
Toxoplasma.
Neoplasms are relatively uncommon in pediatric HIV-1 infected patients.
The age at the initial clinical presentation and CD4 + lymphocyte count are the major factors in
prognosis.
Drugs to be used in HIV-1 infected children arezidovudine and dideoxylnosine.
43. Percentage of HIV infection in child of a HIV +ve mother is:
(OPG5th/220) (KERALA 97)
(a) 20-30%
(b) 10-20%
(c) 70-80%
(d) 100%
44. A 2-year-old girl presents with recurrent episodes of diarrhoea (mucus and blood at times ) hepatosplenomegaly,
perianal excoriation and resistant oral thrush. She weighs 6 kg and measures 78 cm in hieght. The most likely
diagnosis is:
(OPG5th/222) (UPSC/01)
(a) Necrotising enterocolitis
(b) Lactose intolerance
(c) HIV infection
(d) Campylobacter jejuni infection
45. All of the following methods are used for the diagnosis of HIV infection in a 2-month-old child, except:
(a) DNA-PCR
(b) Viral cultue
(c) HIV ELISA
(d) p 24 antigen assay
(OPG5th/222) (AIIMS/03)
42 C
43 A
44 C
45 C
47 C
48 C
49 A
50 C
51 D
52 D
53 B
54 C
55 C
56 D
57 A
58 B
48 / Pediatric Buster
Typhoid fever:
Incubation period is 14 days
Temperature rises in a step ladder pattern
Constipation is frequent
There is bradycardia
Rash comes on 6th day in crops as rose spots which fade on pressure.
Adbomen is distended and a tympanic note is heard on tapping.
Spleen is palpable 1 or 2 cm below the costal margin.
Basal rales are heard over lungs
Child has muttering delirium and pick at bed clothes peculiar of typhoid state.
Hemorrhage and perfoation may occur in 2nd or 3rd wk.
There may be chills, myalgia, anorexia, cough vomiting, arthralgia etc.
Complications: Parotitis; bronchitis; pneumonia; myocarditis, fatty liver; encephalitis; peripheral
neuritis; aphasia; cerebral vein thrombosis; osteomyelitis, uveitis etc.
Widal agglutination test shows a diagnostic titre of 1/80 or more for O antigen of S. typhi.
A four-fold rise in agglutinin titre of nonimmune individual is diagnostic
Elevation of antibody titre against O (somatic) antigen has a better diagnostic value.
Anamnestic reaction means the rise of titre of H antibody with non-tyhoid fevers but the titre of
O antibodies does not rise.
Blood counts show below normal leucocyte count with a moderate neutropenia and relative
lymphocytosis.
Chloramphenicol is useful drug; others are ampicillin, cotrimoxazole and ciplox (avoided in children
due to possible risk to growing cartilage)
Prevention is by Ty 21 a (Typh oral) 3 capsules on 1, 3, and 5 days.)
60. Ghons focus is:
(OPG5th/232) (TN 90)
(a) Primary complex
(b) Post-primary TB
(c) Miliary TB
(d) TB lymph nodes
61. All of the following statements with reference to childhood tuberculosis are correct except:
(OPG5th/232) (UPSC/2001)
(a) Primary complex is mostly symptomatic (b) Mantoux test is useful to detect infection
(c) Early morning
gastric lavage for AFB is a useful test (d) Chest X-ray is the most important tool in diagnosis of miliary
tuberculosis
62. False-negative tuberculin reaction occurs in children in the following except:
(OPG5th/232) (AI 91)
(a) Very severely malnourished child
(b) Patient on corticosteroid therapy
(c) 4-6 weeks following measles
(d) Atypical mycobacteriosis.
63. Mantoux test reading of less than 5 mm indicates:
(OPG5th/234) (JIPMER 91)
(a) Tuberculous infections
(b) Disseminated TB
(c) Susceptibility to TB
(d) Immunity to TB
64. Dose of rifampicin in mg/kg body weight/day for treatment of tuberculosis in a child is:
(a) 5
(b) 10
(c) 15
(d) 20
(OPG5th/236) (AIIMS 81, 91)
65. A poverty stricken mother suffering from active tuberculosis delivers a baby. Which one of the following advices
would be the most appropriate in her case?
(OPG5th/237) (UPSC 96)
(a) Breastfeeding and BCG immunization
(b) Breastfeeding and isoniazid administration
(c) Expressed breast milk and BCG immunization
(d) Stop feeds and isoniazid administration
Indications of treatment of primary TB in children with INH and r-cin for 9 mon are:
1. Positive tuberculin reactors
2. Under age of 3 yrs
3. Recent conversion significant
4. Radiological evidence
5. Presence of tuberculous toxemia
6. Children suffering from or recently recovered from measles or whooping cough.
60 A
61 A
62 D
63 C
64 D
65 B
66. Basanti age 29 years from Bihar presents with tuberculosis and she delivers baby. She will be instructed all
of the following except:
(OPG5th/237) (AI/2001)
(a) Give baby BCG
(b) Withhold breastfeeding
(c) Give ATT for 2 years
(d) Proper disposal of sputum
67. A mother has active TB and recently delivered a child; what is to be done to newborn:(OPG5th/237)(AI/2000)
(a) Give BCG and isolate the baby
(b) Give BCG, INH and RMP and continue breastfed
(c) Give BCG and
no breastfeeding and no isolation
(d) Give BCG and breastfeeding is allowed
Baby born to mother with TB (diagnosed in 3rd trimester or during delivery)
Breastfeeding must be continued.
BCG vaccine should be given at birth
If chest X-ray is normal, then 6 HR
If chest X-ray is abnormal, then 2 HRZ/7 HR
Congenital tuberculosis: 2 HRZ/7HR4
68. Which of the following are true about cerebral malaria:
(OPG5th/242) (PGI 2000)
(a) Quinine is used in the treatment
(b) It is seen more in the hyperendemic areas
(c) Most common
presentation is with seizures
(d) Associated with hypoglycemia
(e) It is more common in infants
MALARIA
Sickle cell trait; thalassemia; G6PD deficiency and altered ATP in erythrocytes are relatively
immune from malaria.
Chronic progressive nephrotic syndrome is only observed in P. malarial infections.
Cerebral malaria is rare in children with protein calorie malnutrition.
Individuals infected with malaria respond poorly to other antigens such as tetanus toxoid.
69. The mechanism of diarrhoea in giardiasis is prevention of absorption of fat by:
(a) Mechanical coating of the walls of intestine
(b) Secondary bacterial infection
(d) Production of endotoxins
70. Treatment of giardiasis is:
(a) Mebendazole
(b) Piperazine
(c) Metronidazole
(d) Bephenium
71. Which of the following is not a usual feature of ascariasis:
(a) Abdominal pain
(b) Urticaria
(c) Anemia
(d) Loefflers syndrome
66 B
67 A
68 A
69 A
70 C
71 C
(OPG5th/251)
(CMS 85)
(c) Production of exotoxins
(OPG5th/251) (AIIMS 83)
(OPG5th/254) (AIIMS 92)
Gastrointestinal System
Intussusception in infants is suspected by severe colicky abdominal pain; blood stained stools like
current jelly and empty right iliac fossa.
2 A
3 B
4 A
5 A
6 A
Gastrointestinal System / 51
Reosmal (mmol/L)
Glucose
Sodium
Potassium
Chloride
Citrate
Magnesium
Zinc
Copper
Osmolarity
125
45
40
70
7
3
0.3
0.045
300
111
90
20
80
10
311
14. Treatment in 6-month-old child with acute watery diarrhoea without signs of dehydration is:
(a) Mothers milk and household fluids
(b) ORS and antibiotics
(c) Mothers milk and antibiotics
(d) Mothers milk and ORS
(OPG5th/273) (JIPMER 95)
15. A 2 years old child 6.5 kg in weight presents with vomiting and diarrhoea for 2 days and skin over abdomen when
lifted takes seconds to come back, the diagnosis goes in favour of:
(OPG5th/273) (AIIMS 2000)
(a) Mild dehydration
(b) Severe dehydration
(c) No dehydration
(d) Dehydration cannot be detected in
this case from abdominal skin
7 B
8 C
9 C
10 A
11 A
12 D
13 A
14 A
15 B
52 / Pediatric Buster
Assessment of Dehydration
Mild dehydration (3-5%): Normal or increased pulse, decreased urine output thirsty, normal physical
examination.
Moderate dehydration (7-10%): Tachycardia, little or no urine output, irritables/lethargic, sunken
eyes and fontanel, decreased tears, dry mucous membranes, mild tenting of the skin, delayed capillary
refill, cool and pale.
Severe dehydration (10-15%): Rapid and weak pulse, decreased blood pressure, no urine output, very
sunken eyes and fontanel, no tears, parched mucous membranes, tenting of the skin, very delayed
capillary refill, cold and mottled.
16. A two old girl has weight of 6.7 kg. She suffered an attack of diarrhoea. If skin is pinched and get back in seconds.
Which of the folowing is true about her:
(OPG5th/273) (ALL INDIA/02)
(a) Severe dehydration
(b) Mild Dehydration
(c) Moderate dehydration
(d) No comment can be made
on these data
17. A child suffering from acute diarrhea is brought to the casualty and is diagnosed as having severe dehydration
with pH of 7.23, serum Na-125, serum K-3, HCO316. The best IV fluid of choice is:
(OPG/5th/274) (AIIMS /MAY/01)
(a) 3 % saline
(b) Normal saline
(c) N/3 saline + 10% dextrose
(d) N/3 saline + 5% dextrose
18. A 5-yr-old boy passed 18 loose stools in last 24 hr and vomited twice in last 4 hours. He is irritable but drinking
fluids. The optimal therapy for this child is :
(OPG5th/ 274) (AI/03)
(a) Intravenous fluids
(b) Oral rehydration therapy
(c) Intravenous fluid initially for 4 hr followed by oral
fluids
(d) Plain water ad libitum.
19. Treatment of choice in severe dehydration is:
(OPG5th/274) (KERALA 94)
(a) Isolyte-P
(b) Normal saline
(c) Plasma
(d) Ringer lactate
Composition of intravenous solution
Fluid
[Na+]
[Cl-]
[K+]
[Ca2+]
[Lactate-]
154
77
38.5
130
154
77
38.5
109
28
20. When severe dehydration in a neonate occurs, amount fluid replacement in l hour:
(a) 20-40 ml/kg
(b) 5-10 ml/kg
(c) 10-15 ml/kg
(d) 15-20 ml/kg
(OPG5th/274) (CUPGEE 2001)
21. A 6-month-old child having severe dehydration comes to the casualty with weak pulse and unrecordable BP.
Repeated attempts in gaining IV access has failed. The next best step is:
(OPG5th /274)(AIIMS /MAY/01)
(a) Venesection
(b) Jugular vein catheterisation
(c) Intravenous IV fluids
(d) Try again
22. Coeliac disease is due to sensitivity to:
(OPG5th/281) (AIIMS 83)
(a) Milk
(b) Maize
(c) Wheat
(d) Meat
23. Which one of the following cereals is not harmful in the case of gluten sensitive enteropathy ?
(a) Rye
(b) Maize
(c) Wheat
(d) Barley
(OPG5th/281) (UPSC 98)
Coeliac disease is characterized by excretion of fat in stools and serum xylose levels less than
20 mg/10 ml. Treatment is exclusion of all cereals contaning gluten (wheat; rye, barley and oats)
from the diet.
16 D
17 B
18 B
19 D
20 A
21 C
22 C
23 B
24 C
25 D
Gastrointestinal System / 53
26. Cystic fibrosis is:
(OPG5th/282) (UP 96)
(a) Autosomal dominant
(b) Autosomal recessive
(c) X-linked dominant
(d) X-linked recessive
27. The following can cause recurrent respiratory infection:
(OPG5th/283) (AIIMS 84)
(a) Tetralogy of Fallot
(b) VSD
(c) Cystic fibrosis
(d) TGV without pulmonary stenosis
Cystic Fibrosis:
Autosomal recessive transmission.
Disease is characterized by chronic airway infection that ultimately leads to bronchiectasis and
bronchiolectasis; exocrine pancreatic insufficiency abnormal sweat gland function and urogenital
dysfunction
There is impaired digestion and absorption; salty skin due to increased secretion of chlorides in the
sweat; Intestinal obstruction; biliary cirrhosis of liver, intussusception; meconium ileus; chronic
diarrhoea and recurrent respiratory tract infection.
Analysis of sweat and chloride is a reliable diagnostic test level of Cl > 70 mEq/L.
There is decrease vital capacity and decreased lung compliance.
Haemophilus and S. aureas are often the infective agents. Mycobacerium tuberculosis is rare.
Earliest chest X-ray change in lung is hyperinflation.
Pneumothorax is common; respiratory failure and cor pulmonale are prominent features in cystic
fibrosis.
Treatment is with pancreatin; suitable antibiotics, humidification of air.
28. A young child has eaten icecream and got the symptoms of abdomen distension, diarrhoea and flatulence. Same
episode is seen with ingestion of icecream/milk also, the child is suffering from deficiency of:
(a) Lactase
(b) Sucrase
(c) Salivary amylase
(d) Pancreatic amylase
(OPG5th/282) (AIIMS/99)
29. A mother kissing her baby finds that the babys skin is salty, the diagnosis is:
(OPG5th/283) (TN 92)
(a) Fanconi syndrome
(b) Thalassaemia
(c) Cystic fibrosis
(d) Niemann-Pick disease
30. Mucoviscidosis is most commonly related to:
(OPG5th/283) (AI 96)
(a) Fibrocystic disease of pancreas
(b) Duodenal atresia
(c) Diaphragmatic hemia
(d) Annular pancreas
31. Cause of meconium ileus is:
(OPG5th/283) (AI 96, 98)
(a) Fibrocystic disease of pancreas
(b) Liver aplasia
(c) Malnutrition
(d) Cirrhosis
32. Abetalipoproteinaemia is first manifested clinically by:
(OPG5th/283) (JIPMER 78, PGI 80)
(a) Hypohydrosis
(b) Tremors
(c) Steatorrhoea
(d) Convulsions
(OPG5th/294) (AIIMS/03)
(c) Idiopathic portal hypertension
27 C
28 A
29 C
30 A
31 A
32 C
33 B
54 / Pediatric Buster
34 B
Haematological Diseases
Hemoglobin:
Six hemoglobins are detected normally in humans as = gower -1
- Embryonic =
gower - 2 Poctland
- Fetal
=
Hb F
Hb A
- Adult
=
Hb A2
In embryos of 4-8 wk gestation the gower hemoglobin predominate but by the 3rd month they have
disappeared.
After 8th gestational wk HbF is the predominant hemoglobin; in the 6 months old fetus it
constitutes 90% of the total hemoglobin then a gradual decline; so that at birth Hbf averages 70%
of the total.
HbA can be detected as early as 16-20 wk of gestation. By the 6 months of gestation there is about
5-10% of HbA and at term 30% by 6-12 months of age the normal adult hemoglobin pattern appears.
Iron deficiency Anemia:
With term pregnancy 900 mg of iron is lost by mother to fetus; placenta and PPH.
Normal infant has 250-300 mg of iron at birth.
Pallor is major symptom of deficiency.
Patients suffer from frequent infections; splenomegaly cardiac enlargement and systolic flow
murmurs and protein losing enteropathy.
Some children develop a liking for nonedible substance (PICA) and are at risk of lead poisoning.
Sequence of changes are
- Decreased storage iron
- Decreased ferritin < 10 mg/ml
- Increase TIBC > 350 mg/dl
- Decreased serum iron
- Increased reticulocyte count
- FEP/Hb > 2.8 mg/g
Elemental iron is given in optimal dose of 5 mg/kg clinical improvement is seen within 24 hr.
Initial bone narrow response is seen in 48 hrs.
Rise in reticulocyte count occurs by 2-3 days and peaks on 10th day of starting the therapy.
1. Child with microcytic-hypochromic anemia not responding to iron the disease to be excluded is:
(a) Thalassaemia
(b) Haemophilia
(c) Renal failure
(d) Sickle cell anemia
(OPG5th/298) (AI 93)
2. Microcytic-hypochromic anemia is seen in all of the following conditions except:
(OPG5th/298) (AI 90)
(a) Beta thalassaemia
(b) Lead poisoning
(c) Iron deficiency anemia
(d) Chronic renal failure.
1 A
2 D
Hamatological Diseases / 56
Microcytic-hypochromic anemia:
- Iron deficiency anemia
- Thalassemia
- Pyridoxine responsive anemia
- Lead poisoning
- Dyserythropoietic anemia
Macrocytic anemia:
- Cobalamin deficiency (Vit B12)
- Folic acid
- Cirrhosis of liver
- Hereditary orotic aciduria
- Di Gugliemos syndrome (AML + Anemia)
- Thiamine responsive
- Hyperthyroidism
- DrugsPAS; colchicine; neomycin DHFRase inhibitors, alcohol 5-FU; hydroxyurea, acyclovir;
zidovudine and Phenytoin.
3. The haemoglobin to appear first in the fetus is:
(NEL 17th/1602) (AIIMS 83)
(a) Hb A
(b) Hb A2
(c) Hb F
(d) Hb Gowers
4. Fetal Hb equals adult Hb at:
(NEL17th/1602) (TN/99)
(a) At birth
(b) 2 months
(c) Oxygen
(d) Ventilation
(e) Digoxin
5. Macrocytosis is seen in all of the following disorders except:
(NEL 17th/1612)(NEL/1379) (UP 97)
(a) Hypothyroidism
(b) Thalassaemia major
(c) Folic acid deficiency
(d) B12 deficiency
6. Megaloblastic anemia secondary to folate deficiency is best corrected by administering folate:
(OPG5th/303) (JIPMER 81, AMU 87)
(a) Orally 2 mg daily
(b) IM 10 mg daily
(c) IV 10 mg daily
(d) 1 gm IM
Megaloblastic Anemia:
Most common in infants feeding on Goats milk (poor source of folate)
Predisposing factors are- Chronic diarrhoea
- Malabsorption
- Recurrent Infections
Patient is pale; irritable; fail to thrive, Increased pigmentation; tremors adn developmental
retrogression is also seen.
Nutritional megaloblastic anemia is unsual after 3-4 yr.
Lab findings are- Macrocytosis (MCV>100fL)
- Polymorphonuclear hypersegmentation
- Fenestrated pattern of chromatin
- Decreased megakaryocytes in bone marrow.
Dose of folic acid to be administred is 2-5 mg/day.
Hematological Disorders:
Transtition from the proerythroblast to the most nature normoblast involves 4 cell divisions over
a 4-day period.
Reticulocyte stays in bone marrow for 2.5-3 days and for another 24 hr in general circulation before
assuming morphologic appearance of a mature RBC.
During the last 3 months of gestation, gamma chain synthesis switches to chain synthesis.
Normal infant has 250-500 gm of iron at the time of birth.
- The level of free haptoglobin decreases.
Plasma hemopexin decreases
3 D
4 B
5 B
6 A
57 / Pediatric Buster
Hereditary Spherocytosis:
Autosomal dominant disorder where spectrin is deficient.
Characterized by anemia; splenomegaly and jaundice.
Hepatosplenomegaly indicates extramedullary hematopoiesis.
Jaundice may be intermittent and tends to be less pronounced in childhood.
Pigment gallstones are common even in childhood.
Chronic leg ulcer is infrequent.
Parvovirus crisis is sometimes encountered.
MCH is usually N or slightly decreased but MCHC is high.
Osmotic fragility is increased.
Coombs test in negative (If +ve suggests immune hemolytic anemia)
Splenectomy inproves the picture but deferred until the child is 4-5 yr.
8. Which of the following is not characteristic of thalassaemia major?
(OPG5th/309) (AIIMS 83)
(a) Hypochromic-microcytic anaemia
(b) Splenomegaly
(c) Target cells
(d) Decreased serum iron
9. In beta thalassemia, there is:
(OPG5th/309) (AIIMS/MAY /01)
(a) Increase in beta chain, decrease in alpha chain
(b) Decrease in beta chain, increase in alpha chain
(c) Decrease in beta chain, decrease in alpha chain
(d) Increase in beta chain, increase in alpha chain
10. A child presented with anemia, which was microcytic hypochromic, and hepatosplenomegaly with target cell.
There was a history of similar illness in the family the best investigation to establish the diagnosis would be:
(OPG5th/310) (AIIMS/NOV/01)
(a) G6PD assay
(b) Electrophoresis
(c) Bone marrow examination
(d) Coombs test
11. A 5-year-old girl came with history of progressively increasing pallor since birth and hepatosplenomegaly. Which
of the folowing is the most relevant test for achieving diagnosis:
(OPG5th/310) (AI/04)
(a) Hb electophoresis
(b) Peripheral smear examination
(c) Osmotic fragility test
(d) Bone marrow
examination.
Beta-thalessemia Major:
Also called cooleys or Littoral anemia,
Most severe form of congenital hemolytic anemia.
Manifests after first 4-6 months of life as failure to thrive
Severe anemia
7 C
8 D
9 B
10 B
11 A
Hamatological Diseases / 58
Beta-thalessemia Minor
Heterozygotes for thalassemia are protected from malaria.
Resembles more or less a iron deficiency anemia.
A characteristic finding is increase in HbA2 (4-8% of Total Hb) with normal levels of HbF.
(Alpha-thalassemia HbH)
Four beta peptide chains polymerize to a tetrameric form forming HbH.
Alpha-thalessemia is most prevalent in the countries of south-east Asia.
No hepatosplenomegaly and if present appears later.
Iron deficiency is severe because HbH is not bound to haptoglobin and lost in urine.
Treatment of Thalassemias:
1. Blood transfusion (Hypertransfusion protocal to keep Hb between 10.5-11gm%) not to rise above
16 gm% and fall below 10.5 gm.
2. Chelation by desferrioxamine (Cataracts are reported on long term use).
3. Spleenectomy (in cases with hypersplenism)
4. Bone marrow transfusion
5. Pharmacological upgrading of HbF production by 5-azacytidine or hydroxyurea.
6. Gene therapy.
12. Ideally children with thalassaemia should be transfused with:
(OPG5th/312) (AIIMS 83)
(a) Packed RBC
(b) Platelet rich plasma
(c) Washed RBC
(d) Blood of polycythaemia
13. The most appropriate drug used for chelation therapy in beta thalessemia major is:
(OPG5th/312) (AI/03)
(a) Oral desferrioxamine
(b) Oral deferiprone
(c) Intramuscular EDTA
(d) Oral succimer
14. Spleen is palpable in all haemolytic anemias in a child of 8 years of age, except in: (OPG5th/314)(UPSC/02)
(a) Thalassemia
(b) Spherocytosis
(c) Sickle-cell anemia
(d) Haemolysis in malaria
Sickel cell anemia:
It is substitution of valine for glutamic acid at the sixth position of b chain produces HbS.
Common in black Africans.
Tendency of sickling depends on the amount of HbS and its interaction with other hemoglobin chain
in the cell.
Sickling increases with
1. HbS > HbC > HbD > HbF
2. Dehydration and increased MCHC
3. Fall in pH.
12 A
13 A
14 C
59 / Pediatric Buster
(OPG5th/327)(AI 97)
(d) BT is normal
16 C
60 / Pediatric Buster
Clotting Time Increased:
Only PT prolongedFactor VII or fibrinogen deficiency
Only PTT prolongedCongenital deficiency of first stage factors, i.e. XII, XI, IX and VIII and von
Willebrands disease
Both PT and activated PTT prolonged-vit K deficiency, severe liver disease and congenital deficiency
of 2nd stage factor; X and V.
17. Aminocaproic acid would be recommended for a haemophiliac child with:
(OPG5th/328) (PGI 79, AIIMS 81)
(a) Epistaxis
(b) Haematuria
(c) Oral bleeding
(d) Haemarthrosis
18. Treatment of neonatal idiopathic thrombocytopenia is:
(NEL17th/1671) (AIIMS 91)
(a) Immunoglobulins
(b) Pulse methylprednisolone
(c) Dexamethasone
(d) Platelets transfusion
19. Most frequent cause of neonatal thrombocytopenic purpura is:
(NEL 17th/1672) (PGI 80, AIIMS 82)
(a) Infection
(b) Erythroblastosis
(c) Drug idiosyncrasy
(d) Large haemangiomas
20. True about idiopathic thrombocytopenic purpura is A/E:
(NEL 17th/1670) (AIIMS/99)
(a) Joint haemorrhage is often seen (b) Increase megakaryocytes in bone marrow (c) Most common in children
(d) Males commonly affected in chronic ITP
Idiopathic Thrombocytopenic purpura:
Characterized by petechial hemorrhages; thrombocytopenia; normal or increased no. of mega
kacyocytes in bone marrow.
There is platelet associated immunoglobulin G (GAIgG).
The disease is due to interaction of platelets and immune complex formed during antibody response
to a viral infection, especially of respiratory tract.
- There may even be neonatal thrombocytopenia.
- Peak age of incidence is 2-8 yr.
- hematemesis, melena and bleeding in joints are unusual.
- Anemia is proportionate to degree of bleeding.
- Spleen is often not palpable.
- Bleeding time prolonged.
- Capilllary fragility test +ve
- Test of coagulation factors normal.
- Short course of prednisolone is effective in reducing bleeding even before platelet count returns
to normal.
- High does I/V lg and immuno suppresants also used. (Azathioprine improves platelet count in
HIV-induced thrombocytopenia)
- Splenectomy is done as last resort.
- All children should receive meningococcal, H. influenzae and pneumococcal vaccine 3 weeks prior
to splenectomy.
- Colchicine and danazol are also being tried in chronic ITP.
- Treatment of chronic ITP is based on symptomatology rather than on the platelet count.
Other Causes of Purpura
- HUS
- TTP
- DIC
- Aplastic anemia
- Cytotoxic therapy
- Infections
- Giant hemangiomas
- Wiskot Aldrich syndrome.
- Anaphylactoid purpura
- Thromboesthenia (Normal number of platelets with defective function)
17 C
18 A
19 A
20 A
Breathing movements are identified as early as 11 wk but are irregular till 20th wk.
By 24th wk the surfactant precussor appears as inclusion bodies in alveolar lining cells.
Intrapleural negative pressure required for the first breath is 40-100 cm of H20
The normal newborn requires about 7 ml of oxygen/min/kg.
Commonest cause of stridor in newborn is laryngomalacia also caused by cystic hygroma and
avascular rings.
Epiglottitis is caused by Haemophilus influenzae B.
Infections croup (croup syndrome) is usually caused by parainfluenzae type 1.
Bacterial superinfections are unusual.
Spasmodic croup occurs between 1-3 yr.
Antibiotics help epiglottitis but not croup as it viral in origin.
Otitis media in children < 6 yr Hemophilus influenzae > 6 yr is pneumococci
Salicylates are responsible for dyspnea in childhood.
2 B
3 D
4 A
5 D
6 C
7 B
8 D
9 B
10 A
11 B
12 A
13 B
14 C
15 C
16 D
63 / Pediatric Buster
Clinical category
Very severe pneumonia
Severe pneumonia
Pneumonia
Cough or cold
Essential feature
Central cyanosis, or not able
to breastfeed or drink, or convulsions, or
lethargy or unconsciousness, or severe
respiratory distress (e.g. head nodding)
Lower chest indrawing or nasal flaring, and
no signs of very severe pneumonia
Fast breathing, i.e. Age RR/min 2 m up to
12 m 50 12m up to 5y 40 and
No indicators of severe or very severe
pneumonia
No fast breathing, and no indicators of
severe or very severe pneumonia
Treatment strategy
Inpatient care IM or IV,
chloramphenicol or ampicillin plus
gentamicin; give oxygen
Inpatient care IM or IV benzyl
penicillin or ampicillin
Home care; oral cotrimoxazole or
amoxycillin or ampicillin or IM
procaine penicillin
Home care; home remedy for cough;
paracetamol for fever
Essential feature
Presence of any of the following in a young
infant, thought to be sick by the mother
Convulsions
Bulging fontanelle
Lethargic or unconscious
Less than normal movement
Fever (axillary temperature > 37.5C) or low
body temperature (axillary temperature < 35.5C)
Many or severe skin pustules
Umbilical redness extending to the skin
Fast breathing (respiratory rate 60 per minute)
Severe chest indrawing
Nasal flaring
Respiratory grunting
Cough and severe undernutrition (weight < 2.0 kg in
the first month, or presence of visible severe wasting)
Not able to feed at all, or no attachment with breast
at all, or no sucking at all
Red umbilicus or umbilicus draining pus, or
Skin pustules
No features suggestive of possible serious bacterial
infection or local bacterial infection
Bronchiolitis:
Recognized cause of wheezing in infants below the age of 2 yr
Usually occur in winter or spring and caused by respiratory syncytial virus.
It is a self limiting disease.
25% cases may progress to bronchial asthma in later life.
Hypoxemia and respiratory acidosis; increase eosinophils in blood respiratory secretion is there.
Respiratory distress out of proportion to extent of disease.
Treatment is symptomatic with cold humidification oxygen.
Antibiotics are generally unnecessary.
Sedatives should not be used.
Ribavarin by aerosol is of immense help.
Best is steroids in case of emergency.
19. A 9-month-old infant presents with a 2-day history of fever, cough and breathlessness following an upper respiratory infection. She is febrile and has a respiratory rate of 80/min. intercostal and subcostal retractions and
extensive rhonchi on ascultation. A chest X-ray reveals a hyperinflated chest. The most likely diagnosis is:
(OPG5th/353) (UPSC/99)
(a) Bronchial asthama
(b) Foreign body aspiration
(c) Bacterial pneumonia
(d) Bronchiolitis
20. A child presents with fever, bilateral crepts and rhonchi, most likely diagnosis is infection with:
(a) Rhinovirus
(b) RSV
(c) Echovirus
(d) Measles
(OPG5th/353) (AIIMS/2K)
5th
21. Which of the following is/are true about bronchiolitis in children:
(OPG /353) (AIIMS /MAY/01)
(a) Caused by respiratory syncytial virus
(b) Hyperinflation of chest
(c) Pleural effusion
(d) May lead
on to bronchial asthma later in life
22. All of the following is true of bronchiolitis except:
(OPG5th/353) (PGI 88)
(a) Caused by RSV
(b) Occurs in school age
(c) Leads to asthma
(d) Treatment is oxygen
23. All of the following statements are true about bronchiolitis except:
(OPG5th/353) (AI 90)
(a) It is caused by respiratory syncytial virus
(b) It shows seasonal variation
(c) It needs antibiotic therapy
(d) It is common below 1 year of age
24. Following are used in treatment of bronchiolitis except:
(OPG5th/353) (AI 93)
(a) Humidified oxygen
(b) IV fluids
(c) Antiviral drugs
(d) Antibiotics
25. The treatment of bronchiolitis is:
(OPG5th/353)
(a) Humidified oxygen
(b) Antibiotics
(c) Steroids
(d) All of the above
26. In a child with exercise induced asthma, which is done:
(OPG5th/354) (JIPMER 80, DELHI 93)
(a) Prophylaxis with steroids
(b) Prophylaxis with beta agonist
(c) Prophylaxis
(d) Breathing exercise
27. Which is not a feature of child hood asthma:
(OPG5th/354) (AIIMS 94)
(a) Raised IgE levels
(b) History of atopic dermatitis
(c) Absence of wheezing after excercise
(d) Improves with age
28. All of the following are beneficial in the management of acute attacks of bronchial asthma in children except:
(a) High concentration of humidified oxygen
(b) Salbutamol nebuliser
(c) Intravenous hydrocortisone
(d) Sodium cromoglycate nebuliser
(OPG5th/359) (AI 91)
17 A
18 A
19 D
20 B
21 A
22 B
23 C
24 D
25 A
26 B
27 C
28 D
Cardiovascular System / 65
29. Treatment of asthma in children used in A/E:
(a) Steroids
(b) Adrenaline
(c) Aminophyllines
Status asthmaticus:
Severe persistent dyspnea
Respiratory rate of over 30 per minute
Prolonged expiration
Pulse rate > 120/minute
Pulsus paradoxus
Cyanosis; silent chest, altered consciousness and pneumothorax
Treatment:
- Humidified O2
- Nebulized b2 agonist
- Corticosteroid
- I/V theophylline
- Avoid sedatives
1st drug for child with severe bronchospasm is s.c. adrenaline
Childhood asthma is characterized by increase lgE, history of atopic dermatitis and improvement
with age.
29 D
10
Cardiovascular System
1 D
2 D
3 A
67 / Pediatric Buster
Congestive cardiac failure:
M.C. causes in infants is myocarditis and primary myocardial disease.
Depending on time on onset.
- Birth - 72 hr
Coarctation of aorta
- 1-2 mon
5 C
6 A
7 A
8 A
9 D
10 B
11 A
Cardiovascular System / 68
Mitral valve disease and chorea are more common in females whereas aortic valve involvement is
seen more in males.
- Major criteria are
- Carditis
- Arthritis
- Subcutaneous nodules
- Chorea
- Erythema marginatum
- Minor criteria are
- (Acute phase reactants) Fever
- Arthalgia precious episode
- Increased ESR and CRP
- Prololonged PR interval in ECG
- Essential criterion is
- Evidence of recent streptococcal infection as indicated by:
Increased ASO titer
Positive throat culture
Recent scarlet fever
Carditis is usually pancarditis and occurs within first two weeks of the onset of rheumatic fever.
Myocarditis is charactrised bycardiac enlargement of first heart sound; protodiastolic (S3) gallop
congestive cardiac failure and a delayed diastolic Carey Coombs murmur.
Mitral regurgitation is the commonest manifestation of acute as well as previous rheumatic
carditis.
Rheumatic arthritis is a fleetin polyarthritis involving large joints and there is no residual damage
of the joint.
Patients who have subcutaneous nodules almost always have carditis. the nodules appear around
6 week on bony prominences and are nontender.
At time of chorea signs of inflammation as elevated ESR have returned to normal.
Erythema marginatum, an early manifestation, is predominantly seen on trunk.
Patient is treated with pencillin, aspirin or steroid along with diet control and rest.
Subcutaneous nodules tends to disappear faster with used of steroid as compared to aspirin.
Patient having carditis with CHF steroid use is must.
Carditis without CHFuse either steroid or aspirin but we almost always use steroid.
If patient does not have carditis it is preferable to use aspirin.
Aspirin and steroids are supposed not to have a place in the management of chorea.
12 A
13 A
14 B
15 C
16 D
17 A
69 / Pediatric Buster
18. Bacterial endocarditis is most commonly seen in:
(OPG5th/387) (UP 94)
(a) VSD
(b) PDA
(c) ASD
(d) AS
19. Infective endocarditis not seen in:
(OPG5th/387) (AI 94)
(a) ASD
(b) TOF
(c) VSD
(d) MR
20. A wide and fixed split second heart sound occurs in:
(OPG5th/400) (AIIMS 83)
(a) Mitral stenosis
(b) Atrial septal defects
(c) VSD
(d) Coarctation of aorta
21. Which one of the following does not produce cyanosis in the first year of life :
(OPG5th/400) (AIIMS/03)
(a) Artrial septal defect
(b) Hypoplastic left heart syndrome
(c) Truncus arteriosus
(d) Double outlet
right ventricle.
22. True about ASD:
(OPG5th/400)
(a) Patient foramen ovale
(b) Increase pulmonary artey flow leads to left parasternal heave
(c) S2 widen
and variable
(d) Systolic murmur due to rapid flow of blood
ASD (Atrial septal defect)
Most common congenital cardiac anomaly recognized in adults
More common in females
Ostium secondum type (90%) more common than primum type (5%); 5% is sinus venoustype.
Congestive cardiac failure is rare
There is wide and fixed split of second heart sound
Ejection systolic murmur is heard at pull area and a delayed diastolic nurmur at lower left sternal
border.
Rheumatic mitral stenosis with an ASD is called lutembachers syndrome
Infective endocarditis is rare because of low pressure gradient.
ECG of ostium secondum type have right axis deviation with right ventricular hypertrophy
Presence of left axis deviation beyond30 suggests the ostium primum type of atrial septal defect.
ASD is repaired in 3-6 yr.
23. VSD shunt reversal is called:
(OPG5th/402) (AIIMS 89)
(a) Eisenmengers syndrome
(b) Eisenmengers complex
(c) Ebsteins anomaly
(d) None of the above
24. Natural course of events in untreated ventricular septal defects except:
(OPG5th/403) (PGI 81, DELHI 85)
(a) Spontaneous closure of defect (b) Development of pulmonary insufficiency (c) Subacute bacterial endocarditis
(d) A normal life without symptoms
25. A young boy had developed congestive failure and was found to have membranous VSD. He Spontaneously showed
improvement in his condition. The is most likely to be due to:
(OPG5th/403) (AIIMS/NOV/01)
(a) Perimembranous closure of VSD
(b) Development of AR
(c) Pulmonary vascular changes
(d) Infective
endocarditis
Ventricular Septal Defect:
Most common congenital cardiac lesion
90% are located in membranous part of ventricular septum
There is pansystolic murmur at 3rd or 4th ICS and a delayed diastolic murmur starting with 3rd
sound at apex.
Patient are born with right, ventricular hypertophy
Patient may have hyperkinetic pulmonary arterial hypertension
May develop CHF in infancy
50% close spontaneously
M.C. congenital lesion complicate by infective endocarditis
Small defects less than 0.5 cm in diameter are known as Rogers disease
Operative treatment consists closure of the VSD with use of a dacron patch.
26. Differential cyanosis and clubbing is seen in:
(a) PDA and reversal of shunt
(b) TOF
(c) VSD + ASD
18 A
19 A
20 B
21 A
22 B
23 B
24 D
25 C
Cardiovascular System / 70
27. Pure left sided failure may be seen with:
(OPG5th405) (AIIMS 78, PGI 81)
(a) ASD
(b) Aortic atresia
(c) Patent ductus arteriosus
(d) Pulmonary valvular obstruction
28. One-year-old child with PDA; which is true:
(OPG5th/405) (AIIMS 95)
(a) Symptoms similar to artopulmonary window
(b) Chances of spontaneous closure high
(c) Indomethacin
may help in closure
(d) Indocarditis is rare
29. Preterm infant with PDA is given...to effect closure of the PDA:
(OPG5th/406) (AP 88)
(a) Corticosteroids
(b) Indomethacin
(c) Aspirin
(d) Hyperbaric oxygen
30. The great danger of patients with patent ductus arteriosus is:
(OPG5th/406) (JIPMER 80)
(a) Syncope
(b) Convulsions
(c) Arrhythmia
(d) Bacterial endocarditis
Patient Ductus Arteriosus:
Communication between the pulmonary artery and the aorta just distal to left subclavian artery.
It closes functionally and anatomically soon after birth.
There is continuous harsh murmur and loud S1.
Wide pulse pressure is found along with left ventricular hypertrophy and pulmonary artery
dilatation.
The cyonosis affect the toes and lower extremities, but not the fingers.
Indomethacin, a prostaglandin synthetase inhibitor can be given orally to close the ductus.
The risk of operation increases after age of 15 yr.
Patients are considered inoperable if a right to left shunt has appeared because of pulmonary
arterial hypertension.
31. All are characteristic of Fallots tetralogy except:
(OPG5th/408) (PGI 85)
(a) Infundibular stenosis
(b) VSD
(c) Overriding aorta
(d) Left ventricular hypertrophy
32. Which one of the following congenital heart disease has cyanosis without cardiomegaly and /or congestive heart
failure?
(OPG5th/408) (UPSC 98)
(a) Transportation of great arteries
(b) Fallots tetralogy
(c) Congenital mitral regurgitation
(d) Congenital pulmonary stenosis
33. Anoxic spells in tetralogy of Fallots are precipitated by:
(OPG5th/408) (PGI/90)
(a) Fever
(b) Exertion
(c) Crying on feeding
(d) All
34. The congenital heart disease that has least chance of heart failure in infants:
(OPG5th/408) (AI 93)
(a) TOF
(b) VSD
(c) TAPVC
(d) ASD
35. Cyanosis and breathlessness in a child is seen in:
(OPG5th/408) (AIIMS 89)
(a) Transposition of great vessels
(b) Tetralogy of Fallot
(c) Ventricular septal defect
(d) Atrial septal
defect
36. Which of the following conditions does not present with CHF in neonate:
(OPG5th/408) (AIIMS 92)
(a) Total anomalous pulmonary venous connection
(b) Coarctation of aorta
(c) Tetralogy of Fallot
(d) Transposition of great vessels
37. In TOF:
(OPG5th/408) (AIIMS 98)
(a) Cynotic spells are due to arrhythmias
(b) CXR and ECG are typically normal
(c) Wide split 2nd HS
on inspiration
(d) Central cyanosis with clubbing
Tetralogy of Fallot:
Commonest cyanotic congenital heart disease in children above 2 yr.
It consists of ventricular septal defect.
- Infundibular pulmonic stenosis
- Overriding or dextroposed aorta
- Right ventricular hypertrophy
The more severe the pulmonic stenosis, the shorter the ejection systolic murmur and the more the
cyanosis.
Congestive failure never occurs in TOF except when complications are present.
Right ventricular hypertrophy is concentric without cardiac enlargement.
27 C
28 A
29 B
30 D
31 D
32 B
33 D
34 A
35 B
36 C
37 D
71 / Pediatric Buster
Patient may present with syncope; anoxic spells (paroxysmal attacks of dyspnea).
Cyanosis may present from birth.
Commonest symptoms are dysnea on exertion and excercise intolerance.
Patient assumes squatting posture on dysneic feeling.
TOF is commonest congenital lesion where squatting is noted.
Patient have cyanosis; clubbing and slightly prominent a waves.
X-ray shows boot shaped heart with upturned apex.
On X-ray there is absence of main pulmonary artery segment (coren sabot). Lung fields are oligemic.
Patient are prone to infective endocarditis; thrombotic diasthesis, paradoxical embolism and
cerebral abscess.
Operation performed are:
Blalock Taussing shunt
Potts shunt
Watersons shunt
38. A blue infant was found to have oligemic lung fields with normal sized heart. The diagnosis is:
(OPG5th/408) (AIIMS/NOV/01)
(a) Transposition of great vessels
(b) Tricuspid atresia
(c) TOF
(d) Pulmonary stenosis
39. Which one of the following is the most common cause of cyanotic congential heart disease?
(OPG5th/408) (UPSC/01)
(a) Dextrocardia
(b) Fallots tetralogy
(c) Atrial septal defect
(d) Coarctation of aorta
40. Pulmonary plethora is seen in all except:
(OPG5th/408) (PGI/89)
(a) VSD
(b) ASD
(c) Fallots tetralogy
(d) PDA
41. Blalock Taussings operation involves:
(OPG5th/409) (AIIMS 92)
(a) Right pulmonary artery with aorta
(b) Left pulmonary artery with ipsilateral subclavian A
(c) Right
pulmonary artery with descending aorta
(d) Left pulmonary artery with ascending aorta
42. Potts shunt is running from:
(OPG5th/409) (AI/2001)
(a) Right subclavian artery to right pulmonary
(b) Descending aorta to left pulmonary
(c) Left subclavian
to left pulmonary
(d) Ascending aorta to right pulmonary
43. Tricuspid atresia all are true except:
(UP/2000) (OPG5th/410) (AIIMS 98)
(a) R.V. hypoplasia
(b) Left axis deviation
(c) Split S2 in inspiration
(d) Pulmonary oligaemia
44. A child with central cyanosis and enlarged left ventricle the probable diagnosis is:
(OPG5th/410) (AI 91)
(a) Tricuspid atresia (b) Eiesenmengers syndrome (c) Tetralogy of Fallot (d) Anomalous pulmonary artery
45. Left axis deviation with left ventricular hypertrophy is seen:
(OPG5th/410) (UP 96)
(a) Tricuspid atresia
(b) Tetralogy of Fallot
(c) Coarctation of aorta
(d) Ventricular septal defect
Tricuspid atresia:
Congenital absence of tricuspid valve
Right venticle hypolastic and pulmonary-stenosis
Patent foramen ovale or atrial septal defect
ECG shows left axis deviation and left ventricular hypertrophy
X-ray shows left ventricular cofiguration
Patient are cyahotic from birth
Anoxic spells and squatting may be present
Fontan operation is done at 4 yr. or more (Atriopulmonary connection)
46. True of tricuspid atresia are A/E:
(OPG5th/410) (UP 97)
(a) Right axis deviation
(b) Left axis deviation
(c) Left ventricular hypertrophy
(d) Severe cyanosis
47. The best position for examination of cardiac murmurs in a child is:
(KERALA 90)
(a) Sitting
(b) Standing
(c) Right lateral
(d) Recumbent
48. Which of the following congenital cyanotic disease presents with gallop rhythm, left parasternal murmur, pericardial friction rub:
(OPG5th/411) (ORISSA/98)
(a) Ebstein anomaly
(b) TA PVC
(c) TGA
(d) Eissenmengers complex
38 C
39 B
40 C
41 B
42 B
43 C
44 A
45 A
46 A
47 D
48 A
Cardiovascular System / 72
Ebstein anomaly:
Diminished pulmonary blood flow resulting from an abnormality of the tricuspid valve.
Cyanosis; effort intolerance, fatigue and paroxysmal attacks of tachycardia
Clubbing is present.
Systolic thrill may be palpable at the left sternal border.
Triple or quadruple sounds heard along with a loud pansystolic murmur.
ECG shows P pulmonale and P mitrale with RBB
WPW type B conduction abnormality may be seen in ECG
There is right ventricular enlargement
Intracardiac ECG is diagnostic of Ebstein anomaly
49. In transposition of great vessels, all are true except:
(OPG5th/413) (AP 97)
(a) Aorta arises from the right ventricle
(b) Mitral valve is continuous with the aortic valve
(c) Causes
jaundice immediately after birth
(d) None of the above
50. Emergency treatment for TGV:
(OPG5th/413) (TN/99)
(a) Balloon septostomy
(b) Oxygen
(c) Ventilation
(d) Digoxin
51. Congestive heart failure, LVH and systolic heart murmur in a 29-day-old child would suggest:
(a) TGA
(b) VSD
(c) Rheumatic fever
(d) TOF
(OPG/5th413) (AIIMS/2K)
52. A neonate has central cyanosis and short systolic murmur on the 2nd day of birth. The diagnosis is:
(a) TGV
(b) TOF
(c) VSD
(d) ASD
(OPG/ 5th413) (AIIMS /MAY/01)
53. A five-day-old. Full term male infant was severely cyanotic at birth. Prostaglandin E was administered initially
and later balloon atrial septostomy was done which showed improvement in oxygenation. The most likely
diagnosis of this infant is:
(OPG5th/413)(AI/04)
(a) Tetralogy of Fallot
(b) Transposition of great vessels
(c) Truncus arteriosus
(d) Tricuspid atresia
Transposition of Great Vessels:
Particulary common in offespring of diabetic mother
Patent ductus is present
There is right ventricular hypertrophy
CHF around 4-10 week of age
On X-ray cardiac silhouette is like egg on side
Right upper lung fields are plethoric
Thymic shadow absent
Ballon septostomy helpful only upto 6-12 wk
< 2 wk-Jatenes switch operation
Other operation of complete TGA is mustard or senning operation
Taussing-Bing anomaly is a variant where aorta comes off the right ventricle and there is a VSD
directly beneath an overriding pulmonary artery.
Associated anomalies with complete TGA are VSD with or without PS, it sided ebstein anomaly
of TR and arterioventricular conduction abnormalities.
54. A neonate is found to have central cyanosis and a cardiac murmur at 30 hours of age. The most probable diagnosis
is.
(OPG5th/413)(UPSC/03)
(a) Endocardial cushion defect
(b) Ventricular septal defect
(c) Transposition of great vessels
(d) Patent
ductus arteriosus
55. True in total anomalous pulmonary connection are A/E:
(OPG5th/414) (AI 97)
(a) The total pulmonary venous blood reaching the right atrium
(b) Always associated with VSD
(c) The oxygen saturation of the blood in the pulmonary artery is higher to that in the aorta
(d) Infracardiac type is always obstructive
56. Figure of eight appearance is seen in:
(OPG5th/414) (AI/2000)
(a) Partial anomalous pulmonary vessels
(b) Total anomalous pulmonary vessels
(c) Truncus arteriosus
(d) Transposition of great vessels
49 C
50 A
51 A
52 A
53 B
54 C
55 B
56 B
73 / Pediatric Buster
57. Neonate has recurrent abdominal pain and diaphoresis on feeding, with murmur, which may lead to MI, diagnosis
is:
(OPG5th/414) (AI/2001)
(a) ASD
(b) VSD
(c) TOF
(d) Coronary anomalous pulmonany venous connection
TAPVC:
Nonobstructive type is more common than obstructive type.
ECG shows right axis deviation and right ventricular hypertrophy
In obstructive type P pulmonale is common.
X-ray showssnowman or figure of 8 configuration in the supracardiac TAPVC.
Obstructive type have ground glass appearance in the lungs.
58. Infantile hypercalcaemia syndrome is most frequently associated with: (OPG5th/416) (AIIMS 80, AIIMS 84)
(a) ASD
(b) VSD
(c) Patent ductus arteriosus
(d) Supravalvular aortic stenosis
59. Sequence of development of Eisenmengers syndrome:
(OPG5th/416) (AIIMS/2K)
(a) Left to right shuntright ventricular hypertrophyright to left shuntpulmonary hypertension
(b) Left
to right shuntpulmonary hypertensionright ventricular hypertrophyright to left shunt
(c) Left to right
shuntright ventricular hypertrophypulmonary hypertensionright to left shunt
(d) Right to left shunt
left ventricular hypertrophypulmonary hypertensionleft to right shunt
60. Paradoxically split second heart sound signifies severe:
(OPG5th/417) (AIIMS 83)
(a) Pulmonary stenosis
(b) Mitral stenosis
(c) Aortic stenosis
(d) Tricuspid stenosis
61. Most common cause of death from aortic stenosis in children is:
(OPG5th/418) (JIPMER 81, PGI 83)
(a) Myocardial infarction
(b) Pulmonary oedema
(c) Atrial flutter
(d) Ischaemic heart disease with
ventricular fibrillation
62. Rib notching of 4-9th ribs with double bulging is seen in:
(OPG5th/419) (AI 98)
(a) Aortic aneurysm
(b) Aortic dissection
(c) Coarctation of aorta
(d) Diaphragmatic hernia
Coarctation of Aorta
Located at junction of arch with the descending aorta distal to left subclavian artery near insertion
of ligamentum arteriosus.
Males are more affected; although females with Turners syndrome frequently have coarctation.
Accompanying anomalies may be PDA; bicuspid aortic valve; congenital aortic stenosis; ASD; VSD;
MR and Berry aneurysm of the circle of willis
Coarctation may be preductal or postductal
Only symptoms are intermittent claudication, pain and weakness of legs and dyspnea on running.
Femorals are impalpable w.r.t. strong brachial ones.
Barium swallow shows characteristic E. sign.
X-ray shows characteristic notching of the lower border of the ribs which tend to appear beyond the
age of 10 years
Deaths are mostly due to CHF; other causes may be intracranial hemorrhage; dissection of aorta,
hypertension, cerebral aneurysm, LVH, rupture aorta or I.E.
63. An infant with severe dehydration secondary to diarrhoea suddenly presents with protein and blood in urine. The
most probable diagnosis is:
(PGI 78, UPSC 89)
(a) Renal vein thrombosis
(b) Pyelonephritis
(c) Acute glomerulonephritis
(d) Lower nephrosis
64. Rib notching is present in:
(OPG5th/419) (UP96)
(a) Coarctation of aorta
(b) Ventricular septal defect
(c) Atrial septal defect
(d) Tetralogy of Fallot
65. Myocarditis can be caused by A/E:
(OPG5th/423) (PGI 88)
(a) Pertussis
(b) Measles
(c) Diphtheria
(d) Scorpion sting
66. Infantile myocarditis and pericarditis is due to:
(OPG5th/423) (TN/99)
(a) Coxsackie A
(b) Coxsackie B
(c) Mumps
(d) Pox virus
67. Sustained severe hypertension in children is commonly suggestive of:
(OPG5th/432) (AI 95, 97)
(a) Coractation of aorta
(b) Pheochromocytoma
(c) Renal parenchymal disease
(d) Drug induced
57 D
58 D
59 B
60 C
61 D
62 C
63 A
64 A
65 B
66 B
67 C
74 / Pediatric Buster
68. The average BP of a 1-year-old child is:
(OPG5th/432) (PGI 78, AMC 81)
(a) 120/80
(b) 75/50
(c) 95/65
(d) 60/30
69. The most common cause of systemic hypertension in children is:
(OPG5th/433) (AIIMS 89)
(a) Coarctation of aorta
(b) Acute glomerulonephritis
(c) Nephrotic syndrome
(d) Congenital adrenal hyperplasia
Hypertension in children:
Average pressure in children from 1-8 years in 95 5 systolic and 65 5 diastolic
Chronic renal disease especially chronic glomerulonephritis is most common cause of hypertension
in children.
2% had coartation and pheochromocytoma as cause of hypertension
Weight loss is an important finding in pheochromocytoma.
70. The most common cause of secondary hypertension in children is:
(OPG5th/433) (PGI
(a) Renal artery stenosis
(b) Renal disease
(c) Systemic vasculitis
(d) Adrenal tumours
71. The most common cause of hypertension in the newborn is:
(NEL17th/1593) (AIIMS
(a) Polycystic kidney
(b) Neuroblastoma
(c) Anaemia
(d) Coarctation of aorta
72. What is true of hydralazine therapy in hypertension:
(NEL 17th/1597) (PGI 80, AIIMS
(a) Drowsiness
(b) Lupus syndrome
(c) Positive Coombs test
(d) Syncope
73. The treatment for symptomatic sick sinus syndrome is:
(NEL 17th/1563) (AIIMS
(a) Atropine only
(b) Only bed rest
(c) Pacemaker implantation
(d) Propranolol only
74. The most common cause of painful pericarditis is:
(NEL17th/1580) (AIIMS 80 BIHAR
(a) Viral
(b) Tuberculous
(c) Uraemia
(d) All of these
68 C
69 B
70 A
71 B
72 B
73 C
74 A
93)
79)
87)
81)
91)
11
Kidneys develop between 3-4 weeks of fetal age and by 22 wk medulla and cortex are clearly
demarcated.
Nephron induction ceases at about 26 wk of gestation; full complement of nephrons is present by
36 wk.
Urine formation is believed to start around 10th week of gestation.
GFR is about 1/3rd of that of adult, i.e. 30 ml/min in 1st wk of life;
2/3 rd of that of adult i.e. 60ml/min in 2nd-3rd month of life;
100-110 ml/min/1.73 m by the age of 2 yr;
Compared to adults, sodium reabsorption in the newborn is very low. Therefore pH of urine of a
newborn is inappropriately high for degree of acidemia.
A healthy infant excretes 15-30 ml/kg/24 hr of urine on the first two days of life and 25-120
ml/kg/24hr during the next 4 weeks.
Oliguria is defined as urine volume of less than 1 ml/kg/hr.
An infant can concentrate his urine to a maximum of 700-800 mOsm/kg whereas the older child
can achieve 1200-1400 mOsm/kg.
IVP- dose of contrast depends on the body wt.
Causes of mild proteinuria are
UTI
Hydronephrosis
Renal T.B.
Absencce of proteinuria does not rule out congenital anomalies of urinary tract.
Microscopic hematuria is found in:
- Idiopathic hypercalciuria.
- Benign familial haematuria
- IgA nephropathy; Alport syndrome
- Membranoproliferative glomerulonephritis.
Isolated proteinuria is found in heavy exercise; fever or dehydration
Asymptomatic proteinuria
- Orthostatic proteinuria
- Chronic glomerular disease
- Reflux nephropathy
- Renal hypoplasia
- Renal tubular disorders
Posterior urethral valves, occurring in males; are recognised cause of Potter facies.
U/L renal agenesis should be suspected in newborns with single umbilical artery.
2 B
3 B
4 C
5 C
6 C
7 A
8 C
9 A
77 / Pediatric Buster
10. A 10-year-old boy with hematuria-5 days, oliguria-1 day, BP 170/120, pedal edema present; possible diagnosis:
(a) Nephrotic disease
(b) AC GN
(c) HUS
(d) Renal vein thrombosis
(OPG5th/449) (PGI 2000)
11. Diarrhea-related hemolytic-uremic syndrome is characterized by all except:
(OPG5th/449)(UPSC/02)
(a) Acute renal failure
(b) Coombs positive hemolytic anemia
(c) Thrombocytopenia
(d) Neutrophilic
leukocytosis
12. In Shigella dysentery associated haemolytic-uraemic syndrome the false statement is:
(OPG5th/449)
(a) Leucocytosis
(b) Neurological abnormalities
(c) Hepatic failure
(d) Thrombotic angiopathy
13. In nephrotic syndrome the essential feature is:
(OPG5th/450) (AIIMS 83)
(a) Proteinuria
(b) Hypoalbuminaemia
(c) Hyperlipidaemia
(d) Oedema
Nephrotic Syndrome
Characterized by
Massive proteinuria
Hypoalbuminemia and edema
Also accompaniments are hyperlipidemia and hypertension.
Heavy proteinuria is the basic abnormality.
Hyperlipidemia is because of increased hepatic synthesis of beta lipoproteins and decreased
lipoprotein lipase activity.
During childhood, it is mostly idiopathic.
Minimal lesion (lipid nephrosis) accounts for 85 percent cases of nephrotic syndrome in children.
There is selective proteinuria of albumin but besides this urinary loss also accounts for loss of
- Throxine binding globulin
- Cholecalciferol binding protein leading to secondary hyperparathyroidism
- Transferrin (Iron resistant anemia)
- loss of metal binding protein (zinc and copper deficiency)
There is also hypercoagulable state due to deficiency of antithrombin III and reduced activity of
protein S or protein C and hyperfibrinogen.
Some have severe lgG deficiency.
Electrophoresis reveals, diminished albumin with increase of and globulins
14. Nephrotic syndrome in a child, all are seen except:
(OPG5th/450) (JIPMER)
(a) Hypoproteinaemia
(b) Hyperlipidaemia
(c) Uraemia
(d) Increased alpha globulins
15. Which of the following is not seen in nephrotic syndrome?
(OPG5th/450) (TN 95)
(a) Hematuria
(b) Massive proteinuria
(c) Hypoalbuminaemia
(d) Hypercholesterolaemia
16. The most common type of renal lesion in children is:
(OPG5th/450) (TN 95)
(a) Lipoid nephrosis
(b) Membrane proliferative glomerulonephritis
(c) Focal glomerulonephritis
(d) Diffuse glomerulosclerosis
17. A 5-year-old child is suffering from nephrotic syndrome is well responding to steroid therapy. What would be the
finding on light microscopy:
(OPG5th/451) (AI/2001)
(a) No finding
(b) Basement membrane thickening
(c) Podocyte lesions
(d) Fusion of foot process
18. Best response to steroids is observed with:
(OPG5th/451) (AI 96)
(a) Focal glomerulonephritis
(b) Lipoid nephrosis
(c) Membranous GN
(d) Membranoproliferative GN
Lipoid nephrosis:
Diffuse epithelial foot process effacement; serum complements are normal.
There is abnormality of T lymphocyte function with deposits of IgM and C3.
Besides anasarca; ascites; hydrothorax and hydrococele is also present.
Heavy proteinuria; hyaline and granular casts, low albumin with rise in 2 globulin and Normal
Blood urea and creatinine values are often encountered.
ARF is rare.
Peritonitis (Pneumococcal) is usually the complication of the NS.
Thromboembolic manifestation may occur but renal vein thrombosis is rare.
10 C
11 A
12 C
13 A
14 C
15 A
16 A
17 A
18 B
19 D
20 B
21 C
22 D
23 B
24 B
25 B
26 A
27 A
79 / Pediatric Buster
There are 4 types; type 3 is rare and type 4 is associated with hyporenineic hypoaldosteronism with
hyperkalemia or tubular hyperresponsiveness to mineralocorticoids.
Type I (Distal RTA)
Type II (Proximal RTA)
1. Minimum urine pH > 5.5
< 5.5
2. % filtered HCO3 excreted < 10
> 15
3. Serum potassiumlow
Low
4. Fanconis s syndromeno
Yes
5. Daily acid excretionlow
Normal
6. Stones/nephrocalcinosisseen
Absent
7. Daily HCO3 replacement < 4 mmol/kg
> 4 mmol/kg
Fanconis syndrome: Phosphates, amino acids, protein and glucose not reabsorbed through renal
tubules. Syndrome includes cystinosis, tyrosinosis and Lowes syndrome; Systemic metabolic acidosis,
hyperchloremia hypokalemia and hypophosphatemia seen.
Tumour induced osteomalacia
Renal tubular acidosis
Chronic use of antacids.
29. In proximal renal tubular acidosis is the most important investigation is:
(OPG5th/465) (AIIMS 89)
(a) Vitamin D resistant rickets
(b) Dehydration and fever
(c) Nephrocalcinosis
(d) Bicarbonate loss
30. A 10-month-old boy, weighing 3 kg has polyuria, polydipsia and delayed motor milestones. Investigations show
blood levels of creatinine 0.5 mg/dl, potassium 3 mEq/L, sodium 125 mEq/L, chloride 88 mEq/L, calcium 8.8 mg/
dl, pH 7.46 and bicarbonate 26 mEq/L. Ultrasonography shows medullary nephrocalcinosis. The most likely
diagnosis is:
(OPG5th/466) (AIIMS/03)
(a) Renal tubular acidosis
(b) Diabetes insipidus
(c) Bartters syndrome
(d) Pseudohypoaldosteronism
Bartters syndrome: consist of
Hypokalemia (urinary K+ excretion)
Increased plasma renin
Hyperaldosteronism; metabolic alkalosis and normal blood pressure
Hypomagnesemia and elevated PGF2 is present
Weakness or periodic paralysis and polyuria occur
Main defect is defective NaCl reabsorption from thick ascending limb of Henles loop (main site for
magnesium absorption).
It may be mimicked by Mg++ deficiency; diuretic use or vomiting.
Treatment is by aldosterone antagonists to prevent potassium loss and beta blockers may lower
renin production use of PG synthetase inhibitors as indomethacin is beneficial in it.
31. In a child with diabetes insipidus; the osmolalities are:
(a) Serum-300; Urine-50
(b) Serum-30; Urine-290
(c) Serum-50; Urine-500
(OPG5th/466) (AI/2000)
(d) Serum-260; Urine-30
28 D
29 D
30 C
31 A
32 D
80 / Pediatric Buster
33. Most common cause of urine obstruction in a boy:
(OPG5th/467) (AI/2001)
(a) Anterior urethral valves
(b) Posterior urethral valves
(c) Stone in urethra
(d) Duplication of renal
pelvis
34. Which one of the following is the most common cause of abdominal mass in neonates? (OPG5th/467) (AI/03)
(a) Neuroblastoma
(b) Wilms tumor
(c) Distended bladder
(d) Multicystic dysplastic kidneys
35. In a male fetus,ultrasound identification of bilateral hydronephrosis and bladder dilatation is diagnostic of:
(OPG5th/467)(UPSC/02)
(a) Polycystic kidney disease
(b) Multicystic dysplastic kidney
(c) Uteropelvic junction obstruction
(d) Posterior urethral valve syndrome
36. One-year-old male child presented with poor urinary stream since birth. The investigation of choice for evaluation
is:
(OPG5th/467) (AIIMS/03)
(a) Voiding cystourethrography (VCUG)
(b) USG bladder
(c) Intravenous urography
(d) Uroflowmetry
37. A 13-year-old boy is referred for evaluation of nocturnal enuresis and short stature. His blood pressure is normal.
The hemoglobin level is 8 g/dl, urea 112 mg/dl, creatinine 6 mg/dl, sodium 7 mg/ dl, phosphate 6 mg/dl and
alkaline phosphates 300 U/l. Urinalysis shows trace proteinurina with hyaline casts. Ultrasound shows bilateral
small kidneys and the micturating cystourethrogram is normal. The most likely diagnosis is
(AIIMS/03)
(a) Alports syndrome
(b) Medullary sponge kidney
(c) Chronic glomerulonephtritis
(d) Nephronophthisis.
38. Which one of the following statements is false with regard to pyuria in children?
(AI/03)
(a) Presence of more than 5WBC/hpf for girls and more than 3 WBC/hpf for boys. (b) Infection can occur without
pyuria
(c) Pyuria may be present without Urinary tract infection
(d) Isolated pyuria is neither confirmatory
nor diagnostic for UTI.
39. Which one of the following statements is false with regard to Xanthogranulomatous pyelonephritis in children:
(AI/03)
(a) Often affects those younger than 8 yrs of age
(b) It affects the kidney focally more frequently than diffusely
(c) Boys are affected more frequently
(d) Clinical presentation in children is same as in adults.
40. Read the passage carefully and answer the following three questions. Passage: One and a half-year-old girl child,
fed mostly on dilute cows milk,develops diarrhea which persists for 10 days. This was followed by swelling on
feet. The likely diagnosis is
(OPG5th/460)(UPSC/02)
(a) Kwashiokor
(b) Acute renal failure
(c) Congestive cardiac failure
(d) Indian childhood cirrhosis
41. One life-threatening complication of this condition is:
(OPG5th/460)(UPSC/02)
(a) Hyperkalemia
(b) Hypernatremia
(c) Metabolic acidosis
(d) Hypoglycaemia
42. The most important aspect of management is:
(OPG5th/460)(UPSC/02)
(a) Diuretics
(b) Fluid restriction
(c) Diet with adequate calories and protein
(d) Potassium restriction
33 B
34 D
35 D
36 A
37 D
38 D
39 B
40 B
41 A
42 D
12
Hormonal
Hypothalamic
2 D
3 C
4 C
5 A
6 C
Acquired Hypothyroidism:
Growth retardation
Ratio of upper and lower segment is disproportionate
Delayed dental development
Delayed puberty with galactorhoea and sexual precocity and high FSH and LH
Enlarged sella
Pseudotumour cerebri
Gross mental retardation is not usually present.
Goiter is freqently encountered.
7. Which of the following X-ray findings in a newborn infant would be most suggestive of hypothyroidism?
(a) Osteoporosis
(b) Epiphyseal dysgenesis
(c) Prominent thymic shadow (OPG5th/483)(AIIMS 78, 80, 81)
(d) Absence of ossification of hamate bone
8. An 11-year-old child, who is mentally and physically retarded with delayed bone age had calcifications in the
epiphyses on X-ray. The probable diagnosis:
(OPG5th/483) (AIIMS/NOV/01)
(a) Hypopituitarism
(b) Hypothyroidism
(c) Malnutrition
(d) Hypoadrenalism
9. Which of the following is a feature of hypothyroid cretinism:
(OPG5th/483) (AIIMS 92)
(a) Goitre with focal seizures
(b) Blindness with mental retardation
(c) Deafness with facial palsy
(d) Goitre with facial palsy
10. Infant with no social smile, no eyebrows, protruded tongue. Diagnosis is:
(OPG5th/483) (TN/99)
(a) Cretinism
(b) Downs syndrome
(c) Mucopolysaccharidosis
(d) Rickets
11. Which of the following is true regarding cretinism:
(OPG5th/483) (AI/2001)
(a) Short limbs compared to trunk
(b) Proportionate shortening
(c) Short limb and short stature
(d)
Short limb long stature
12. Congenital hypothyroidism is diagnosed most early by:
(OPG5th/484) (AIIMS 90)
(d) PBI
(a) RAIU
(b) TSH
(c) T3 leveis
13. A 6-month-old infant is brought with a history of constipation and excessive sleepiness. On examination, he is
lethargic, has periorbital puffiness, large tongue and umbilical hernia . The investigation which will help to
diagnose this condition is:
(OPG5th/484) (UPSC/01)
(a) T4 TSH assay
(b) Karyotyping
(c) Rectal mucosal biopsy
(d) Knee X-ray
14. In neonatal screening programme for detection of congenital hypothyroidism, the ideal place and time to collect
the blood sample for TSH estimation is:
(OPG5th/484) (AIIMS/03)
(a) Cord blood at time of birth
(b) Heal pad blood at time of birth
(c) Heal pad blood on 4th day of birth
(d) Peripheal venous blood on 28th day
15. Ambiguous genitalia is not seen in:
(OPG5th/488) (KERALA 94)
(a) Gonadal agenesis
(b) Gonadal dysgenesis
(c) Hermaphroditism
(d) Super female
Ambiguous Genitalia : Discrepancy between external and internal genitalia.
Chromosormal Anamolies - 45 X 0/46 XY
XXY (Klinefelter syndrome)
Pseudohermaphroditism
- Female
- Exposure to androgen as maternal mediation or congenital
adrenal hyperplasia
7 B
8 B
9 C
10 A
11 C
12 B
13 A
14 A
15 D
83 / Pediatric Buster
Male
20. An 8-day old breastfed baby presents with vomiting, poor feeding and loose stools. On examination the heart rate
is 190 minute, blood pressure 50/30 mmHg, respiratory rate 72 breaths/minute and capillary refill time of 4
seconds. Investigations show hemoglobin level of 15 g/dl, Na 120 mEq/L, K 6.8 mEq/L, Cl 81 mEq/L bicarbonate
15 mEq/L, urea 30 mg/dl and creatinine 0.6 mg/dl. The most likey diagnosis is:
(OPG5th/490) (AIIMS/03)
(a) Congenital adrenal hyperplasia
(b) Acute tubular necrosis.
(c) Congenital hypertrophic pyloric stenosis
(d) Galactosemia.
21. The most common enzymatic defect in congenital adrenal hyperplasia (adrenogenital syndrome) is:
(OPG5th/489)(UPSC/03)
(a) 11-hydroxylase deficiency
(b) 17-hydroxylase deficiency
(c) 21-hydroxylase deficiency
(d) 3-beta
dehydrogenase deficiency
22. In the female, congenital adrenal hyperplasia causes:
(OPG5th/490) (AIIMS 83)
(a) Infant hercules
(b) Macrogenitosomia praecox
(c) Female pseudohermaphrodites
(d) None
16 A
17 C
18 A
19 A
20 A
21 C
22 C
23 A
24 A
25 B
26 B
27 B
28 B
29 B
30 C
31 D
32 B
33 A
If urinary 17-ketosteroids are elevated and tests are small; congenital adrenal hyperplasia is most
likely diagnosis.
Highly potent long acting analogues of LHRH are being used for management of true precocious
puberty.
Medroxyprogesterone acetate for peripheral precocious puberty.
34. The diagnosis is when familial polyostosis, precocious puberty and pigmentation is:
(OPG5th/491) (AI 93)
(a) Tuberous sclerosis
(b) McCune-Albright syndrome
(c) Klinefelters syndrome
(d) SLE
35. Delayed puberty in children are associated with:
(OPG5th/492) (PGI 2K)
(a) Poliomyelitis
(b) Hypopituitarism
(c) Hypothyroidism
(d) Anorexia nervosa
Delayed Puberty:
Sexual development (increase in the size of testes) are absent by age of 14 years in boys and by
13 years (breast budding) in girls.
34 B
35 D
13
2 E
3 A
4 D
87 / Pediatric Buster
6 A
7 B
8 A
9 A
10 B
11 C
12 B
13 B
14 C
Common bacterial
pathogens
Initial antibiotic
regimens
Newborn
Escherichia coli
Klebsiella pneumoniae
Listeria monocytogenes
Enterococcus sp.
Salmonella sp.
H.influenzae
S.pneumoniae
Group B Streptococcus
Listeria monocytogenes
H.influenzae
S. pneumoniae
N. meningitidis
Ampicillin plus
aminoglyccoside
or cefotaxime
4-12 weeks
15 A
16 C
17 C
18 B
19 B
20 C
21 C
22 C
23 A
24 A
89 / Pediatric Buster
S/S in neonates; vacant stare; persistent vomiting with fever, refusal to suck after a normal feeding
pattern has been established, poor tone poor cry; circulatory collapse, Tremors convulsion and
neurological deficit.
S/S in older children; irritability; photophobia, high fever cheyne stokes; seizures; generalized flexed
posture, hypertonia, marked neck rigidity; Kernigs sign positive; and Brudzinski sign positive.
Pneumococcal meningitis:
Common cause of septic meningitis at all ages except for first few weeks.
Follows otitis media, sinusitis, pneumonia or head inhury
Subdural effusion is a usual complications.
26. Common organism causing meningitis in 6 months to 2 years of age:
(a) Streptococcus
(b) H. influenzae
(c) Neisseriae
(d) Staphylococcus
Haemophilus meningitis:
Usually type B organisms
Subdural effusions follow the initial illness.
Convulsions are common.
Residual auditory deficit is a common complications.
Staphylococcal meningitis:
Occurs usually in the newborn baby with associated umbilical infection; pyoderma or septicemia.
27. Aseptic meningitis is mostly caused by:
(OPG5th/517) (UPSC 85, JIPMER 87)
(a) Adenovirus
(b) Enterovirus
(c) Arbovirus
(d) Herpes virus
28. The most common presentation of neonatal meningitis is:
(OPG5th/517) (ORISSA 98)
(a) Bulging fontanels
(b) Nuchal rigidity
(c) Poor feeding
(d) Convulsion
29. The most common complication following meningococcal meningitis is:
(OPG5th/518) (AIIMS 85)
(a) Subdural effusion
(b) Hydrocephalus
(c) Arthritis
(d) Waterhouse-Friderichsen syndrome
Meningococcal meningitis:
Epidemics are caused by sero type A organism
Carrier state is common in children.
Children show petechial hemorrhages.
Meningococcemia may be associated with acute fulminating illness with adrenal insufficiently;
hypotension and shock. This is MC complication called Waterhouse-Friderichsen syndrome.
30. The most common complication of Haemophilus influenzae meningitis is:
(OPG5th/518)
(a) Arthritis
(b) Bronchopneumonia
(c) Otitis media
(d) Subdural effusion
31. Subdural effusion is most commonly associated with meningitis caused by:
(OPG5th/518) (AI 89)
(a) Haemophilus influenzae
(b) Mycobacterium tuberculosis
(c) Neisseria meningitis
(d) E. coli.
32. A patient with meningitis has the CSF finding of reduction in glucose, increase in protein and moderate to low
chlorides and rise in polymorphs; the diagnosis is:
(OPG5th/518) (AIIMS/99)
(a) Meningococcal meningitis
(b) TBM
(c) Viral meningitis
(d) Fungal meningitis
33. In meningitis in children, most common complication is:
(OPG5th/518) (AIIMS 94)
(a) MR
(b) Seizures
(c) Hearing loss
(d) Hydrocephalus
Complications of meningitis:
1. Neurological deficit
2. Mental deterioration
25 A
26 B
27 B
28 C
29 D
30 D
31 A
32 A
33 B
Cerebritis
Brain abscess
Focal fits
Deafness
Subdural empyema (H. influenzae or Strepto pneumoniae)
Internal hydrocephalus
Spinal cord compression
35 A
36 B
37 B
38 A
39 C
40 C
41 D
42 A
43 A
91 / Pediatric Buster
44 D
45 A
46 D
47 B
48 C
49 D
50 C
52 D
53 B
54 A
55 B
93 / Pediatric Buster
Hydrocephalus:
Characterized by increased CSF pressure
M.C. cause of obstructive hydrocephalus in a child is aqueduct stenosis.
Other causes may be Dandy Walker syndrome (posterior fossa cyst continuous with fourth ventricle)
and Arnold-Chiari syndrome; intrauterine infections as rubela, CMV and toxoplasmosis.
M.C. cause of acquired hydrocephalus is post-inflammatory stricture after bacterial meningitis.
Other acquired causes areposterior fossa tumors and intracranial hemorrages.
C/F
Headache
Papilloedema
Nausea
Ophthalmoplegia
Vomiting
Irritability
Impaired upward gaze.
Normal pressure hydrocephalus:
D - Dementia
I - Incontinence
A - Ataxia
L - Low IQ
E - Extensor plantar
D - Dysphagia
Diagnosis:
Increase in the head circumference in the first 3 months of life by more than 1 cm every fortnight.
Excessive molding of skull bones during labor.
Widening os squamoparietal sutures.
Treatment is repeated lumbar puncture.
or
Shunt operation with or without acetazolamide
56. Common cause of hydrocephalus in children is:
(OPG5th/529) (AI 98, 96)
(a) Congenital anomaly
(b) Perinatal injury
(c) Postinflammatory stricture
(d) Brain tumours
57. Most common cause of obstructive hydrocephalus in a child is:
(OPG5th/529) (JIPMER 93)
(a) Dandy-Walker syndrome
(b) Arnold-Chiari malformations
(c) Aqueductal stenosis
(d) Meningitis
58. Neonatal hydrocephalus is seen in A/E:
(OPG5th/529) (UP 94)
(a) Intraventricular haemorrhage
(b) Aqueductal stenosis
(c) Brain tumour
(d) AIDS
59. All of the following cause congenital hydrocephalus except:
(OPG5th/529)
(DELHI/94)
(a) Toxoplasma
(b) TBM
(c) Rubella
(d) Arnold-Chiari syndrome
60. The most common cause of hydrocephalus in children is:
(OPG5th/529) (AI 96)
(a) Posterior fossa tumours
(b) Congenital malformation
(c) Complication of meningitis
(d) Rupture of intracranial aneurysm
61. Dandy-Walker syndrome is due to obstruction of:
(OPG5th/530) (AIIMS 80, JIPMER 91, 92)
(a) Foramen of Monro
(b) Foramen of Morgagni
(c) Foramen of Magendie and Luschka
(d) Arachnoid granulations
62. All of the following are neural tube defects except:
(OPG5th/531) (AI/04)
(a) Myelomeningocele
(b) Anencephaly
(c) Encephalocele
(d) Holoprosencephaly
NEURAL TUBE DEFECTS include
1. Spina bifida (meningocele, meningomyelocele, spina bifida occulta)
2. Anencephaly
3. Encephalocele
4. Craniorhachischisis
5. Iniencephaly
63. In unexplained ataxia in a child ...poisoning is a possibility:
(a) Arsenic
(b) Lead
(c) Salicylate
(d) Mercury
56 C
57 C
58 D
59 B
60 C
61 C
62 D
63 B
Cerebral Palsy
Nonprogressives neuromuscular disorders of cerebral origin.
Not a familial disease
Incidence 2/100 live birth may be due to birth trauma, anoxia, hypoglycemia or infections.
Child posture and movement are permanently impaired.
Intellectual function may or may not be affected.
Pyramidal (Spastic type) is M.C.; characteristics are
- Abnormal neonatal reflexes (prolonged)
- Hyperexcitable and firm grasp reflex
- Inability to flex knees or limbs
- Opisthotonus
- Stretch tendon reflex brisk
- Diplegia
In atonic type
- Despite hypotonia
- Tendon reflexes are brisk and Babinski response is +ve
Kernicterus is associated with extrapyramidal athetoid type.
These children may associate dental defects as enamel hypoplasia.
65. Cerebral palsy which manifests after the age of one year is most often:
(OPG5th/540) (JIPMER 93)
(a) Ataxic
(b) Spastic
(c) Diplegia
(d) Mixed
66. Cerebral palsy is defined as a:
(OPG5th/540) (JIPMER 80, UPSC 87)
(a) Nonprogressive neuromuscular disorder of cerebral origin (b) Progressive neuromuscular disorder of cerebral
origin (c) Nonprogressive neuromuscular disorder of peripheral origin (d) Progressive neuromuscular disorder
of peripheral origin
67. Preventable cause of mental retardation is:
(OPG5th/543) (AI 95)
(a) Down syndrome
(b) Phenylketonuria
(c) Cretinism
(d) Cerebral palsy
65 B
66 A
67 C
68 B
69 B
95 / Pediatric Buster
Tuberous Sclerosis complex: Autosomal dominant
Cardinal features are skin lesions; convulsions and mental retardation; may affect heart; kidney,
eye, bone and lung.
Tubers may be (-nt upto 3-4 yr) in subependymal region; calcify and project in ventricles as candle
dripping.
Hydrocephalus is seen
MRI is diagnostic
Patients develop infantile spasms and hypsarrhthmic EEG and later develop myoclonic epilepsy.
Brain tumours are less but chances of astrocytoma is there
Skin lesions areAsh
Leaf macules (early cutaneous sign)
Adenoma sebaceum
Shagreen patch (lumbosacral)
50% have rhabdomyoma of heart which resolve spontaneously.
70. Sphenoid wing dysplasia is seen in:
(OPG5th/546) (JIPMER/2K)
(a) Von Hippel Lindaus disease (b) Neurofibromatosis (c) Sturge Weber syndrome (d) Bournvilles disease
71. True about Duchennes muscular dystrophy are A/E:
(OPG5th/547) (AI 96)
(a) X-linked recessive
(b) Cardiomyopathy
(c) Respiratory failure in thud
(d) Congenital hypotonia
Duchennes type pseudohypertrophic muscular dystrophy
X-linked recessive inheritance with a high mutation frequency.
One-third of new cases have no previous family history.
The muscle fibres vary in size; clusters of basopholic fibres with vesicular nuclei; generalized fibrosis
focal areas of necrosis, and phagocytosis are observed.
Symmetric weakness; initially pelvifemoral later weakness in leg shoulder girdle and then trunk
muscles, pseudohypertrophy of calves, reduced intelligence and cardiac involvement.
Gowers sign is observed. Waddling gait with a compensatory lumbar lordosis.
Most patients die in the second decade due to intercurrent or heart failure.
Serum levels of creatine phosphokinase are elevated in most of the carrier females.
Other features are:
Pseudohypertrophy of deltoid and infraspinatus, fatty infitration of heart, respiratory infections.
Macroglossia, bone thinning, scoliosis, pathological fracture and loss of all reflexes except ankle jerk.
ECG shows tall R waves and deep Q waves in V1 lead.
Steroid may improve strength but no effect on prognosis
72. Which does not present as floppy baby:
(a) Littles disease
(b) Werdnig-Hoffman syndrome
71 D
72 A
96 / Pediatric Buster
Misc
PEM
Rickets
Ehler Danlos syndrome
CHDS
Prader-Willi syndrome
Cretinism
Infant botulism
73 A
74 D
75 B
14
Malignancies in Childhood
Leukemia:
Most common malignancy of childhood; second is brain tumor.
The frequency of leukemia increases with
Fanconis anemia
Down syndrome
Blooms syndrome
Klinefelters syndrome
Wiskott Aldrich syndrome and ataxia telangiectasia
Radiation exposure increases the risk of all leukemias except CLL and hairy cell.
Incidence of leukemia is 13/lac population
1. The most common type of leukaemia in children is:
(OPG5th/561) (AI
(a) ALL
(b) AML
(c) CLL
(d) CML
2. The most common haematological malignancy in children is:
(OPG5th/561) (KERALA
(a) CLL
(b) CML
(c) AML
(d) ALL
3. Blast cells of acute lymphocytic leukemia in childhood contain:
(OPG5th/562) (UP
(a) Surface antigen
(b) CALLA +ve
(c) Antibodies on WBC
(d) Excessive mitochondria
4. Poor prognosis in acute lymphatic leucaemia is indicated by all, except: (OPG5th/562) (JIPMER 80, UPSC
(a) Intrathoracic mass
(b) Children between 2 and 10 years
(c) Cranial secondaries
(d) WBC around 10,000 mm
5. Best prognosis in ALL in:
(OPG5th/562)(UP
(a) Pre B cell
(b) Precursor B cell
(c) B cells
(d) T cells
ALL:
Accounts for 70-80% of all leukemias
Incidence is 1:2000 live birth.
Peak age of onset is 3-7 yr; more common in males.
It has three subtypes L1, L2, L3.
L1 carries the best prognosis.
L3 is associated with EBV
Cytochemically all takes PAS stain and coarse granules are seen in positive cells, Tdt is present
in 90% of leukemic lymphoblasts.
Tdt is not found in L3 subtype
The cells may be CALLA +VE or CALLA-VA, 90% or 10%
Patient may demonstrate hyperdiploidy or aneuploidy. Hyperdiploidy is associated with good prognosis.
1 A
2 D
3 B
4 D
5 A
89)
94)
96)
89)
96)
Malignancies in Childhood / 98
99 / Pediatric Buster
6. Mediastinal widening is found in:
(OPG5th/563) (AIIMS 89)
(a) Histiocytic lymphoma
(b) Prolymphatic leukaemias
(c) CML
(d) ALL
7. Which type of leukaemia given prophylactic methotrexate for extracranial irradiation:
(OPG5th/564) (AI 97)
(a) ALL
(b) AML
(c) CLL
(d) CML
8. A 5-year-old child presents with history of fever off-and-on for past 2 weeks and petechial spots all over the body
and increasing pallor for past 1 month. Examination reveals splenomegaly of 2 cm below costal margin. The most
likely diagnosis is:
(OPG5th/563) (AI/04)
(a) Acute leukemia
(b) Idiopathic thrombocytopenic purpura
(c) Hypersplenism
(d) Aplastic anemia.
9. Wilms tumor is associated with all of the following except:
(OPG5th/574) (AI 90)
(a) Aniridia
(b) Beckwith syndrome
(c) Hemihypertrophy
(d) Polycystic kidney
10. True about juvenile CML:
(OPG5th/569) (UP 96)
(a) Philadelphia chromosome
(b) High HbF content
(c) Low Alkaline PO4
(d) Massive splenomegaly
Juvenile CML:
Children < yr
Philadephia chromosome is negative
HbF is 15-50%
Facial rash; Bleeding manifestation and lymphadenopathy is frequent
Frequent normoblasts on peripheral smear
Poor response to busulphan.
Median survival is 9 months
11. Which of the following childhood malignancies has highest cure rater:
(OPG5th/575) (PGI 81, AMU 86)
(a) Wilms tumour
(b) Neuroblastoma
(c) Retinoblastoma
(d) Rhabdomyosarcoma
Wilms Tumor (Nephroblastoma) Most common abdominal malignancy in children > 1 yr (< 1 yr neuroblastoma).
Peak age incidence 3-4 yr.
Arise from embryonic nephrogenic tissue from abnormal proliferation of metanephric blastema.
Consists of mixture of epithelial and stromal elements. (multicentric)
Associated with deletion of short arm chromosome 11.
More prevalent with associated anomalies as WAGR syndrome (Wilms tumor, anidridia,
genitourinary anomalies (Horseshoe kidney and mental retardation) and hemihypertrophy,
neurofibromatosis and Beckwith syndrome
M.C. presentation is painless smooth abdominal lump.
Triad of symptoms will be
Lump
Fever
Hematuria
Hypertension is also accompanying.
Usually U/L but in 5-10% may be B/L
Metastasize hematogenously to lung.
Prognostic factor is its histology.
Staging
I - Tumour limited to kidney and completely resected.
II - Tumour extends beyond the kidney but still resected completely.
III - Residual nonhematogenous tumour confirmed to abdomen after resection
positive lymph node and massive tumour spillage
IV - Hematogenous metastases.
V - B/L renal involment
Patient having a predominantly epithelial type of tumour has favoured history.
Egg shell pattern calcification on X-ray
USG and IVP helps in diagnosis.
6 D
7 A
8 A
9 D
10 B
11 A
12. A 4-year-old male child has painless haematuria persisting for several months. On examination, a nonballotable
mass was detected in the right loin. The most likely diagnosis is:
(OPG5th/574) (UPSC/01)
(a) Renal tuberculosis
(b) Wilms tumuor
(c) Hydronephrosis
(d) Renal oxalate stone
13. Neuroblastoma true are A/E:
(OPG5th/573) (PGI 87)
(a) Associated with mild hypertension
(b) Rare tumour to undergo regression
(c) Commonest tumour of
adrenal cortex
(d) Treatment is excision and postoperative radiotherapy
14. A-1-year-old child presenting with abdominal mass and calcifications on X-ray is suggestive of:
(a) Wilms tumour
(b) Neuroblastoma
(c) Teratoma
(d) Rhabdomyosarcoma (OPG5th/573) (PGI 86, 93)
15. Regarding childhood malignancy the true statement is:
(OPG5th/573) (AIIMS 94)
(a) AML is the most common
(b) 10% of CML have spleen involvement
(c) Neuroblastoma is common in
the 1-4 years age group
(d) CNS involvement is the most common presentation of lymphoma
Neuroblastoma:
Most common malignant tumour of infancy (50% case occur in first 2 yrs.)
At birth, patient may be born with metastases to placenta.
Autosomal dominant inheritance in familial incidence.
Arise from neuroblast and sites are adrenal, sympathetic chain, retroperitoneal area, posterior
mediastinum or cervical area.
Have a high frequency of spontaneous regression.
Neuroblastoma is seen in patients with
Neurofibromatosis
Nesidioblastosis
Hirschsprungs disease
Pathologically has characteristic of Homer Wright pseudorossette.
Most common presentation is asymptomatic, irregular, firm, abdominal mass.
May also present with Horner syndrome; mediastinal mass or orbital metastasis with proptosis,
periorbital ecchymosis or bilateral black eyes (Panda eyes).
Excess catecholamine (principally norepinephrine) causes flusing, sweating and hypocalcemic,
watery diarrhoea (due to VIP).
Dancing eye syndrome (opsomyoclonus and nystagmus) paraplegia or myasthenia gravis may also
present.
Spinal involvement best detected by MRI.
Patient may have widespread subcutaneous nodule blue in colour
(Bluebeary muffin baby).
X-ray abdomen show stippled calcification
USG is helpful in diagnosis; CT helps in staging but standard mode of imaging is MRI.
MIBG isotope scan is sensitive method to know primary and secondary.
Site of metastasis
In Infant
- Liver
In older children
- Bone (M.C. skull and diaphysis of distal femur
and humerus).
Most accurate method of biochemical diagnosis is urinary excretion of VMA and HVA (metabolites of
catecholamine).
Evans stagingStage
I - Tumor confined to single organ and can be completely resected.
II - Tumor extending beyond the organ but not crossing midline; regional lymph
nodes on hemilateral side may be involved.
III - Tumour crossing beyond the midline may have B/L lymph nodes.
12 B
13 C
14 B
15 C
- Distant metastasis.
- Patients who otherwise be of stage I or II but have metastasis confined to
liver; skin or bone marrow without cortical bone involvement (Prognosis is good).
TOC for stage I, II and IVs is surgery.
Neurone specific enolase and serum ferritin levels are important tumor and prognostic marker.
In advanced diseaseserum ferritin appears to be more critical prognostic factor.
Thoracic and head neck tumors do better than abdominal tumour.
Presence of opsoclonus or nystagmus, VIP, mature history, good nutrition, normal serum ferritin and
neuron specific enolase have good prognosis.
In stage III and IV, chemotherapy is used with cyclophosphamide and adriamycin.
17 D
18 A
19 C
20 B
21 C
22 A
23 D
M.C. secondary malignancy in retinoblastoma is osteogenic sarcoma (lung and breast cancer)
High prevalence of retinoblstoma is reported with trisomy 21
CEA may be elevated in retinoblastoma
24. Child with permeative bone lesions involving all body bones is likely to be suffering from:
(OPG/5th578) (AIIMS/2K)
(a) Neuroblastoma
(b) Histiocytosis X
(c) Metastases of Wilms tumor
(d) Osteomalacia
25. A child presents with seborrheic dermatitis, lytic skull lesions, ear discharge and hepatosplenomegaly; likely
diagnosis is:
(OPG5th/578) (AI/2001)
(a) Leukemia
(b) Lymphoma
(c) Histiocytosis X
(d) Septicemia
26. An eight-year-old boy presents with back pain and mild fever. His plain X-ray of the dorsolumbar spine reveals
a solitary collapsed dorsal vertebra with preserved disc spaces. There was no associated soft tissue shadow. The
most likely diagnosis is:
(OPG5th/578) (AI/03)
(a) Ewings sarcoma
(b) Tuberculosis
(c) Histiocytosis
(d) Metastasis
24 B
25 C
26 C
15
2 A
3 A
4 D
5 B
6 C
7 B
8 B
9 A
10 B
Lacunar skull:
Bony defect in frontal and parietal region
Associated with meningocoele
Presence of craniosynostosis
19. Scapocephaly is due to premature closure of the:
(NEL17th/1992)(ORISSA 99)
(a) Coronal suture
(b) Metopic suture
(c) Sagittal suture
(d) Lambdoid suture
20. Porencephaly is due to:
(NEL17th/1987)(MP/2K)
(a) Dandy-Walker syndrome
(b) Cerebral infarction
(c) Fetal alcohol syndrome
(d) Trisomy 13
21. Phocomelia is:
(OPG5th/600) (AI 96)
(a) Reduplication of bones
(b) Defect in long bones
(c) Absence of brain
(d) Defect in short bones
11 D
12 C
13 B
14 B
15 A
16 C
17 B
18 C
19 C
20 A
21 B
16
Inborn Errors of
Metabolism
2 B
3 B
4 D
5 C
6 B
Alkaptonuria:
Deficiency of homogentisic acid oxidasealkaline urine
Ochronotic arthritis commonly involves shoulders and hiprenal stone and nephrosis.
Ascorbic acid is sometimes recommended.
Homocystinemia (Autosomal recessive)
Deficiency of cystathionine beta synthetase, methionine and homocysteine level in blood elevated
and in 50% cases in brain also.
Subluxation of lens (downwards and medially); recurrent thromboembolic episodes convulsions,
mental retardation and osteoporosis and lesions of lens, glaucoma and marfanoid habitus.
Activation of Hageman factor is observed.
Plasma level of folate is observed.
Treatment: large doses of pyridoxine for life long.
Presence of homocysteine in urine is detected by cyanide nitroprusside test.
Risk of coronary, peripheral and cerebral vascular disease
8. Characteristic clinical picture of Maple syrup urine disease:
(a) Choreoathetosis
(b) Flaccid paralysis
(c) Petit mal disorder
(OPG5th/613)
(OPG5th/613) (JIPMER 81, DELHI 86)
(c) Involves delayed weaning
(d) Ensures
(e) Is best monitored by liver function tests
Galactosemia:
An autosomal recessive disorder manifests within few days after birth (after milk infestion)
Deficiency or galactose 1 phosphate uridyl transferase.
Persistence of jaundice, enlarged liver and oildrop cataract.
Child becomes mentally retarded.
Galactose free diet for whole life. Mental retardation is irreversible.
11. All of the following cause splenomegaly except:
(a) Gauchers disease
(b) von-Gierkes disease
7 B
8 D
9 C
10 A
11 B
Miscellaneous / 107
12. Child with recurrent hypoglycemic attacks and hepatomegaly is likely to have:
(OPG5th/614) (JIPMER 93)
(a) von-Gierkes disease
(b) Neonatal diabetes
(c) Neonatal hepatitis
(d) Galactosemia
13. Which of the following symptoms are associated with gargoylism (Hurlers syndrome): (OPG5th/615) (ORISSA 99)
(a) Hepatosplenomegaly
(b) Corneal opacities
(c) Profuse nasal discharge
(d) All of the above
14. A 2-year-old with coarse facial features and hepatosplenomegaly showed tall QRS waves in the ECG.The
diagnosis is most likely to be:
(OPG5th/615) (AIIMS/NOV/01)
(a) Glycogen storage disease type II
(b) Hurlers disease
(c) Hunters disease
(d) Hemochromatosis
15. In Gauchers disease there is accumulation of....inside the cells:
(OPG5th/616) (TN 95)
(a) Galactosidases
(b) Sphingomyelins
(c) Glucosidases
(d) Cerebrosides
16. Which of the following is associated with chronic liver disease?
(OPG5th/618) (AIIMS 83)
(a) Hepatitis A
(b) Alpha-1 antitrypsin deficiency
(c) Malaria
(d) Infections mononucleosis
17. Which is not a feature of Wilsons disease in a child:
(OPG5th/618) (AIIMS 91)
(a) Fanconi syndrome
(b) Sensory changes
(c) Haemolytic anemia
(d) Chronic active hepatitis
18. All of the following are recognised features of Wilsons disease except:
(OPG5th/618) (UP 97)
(a) Psychological disturbances
(b) Increased ceruloplasmin levels
(c) Increased copper content of liver
(d) Histopathological features of chronic active hepatitis
Wilsons diseaseautosomal recessive disorder with accumulation of copper in cytoplasm of liver cells.
- Hemolysis
- Affection of proximal renal tubulesFanconi syndrome
- Kayser-Fleischer ring.
- Chronic active hepatitis
- CNS manifestation with cerebellar ataxia and parkinsonism; rigidity in older patient.
- There is no sensory involvement.
- Low serum levels of ceruloplasmin and reduction of total serum copper level.
- D-penicillamine is used as chelating agent.
12 A
13 D
14 B
15 D
16 B
17 B
18 B
17
Miscellaneous
1. Interstitial keratitis in an 8-year-old girl can occur in all of the following conditions except:
(a) Tuberculosis
(b) Leprosy
(c) Filaria
(d) Syphilis
(OPG5th/621) (UPSC 96)
2. Consider the following statements : Cherry red spot of the macula is seen in A/E: (OPG5th/625) (UPSC 98)
(a) Tay-Sachs disease
(b) Sandhoff disease
(c) Niemann-Pick disease
(d) Generalised gangliosides
(e)
None
3. In infants, the cause of blindness arising out of oxygen toxicity is:
(OPG5th/626) (UPSC 97)
(a) Degeneration of crystalline lens
(b) Growth of blood vessels into vitreous followed by fibrosis
(c) Damage
to cornea
(d) Enzyme defect in lens
4. In children atopic dermatitis is associated with increase in:
(OPG5th/634) (UPSC 96)
(a) Immunoglobulin A
(b) Immunoglobulin E
(c) Immunoglobulin G
(d) Immunoglobulin M
5. A 3-year-old child is seen with coma, weakness, salivation and one sided constricted pupil. The most probable
diagnosis is:
(OPG5th/644)
(a) Tetanus
(b) Organophosphorus poisoning
(c) Phenobarbitone poisoning
(d) Electrolyte imbalance
6. The major toxicity of acetaminophen overdose involves:
(OPG5th/646) (PGI 78, AIIMS 84)
(a) The CNS
(b) The heart
(c) Liver
(d) Acid-base metabolism
7. Child with a history of consumption of iron tablets is symptomatic. Treatment will be: (OPG5th/646) (PGI 2000)
(a) Desferrioxamine 100 mg/IV
(b) Stomach wash
(c) Exchange transfusion
(d) X-ray to delineate number
of capsules swallowed
8. Water tolerance test is useful in differentiating the short stature as a result of:
(NEL17th/15) (AIIMS 81)
(a) Hypopituitarism
(b) Gonadal dysgenesis
(c) Primordial dwarfism
(d) Mild hypothyroidism
9. Preference of use one hand (handedness) is evedent by:
(NEL17th/49) (KERALA 97)
(a) 6 months
(b) 1 year
(c) 2 years
(d) 3 years
10. Waking up at night screaming with fear, at 2.5 years age is generally a manifestation of:
(NEL 17th/82)(PGI 78, 79, 80)
(a) Organic illness
(b) Normal development pattern
(c) Castration anxiety
(d) Separation anxiety
11. Which of the following childhood disorder improves with increase in age:
(NEL17th/88)(MP/2K)
(a) Conduct disorder
(b) Emotional problems
(c) Temper tantrum
(d) Sleep disorder
12. A two-year old girl child is brought to the OPD with features of hand wringing stereotype movements, impaired
language and communication development, breath-holding spells, poor social skills and deceleration of head
growth after 6 months of age. The most likely diagnosis is:
(NEL17th/94)(AIIMS/03)
(a) Aspergers syndrome
(b) Retts syndrome
(c) Fragile X syndrome
(d) Cotard syndrome
13. Abstract thinking occur at the age of:
(NEL17th/103) (JIPMER 93)
(a) 2 years
(b) 3 years
(c) 9 years
(d) 12 years
14. All are complications of formula fed baby over human milk fed baby except:
(NEL17th/158) (JIPMER 95)
(a) Necrotising enterocolitis
(b) Otitis media
(c) Hypocalcaemia
(d) Vitamin K deficiency
1 C
14 D
2 E
3 B
4 B
5 B
6 C
7 A
8 A
9 D
10 D
11 D
12 B
13 C
BREAST MILK
- Presence of long chain polyun saturated fatty acids (LC PUFA) in breast milk is responsible for
better visual and cognitive development of child.
- CMV, human T-cell lymphotropic virus type I, rubella, hepatitis B and herpes simplex virus have
been demonstrated in breast milk.
- Most of the milk is obtained early in the feeding (50% in the first 2 minute and 80-90% in the first
4 minute)
16. Sellicks manoeuvre is done for:
(NEL17th/291) (AIIMS 97)
(a) Prevent gastric aspiration
(b) CPR
(c) Assisted respiration
(d) Removal of foreign body
17. In pediatric advanced life support, intraosseous access for drug/fluid administration is recommended for pediatric
age of:
(NEL17th/293)(AIIMS/02)]
(a) <1 year age
(b) <5 years age
(c) <6 years age
(d) Any age
INTRAOSSEOUS ACCESS
Intraosseous line: Most common sites for insertion are the upper tibia, sternum and anterior superior
iliac spine.
18. Following is seen in cold injury to newborn baby except:
(NEL17th/336) (AI 99)
(a) Metabolic acidosis
(b) Sclerema
(c) Bradycardia
(d) Extreme shivering
19. Serum cholesterol is usually high in all the following conditions except:
(NEL17th/379) (AI 89)
(a) Nephrotic syndrome
(b) Primary biliary cirrhosis
(c) Kwashiorkor syndrome
(d) Diabetes mellitus
20. A child come with abdoman pain, arthralgria, hematuria, hypertension diagnosis is: (NEL17th/416) (JIPMER 98)
(a) Haemolytic uremic syndrome
(b) Porphyria
(c) Rheumatic fever
(d) Dengue
21. Which is a high risk neonate:
(NEL17th/452) (PGI 89)
(a) Less than 1500 gm
(b) Previous neonatal death
(c) Less than 37 weeks
(d) All
22. Splenic rupture in children is best diagnosed by:
(NEL/469) (AIIMS 89)
99
(a) Paracentesis
(b) Ultrasound
(c) Plain X-ray
(d) Tc scan
23. All of the following groups newborns are at an increased risk of hypoglycemia except: (NEL17th/508t)(AIIMS/02)
(a) Birth asphyxia
(b) Respiratory distress syndrome
(c) Maternal diabetes.
(d) Post term infant.
24. CMV infection in mother the newborn is likely to develop:
(NEL17th/523) (AIIMS 99)
(a) Blood dyscrasia
(b) Myocarditis
(c) Encephalitis
(d) Deafness
CMV disease: Transmitted through oropharyngeal secretion, urine, cervical and vaginal excretions,
spermatic fluid; breast milk and blood. Congenital CMV infection develops in approximately 5% of
infected fetuses and risk to foetus is greatest when the pregnant woman has primary CMV infection.
Only 1% foetus are infected in women with recurrence of previous infection
90-95% of infected foetuses are asymptomatic
The asymptomatic ones develop psychomotor, senosineural hearing loss, ocular or dental
abnormalities over the next years.
CF: Anemia, thrombocytopenic purpura, hepato-splenomegaly, neonatal hepatitis, jaundice,
meningoencephalitis; microcephaly, intracraninal calcification and chorioretinitis, petechial rash.
Diagnosis is by serum lgM and SGOT; confirmed by isolation of CMV.
25. Enzyme replacement therapy is available for the treatment of the following disorder: (NEL17th/464)(AIIMS/03)
(a) Gaucher disease
(b) Niemann-Pick disease
(c) Hunter syndrome
(d) Phenylketonuria.
15 D
16 A
17 C
18 B
19 C
20 B
21 D
22 B
23 D
24 C
25 A
Miscellaneous / 110
26. History of dislike for sweet food items is typically present in:
(NEL17th/477)AIIMS/03)
(a) Diabetes mellitus
(b) Glycogen storage disease
(c) Hereditary fructose intolerance
(d) Galactosemia.
HEREDITARY FRUCTOSE INTOLERANCE
Due to deficiency of fructose 1-6 bisphosphate aldolase (Aldolase B)
Rapid accumulation of fructose 1-phosphate causing severe toxic symptoms on exposure to fructose.
Gene present on chromosome 9q 13-32.
Patients are perfectly healthy and asmptomatic until fructose or sucrose (table sugar) is ingested
(usually from fruit or sweetened cereal).
Jaundice, hepatomegaly, vomitting, lethargy, irritability convulsions.
Reducing substance in urine.
Treatment is complete elimination of all sources of sucrose, fructose and sorbitol from diet, which
often patient learns to do himself.
27. Which one is not unfavourable for fetal development:
(a) Herpes
(b) Rubella
(c) Alcohol
(d) Tetracycline
28. Closure of the anterior fontanelle is delayed except: in:
(a) Down syndrome
(b) Osteogenesis imperfecta
(c) Hypogonadism
(e) Meningomyelocele
Large anterior fontanalle
Achondroplasia
Apert syndrome
Athyrotic hypothyroidism
Cleidocranial dysostosis
Congenital rubella syndrome
Hallermann-Streiff syndrome
Hydrocephaly
Hypophosphatasia
(NEL
17th/524)
(AIIMS 89)
(NEL
(d) Insulin
17th/530)
(PGI 89)
Contraindication of breastfeeding
Mothers with septicemia, active tuberculosis, typhoid, breast cancer or malaria should not breastfeed.
30. Asphyxial injury in a term baby is characterized by all except:
(a) Seizures
(b) Differential hypotonia (lower limbs>upper limbs)
in clearing oral secretions.
(NEL17th/566)(AIIMS/03)
(c) Altered sensorium
(d) Difficulty
27 A
28 C
29 B,C 30 B
31 D
32 C
33. A 2-day-old newborn baby presented with microcephaly, macroglosia, visceromegaly and a blood glucose level of
20 mg/dl. What is the most likely diagnosis?
(NEL17th/616)(ORISSA 98)
(a) Prader-Willi syndrome (b) Beckwith-Wiedemann syndrome (c) Werner syndrome (d) Cockayne syndrome
34. Lymphocyte transformation is a sensitive test most useful in identification of:
(NEL17th/650-51) (PGI 79, AIIMS 81)
(a) Drug reaction
(b) Contact dermatitis
(c) Pollen allergy
(d) food allergy
35. All true of Hair cartilage hypoplasia syndrome except:
(NEL17th/698) (AIMS 97)
(a) Short strature dwarf
(b) Sparce hair
(c) Neutropenia
(d) T cell dysfunction
36. Most common opportunistic infection in children with neutropenia is:
(NEL17th/722) (JIPMER 93)
(a) Gram-negative bacilli
(b) Streptococci
(c) Staphylococci
(d) Pneumococci
37. A 3-year-old boy is brought to the casualty by his mother with progressive shortness of breath for 1 day. The
child has history of bronchial asthma. On examination, the child is blue, grasping and unresponsive. What will
you like to do first?
(NEL17th/772)(AIIMS/02)
(a) Intubate
(b) Administer 100% oxygen by mask
(c) Ventilate with bag and mask
(d) Administer
nebulised salbutamol
38. A 5-yr-old boy a known patient of bronchial asthma, present with cough wheezing and breathlessness.
Examination show respiratory rate 48/minute, pulsus paradoxus and bilateral rhonchi. The most appropriate
immediate treatment is:
(NEL17th/772) (UPSC/02)
(a) Intravenous theophylline and corticosteroids
(b) Intravenous theophylline and nebulized salbutamol
(c) Nebulized salbutamol and ipratropium
(d) Nebulized salbutamol and intravenous corticosteroids
39. Most common cause of chronic arthritis in children:
(NEL17th/799) (PGI 2000)
(a) Rheumatic fever
(b) Rheumatoid arthritis
(c) JRA
(d) Rheumatic arthritis
40. Blood and mucus in stools are seen with all except:
(NEL17th/799) (AI 89)
(a) E. histolytic
(b) Shigella
(c) E. coli
(d) V. cholera
41. Common features of sarcoidosis in childhood include:
(NEL 17th/822) (AIIMS 78, PGI 79, 81)
(a) Parotitis
(b) Erythema nodosum
(c) A high incidence of spontaneous remission
(d) All are true
42. A 6-year old boy is brought to emergency with purpuric and petechial rash all over the body. He appears
semiconscious and febrile. Total leucocyte count is 20000 per mm3 with 88% polymorphs. Platelet count is
1.2 lac/mm3. The most likely diagnosis is:
(NEL17th/897)(UPSC/04)
(a) Idiopathic thrombocytopenic purpura
(b) Meningococcemia
(c) Staphylococcal
(d) Dengue hemorrhagic
fever
43. Bad prognosis in meningococcal infection is indicated by:
(NEL17th/898)PGI 88)
(a) Hypertension
(b) Rash more than 3 days
(c) Leucocytosis
(d) Leucopenia
44. Which is not true of congenital syphilis:
(NEL 17th/979) (JIPMER 92)
(a) Severe periostitis
(b) VDRL negative indicates absence of disease
(c) Osteochondritis occurs after 6
month
(d) Bone within bone appearance
45. Enamel hypoplasia is associated with all except:
(NEL17th/1038) (JIPMER 79, AIIMS 81)
(a) Cerebral palsy
(b) Nephrosis
(c) Rubella
(d) Gardners syndrome
46. Rota virus immunity in children occur above....years:
(NEL 17th/1081) (AMC 87 AIIMS 84)
(a) 2
(b) 5
(c) 7
(d) 10
47. A 6-month-old infant presents to the diarrhea clinic unit with some dehydration. The most likely organism
causing diarrhea is:
(NEL17th/1082)(AIIMS/03)
(a) Entamoeba histolytica
(b) Rotavirus
(c) Giardia lamblia
(d) Shigella
33 B
46 B
34 A
47 B
35 C
36 C
37 A
38 D
39 D
40 D
41 D
42 B
43 D
44 B
45 B
Miscellaneous / 112
48. Fatty change occurs in A/E:
(NEL17th/1129)(UP 96)
(a) Kwashiorkor
(b) Marasmus
(c) Miliary tuberculosis
(d) Alcoholic liver disease
49. Cirrhosis with portal hypertension is seen in all of the following except:
(NEL17th/1155) (AI 91)
(a) Cystic fibrosis
(b) Alpha-1 antitrypsin deficiency
(c) Congenital hepatic fibrosis
(d) Wilsons disease.
50. The following is not a feature of Pierre-Robin syndrome:
(NEL17th/1209) (JIPMER 91)
(a) Hearing defect
(b) Coloboma irides
(c) Respiratory distress
(d) Mandibular hypoplasia
51. Primary symptom of hiatus hernia during the newborn period is:
(NEL 17th/1221) (AIIMS 80, UPSC 86)
(a) Anorexia
(b) Anaemia
(c) Vomiting
(d) Distended abdomen
52. Lactase deficiency in children is characterised by:
(NEL 17th/1268) (UP 97)
(a) Dumping syndrome
(b) Abdominal bloating, cramps and diarrhoea
(c) High pH of the stool
(d) Low fatty acid levels in blood
53. Which of the following does not cause gastroenteritis in infants:
(NEL 17th/1278) (UP 95)
(a) Adenovirus
(b) Calcivirus
(c) Reovirus
(d) Rotavirus
54. What is true of canals of Lambert in the alveolar spaces of the lung:
(NEL 17th/1374) (PGI 78, 80)
(a) Bronchi alveolar connection
(b) Prevent atelectasis
(c) Delay in collapse
(d) All
55. Percentage of HbF in a 6-month-old infant is:
(NEL17th/1376) (KERALA 97)
(a) 10
(b) 30
(c) 50
(d) 60
56. Acanthocytosis is usually not associated with:
(NEL17th/1395) (PGI 80, AIIMS 81)
(a) Steatorrhoea
(b) Failure to thrive
(c) Abnormal stools
(d) Abnormally high plasma cholesterol
57. A 3-month-old infant presents with intermittent respiratory strider since 10 days of age the most likely
diagnosis is:
(NEL 17th/1409) (AIIMS 95)
(a) Laryngomalacia
(b) Tracheoesophageal fistula
(c) Laryngotracheobronchitis
(d) Neoplasm
58. Primary treatment of choice for hepatoma in childhood is:
(NEL17th/1473) (JIPMER 81, AMC 82)
(a) Vitamin B12
(b) Surgery
(c) Chemotherapy
(d) Radiation therapy
59. All cause nephrotic syndrome except:
(NEL17th/1500) (TN/89)
(a) Minimal lesion glomerulonephritis
(b) Membranous glomerulonephritis
(c) Post-streptococcal glomerulonephritis
(d) Focal glomerulosclerosis
Post-streptococcal acute glomerulonephritis :
Caused by strains 4 and 12 causing pharyngitis and type 49 causing pyoderma.
Occurs in school age children susually 1-2 wk after streptococcal infection.
Typical example of immune complex disease.
Maximum endocapillary proliferation and subepithelial humps of Ig G and complement is seen
pathologically.
Rapid edema; smoky urine (haematuria) and red casts are diagnostic.
Hypertension is usually present.
Pus cells are also present in urine.
ESR in increased and ASO titres are elevated.
Serum complement C3 is low and comes to normal within 5-6 wk.
Atypical presentation may be convulsions.
LVF or
ARF
95% recover spontaneously or with conservative therapy.
Excellent prognosis in childhood.
60. Primary antiphospholipid syndrome is characterised by all the following clinical features except:
(NEL 17th/1668)(UPSC 96)
(a) Recurrent foetal loss
(b) Arterial and venous thrombosis
(c) Foetal cardiac abnormalities
61. Ankle colonus of 5 contractions in a 2-months-old infant:
(NEL17th/1671) (AIIMS 79)
(a) Indicates neurological disease
(b) Often normal
(c) Abnormal at any age
(d) Normal in early years
of language development
48 B
61 B
49 A
50 B
51 C
52 B
53 C
54 D
55 A
56 B
57 A
58 B
59 C
60 C
63 B
76 B
64 C
77 A
65 C
78 A
66 B
79 B
67 B
80 C
68 C
81 D
69 C
70 B
71 D
72 C
73 C
74 B
82 B
83 E
84 A
85 A
18
Self-assessment
Juvenile rheumatoid arthritis (JRA) (Stills disease) : Defined as arthritis of one or more joints
with onset before age of 16 yr and persisting for at least 6 wk.
Types
(a) Pauciarticular
(b) Polyarticular
(c) Systemic (with fever and rash)
1 A
2 C
3 B
4 C
5 D
6 A
7 C
8 B
9 B
Self-assessment / 116
PauciarticularMost frequent type of JRA
Type Imore common in females
- Age of onset 5 yr.
- Knees are more commonly affected; also involved are ankles and elbows, small joints escape.
- Iridocyclitis; secondary glaucoma and cateract may occur.
- Antinuclear antibodies are positive and rheumatoid factor is negative (HLA DR -5 DR W-8)
Type IImore in boys older than 8 years
- Hip girdle usually involved.
- Sacroileitis may develop.
- ANA and rheumatoid factor -ve.
- HLA B27.
PolyarticularGirls affected. Loss of lumbar lordosis, diminished mobility of spine and low back pain.
Type I
Theumatoid factor positive; arthritis is more severe. HLA DR 4
Type II
Rheumatoid factor negative; PIP involved; flexion deformities. Hip development is
affected due to lack of mobility.
Systemic JRAMore in boys
Intermittent fever. Characterisitic evanscent maculopapular rash with central clearing
- Pericarditis and interstitial lung disease
- Lymphadenopathy and splenomegaly
- ANA +ve RA -ve
Amyloidosis develops in long-standing disease.
Prognosis is relatively better in sero-negative polyarticular JRA.
Naproxen or indomethacin is drug of choice.
10. True in artificial feeding are A/E:
(UP 96)
(a) Eczema
(b) Jaundice
(c) Tetany
(d) Enteropathic necrotising
11. Ovulation and ability to become pregnant usually follow menarche by about:
(AIIMS 81)
(a) 5-6 months
(b) 2-4 months
(c) 1 month
(d) 12-24 months
12. Sneezing in a neonate indicates:
(a) Nasal allergy
(b) Syphilis
(c) Nasopharyngitis
(d) A normal phenomenon
13. Neonatal seizure with cataract is seen in A/E:
(UP 97)
(a) Galactosaemia
(b) Toxoplasma
(c) Tay-Sachs disease
(d) Premature with birth asphyxia
14. A 6-month-infant exclusively on breastfeeding develops deficiency of:
(UP 96)
(a) Iron
(b) Vitamin C
(c) Vitamin A
(d) Calcium
15. Commonest cardiac rhythm disturbance encountered in children is:
(KARNATAKA/98)
(a) Supraventricular tachycardia
(b) Ventricular tachycardia
(c) Sinus tachycardia
(d) Junctional rhythm
16. Lowes syndrome is characterised by the following except:
(AIIMS 84)
(a) Hypophosphataemic rickets
(b) Undescended testes
(c) Defect in the CNS and eyes
(d) Aminoaciduria
17. Continuous pharyngeal suction in an asphyxiated infant is not recommended because it may lead to:
(a) Vomiting
(b) Seizures
(c) Bradycardia
(d) All of the above
(AIIMS 83)
18. Which is an abnormal finding in a neonate:
(PGI 89)
(a) Glycosuria
(b) Bacteriuria
(c) WBCs in urine
(d) Hyperbilirubinaemia
19. Cardiomyopathy is a recognised finding in all except:
(PGI 81, BHU 87)
(a) Friedreichs ataxia
(b) Transfusion haemosiderosis
(c) Cystic fibrosis
(d) Muscular dystrophy
20. The most common cause of convulsions in a newborn on the first day:
(AI 92)
(a) Hypocalcaemia
(b) Hypoglycaemia
(c) Anoxia
(d) Head injury
21. Which of the following drugs can cause dyspnoea in children:
(PGI 79, AIIMS 79, 82)
(a) Paracetamol
(b) Salicylates
(c) Ampicillin
(d) Propranolol
22. Increased learning disabilities are seen in:
(AIIMS/99)
(a) PKU
(b) Down syndrome
(c) Cretinism
(d) Attention deficit
10 B
11 D
12 D
13 C
14 A
15 A
16 B
17 C
18 B
19 C
20 C
21 B
22 D
24 C
37 A
25 C
38 A
26 A
39 B
27 D
40 D
28 B
41 D
29 A
42 D
30 B
43 A
31 C
44 A
32 B
33 A
34 B
35 D
Self-assessment / 118
45. Following a scratch test a reaction of a moderate wheal and flare, without pseudopods. The test is interpreted
as:
(a) 4 plus
(b) 3 plus
(c) 2 plus
(d) 1 plus
(PGI 80, 81)
46. A child presented with effortless vomiting, we will come across all of the following biochemical findings except:
(a) Decrease urine potassium
(b) Decrease serum potassium
(c) Increase serum
(AIIMS/99)
bicarbonate
(d) Increase pH of blood
47. Chromatin positive seminiferous tubule dysgenesis had been seen in association with:
(AIIMS 78, 85, 87)
(a) Hypothyroidism
(b) Hypopituitarism
(c) Adrenal hyperplasia
(d) Down syndrome
48. Pericardial knock is characteristically heard in which of the following heart lesion? (PGI 81, KERALA 86)
(a) Pericardial effusion
(b) Rheumatic carditis
(c) Cardiac myxoma only
(d) Toxic myocarditis
49. What is the normal average stool sodium content in a newborn who is the fed on breast milk:
(PGI/90)
(a) 19 mEq/L
(b) 26 mEq/L
(c) 22 mEq/L
(d) 27 mEq/L
50. Which of the following would be considered normal in a 4-year child?
(PGI 80)
(a) Tics
(b) Hyperexcitability
(c) Anxiety
(d) Hyperkinesis
51. Which adjustment mechanism is probably most commonly used by children?
(PGI 80, NIMHANS 83)
(a) Aggression
(b) Fantasy
(c) Projection
(d) Somatisation
52. Ectopic ureter may be frequently associated with:
(JIPMER 81, AMU 89)
(a) Oliguria
(b) Dysuria
(c) Bilateral hydroureter
(d) Paradoxical incontinence
53. Bat wing appearance in X-ray is seen in:
(KERALA 94)
(a) Bronchial asthma
(b) Cardiogenic pulmonary oedema
(c) Mitral stenosis
(d) Pulmonary embolism
54. Which of the following is not a complication of smoking in pregnancy:
(JIPMER 95)
(a) Congenital malformation
(b) Intrauterine growth retardation
(c) Poor weight gain
(d) Foetal death
55. If present of definitive pathological significance in sputum is:
(AI 95)
(a) Mycobacterium tuberculosis
(b) Klebsiella
(c) Streptococcus
(d) Staphylococcus
56. In the labratory, for amplification of a single DNA chain polymerase chain reaction was allowed to continue for
3 cycles. At the end of this the number of DNA chains would be:
(AIIMS/NOV/01)
(a) 4
(b) 8
(c) 16
(d) 3
57. Micronodular cirrhosis is caused by:
(TN 95)
(a) Alcoholic cirrhosis
(b) Indian childhood cirrhosis
(c) Biliary atresia
(d) Wilsons disease
58. An infant is crying excessively even after being given good feed. He is passing a large quantity of urine and repeatedly
getting dehydrated. Urine examination shows no proteinuria and a specific gravity of 1004. This is a case of:
(UPSC 96)
(a) Diabetes mellitus (b) Diabetes insipidus (c) Congenital nephritic syndrome (d) Protein losing enteropathy
59. In children with cerebral oedema, which one of the following corticosteroids will be effective?
(UPSC 96)
(a) Hydrocortisone
(b) Prednisolone
(c) Dexamethasone
(d) Betamethasone
60. All of following statements are true except:
(DELHI 96)
(a) At 2-3 years of age, lymphocytes from 6-70% of total leucocytes
(b) After puberty, neutrophils replace
lymphocytes as the predominant leucocyte
(c) Lymphocytes are an important protective against influence
childhood infection
(d) There is no change in proportion of lymphocytes with age
61. The maximum number of deaths in children occur in following age group:
(DELHI 96)
(a) 2-5 years
(b) 1-2 years
(c) first 7 days
(d) 6-12 months
62. Shakir tape is used for:
(DELHI 96)
(a) Measurement of height
(b) Measurement of length of infant
(c) Measurement of midarm circumference
(d) Measurement of skin pad thickness
Shakirs tape to measure nutritional status by measuring mid arm circumferances, having 3 zones
1. Green (> 13.5 cm) Normal
2. Yellow (12.5-13.5 cm) Border line malnutrition
3. Red (< 12.5 cm) Severe malnutrition.
46 A
59 C
47 D
60 D
48 A
61 C
49 A
62 C
50 D
63 D
52 D
53 B
(ASSAM 95)
(d) Crown heel length
54 A
55 A
56 B
57 A
65 D
78 B
66 A
79 C
67 A
80 D
68 A
81 B
69 C
70 B
71 B
72 C
73 D
74 A
75 B
76 B
Self-assessment / 120
82. The use of steroids in neonates is required in:
(a) Bronchial aplasia
(b) Meconium aspiration syndrome
(c) Congenital polycystic disease
(d) Craniopharyngioma
83. Prednisolone is the treatment of choice in a haemophiliac with:
(a) Spontaneous haematuria (b) Gingival bleeding (c) Traumatic hemarthrosis (d) None
(AI 94)
(AIIMS 81)
83 A
96 D
84 B
97 C
85 C
98 A
86 B
99 D
87 C
88 B
89 C
90 C
91 A
(AI 88)
the diagnosis would be:
(d) ECG
(AI 87)
grade of malnutrition in this
(AIIMS 87)
(PGI 87)
(PGI 87)
(e) Bronchogenic carcinoma
(PGI 87)
92 B
93 C
94 C
101 D
102 C
103 C
104 D
105 C
106 D
107 E
108 D
109 A
110 D
111 A
112 A
Self-assessment / 122
114. Infantile diarrhoea can be caused by all except:
(AI 89)
(a) Rotavirus
(b) Retrovirus
(c) Adenovirus
(d) Calci virus
115. Suppression of lactation is indicated in all except:
(AI 89)
(a) Neonatal death
(b) Mid trimester abortion
(c) IDDM
(d) Lithium administration
116. The most common feature of hypothyroidism in children is:
(PGI/87)
(a) Cataract
(b) Recurrent seizure
(c) Cold extremities
(d) Laryngospasms
117. Haematoma of the sternomastoid muscle detected in a 16-days-old infant requires:
(BHU 87)
(a) Immediate surgical evacuation
(b) Prophylactic antibiotic therapy
(c) No immediate therapy
(d) Surgical intervention within 2 weeks
118. A 4 kg infant with severe dehydration (10% dehydration) needs the following amount of intravenous fluid in the
first 24 hours:
(JIPMER 80, PGI 83)
(a) 500 ml
(b) 800 ml
(c) 100 ml
(d) 1200 ml
119. Neonatal thrombocytopenia is most commonly due to:
(AIIMS/99)
(a) Auto immune thrombocytopenia
(b) Isommune thrombocytopenia
(c) SLE
(d) None of the above
120. Causes of neonatal seizure which carry the best prognosis:
(JIPMER/84)
(a) Birth asphyxia
(b) Hypoglycaemia
(c) Bacterial meningitis
(d) Late onset hypocalcemia
121. The most common complication following smallpox vaccination:
(AIIMS 83)
(a) Allergic rashes
(b) Eczema vaccination
(c) Encephalitis
(d) Vaccina gangrenosa
122. The most common cause of aplastic anemia is:
(AIIMS 83)
(a) Idiopathic
(b) Chloramphenicol
(c) Phenylbutazone
(d) Petroleum products
123. Posterior cervical lymph node enlargement in children is most often due to:
(AIIMS 83)
(a) TB
(b) Hodgkins disease
(c) Acute tonsillitis
(d) Pediculosis capitis
124. Recurrent abdominal pain in children most often due to:
(AIIMS 83)
(a) Roundworms
(b) Emotional/behavioural problems
(c) Amoebiasis
(d) Giardiasis
125. In the management of a child unconscious from poisoning; the first thing to do is to:
(AIIMS 83)
(a) Establish airway
(b) Take detailed history
(c) Blood for chemical analysis
(d) Gastric wash out
126. From which of the following the highest percentage of iron is absorbed?
(AIIMS 83)
(a) Spinach
(b) Soya bean
(c) Mutton
(d) Egg
127. The most important factor to overcome protein-energy malnutrition in children less than 3 years is: (AIIMS 83)
(a) Supply of subsidised food from ration shop
(b) Early supplementation of solids in infants
(c) Immunization to the child
(d) Treatment of anemia and pneumonia in infant and toddlers
128. The most common cause of neonatal death in India is:
(AIIMS 83)
(a) Prematurity
(b) Congenital malformations
(c) Metabolic disease
(d) Birth injury
129. Breastfeeding is best for the baby:
(AIIMS 83)
(a) But has no effect on the mother
(b) But is likely to affect the mothers appearance significantly
(c) But
may cause breast cancer in the mother
(d) And is also good for the mother
130. The average faecal sodium excretion in rotavirus diarrhoea is:
(AIIMS 84)
(a) 120 mEq/L
(b) 90 mEq/L
(c) 30 mEq/L
(d) 75 mEq/L
131. Breastfed babies as compared to artificially fed babies have all the following benefits except:
(AIIMS 84)
(a) Lower incidence of diarrhoea episodes
(b) Lower incidence of respiratory infection
(c) Lower incidence
of late onset tetany
(d) Lower incidence of future obesity
ADVANTAGES OF BREASTFEEDING
Counters risk of allergic disorders like asthma and eczema
Cancer (lymphoma) risk is reduced
Configuration of jaw is better because of the mechanism of sucking at the breast.
Caries in teeth are less common.
Coronary artery disease risk in later life is reduced.
Clever children: Premature babies who were breastfed, turned out to be more intelligent than the
artificially fed.
Crib death or sudden infant death syndrome risk is probably reduced in breastfed babies.
114 B
127 B
115 C
128 A
116 B
129 D
117 C
130 A
118 B
131 D
119 B
120 D
121 C
122 A
123 D
124 C
125 A
126 C
133 D
146 D
134 A
147 A
135 C
148 D
136 D
149 D
137 A
150 C
138 A
151 A
139 C
152 A
140 C
153 B
141 A
154 C
142 D
155 B
143 B
144 A
Self-assessment / 124
156. Which enzymatic reaction is depressed in malnutrition:
(PAL 93)
(a) Conjugation
(b) Carboxylation
(c) Oxidation
(d) Hydroxylation
157. Most common cause of intracerebral calcification in a child in India is:
(JIPMER 93)
(a) Tuberculoma
(b) A-V malformation
(c) Hyperparathyroidism
(d) Toxoplasmosis
158. Lutembachers syndrome refers to:
(JIPMER 79, AIIMS 81)
(a) Ostium primum defect with mitral stenosis
(b) Ostium secundum defect with mitral stenosis
(c) Ostium secundum defect with mitral incompetence
(d) None
159. Spasticity is associated with all except:
(PGI 80, AMU 86)
(a) Clonus
(b) Quadriplegia
(c) Clasp-knife rigidity
(d) LMN type paralysis
160. The behaviour of an adolescent is probably best described as being:
(JIPMER 80, AIIMS 83)
(a) Domineering
(b) Belligerent
(c) Aggressive
(d) Paradoxical
161. A child with increased intracranial tension (BP-160/120) and papilledema comes with convulsion, he was given
diazepam to control seizures, next step of treatment will be:
(AI/2000)
(a) I/V frusemide
(b) Sublingual nifedipine
(c) Sublingual nitrate
(d) Nitropruside infusion
162. Child with which of the following throat infection needs systemic antibiotic therapy: (JIPMER 81, DELHI 92)
(a) Pneumococci
(b) B. haemolytic streptococci
(c) Staph aureus
(d) All
163. The major site of active haemopoiesis in a foetus of 5 months is:
(AIIMS 81, PGI 81)
(a) Spleen
(b) Liver
(c) Lymph nodes
(d) Bone marrow
164. Under pulse polio programme, target age group is:
(JIPMER 98)
(a) Under 1 year
(b) Under 3 years
(c) Under 5 years
(d) Under 10 years
165. All the following hormones can affect growth of a child except:
(JIPMER 98)
(a) GH
(b) ACTH
(c) Insulin
(d) Somatostatin
166. What is correct in child:
(AIIMS 89)
(a) Bacterial sore throats most common
(b) Antibiotics required in all sore throats
(c) Antibiotics required
in cervical lymphadenitis with fever
(d) Tonsillectomy is indicated in recurrent sore throat
167. Diagnosis should be made only after abduction and external rotation, the site of lesion is:
(a) C3, C4
(b) C5, C6
(c) C7, C8
(d) T1, T2
(AIIMS/99)
168. A 6-day-old newborn infant develops ketonuria, seizures and hypoglycemia. The likely diagnosis is: (AIIMS/99)
(a) Tyrosinemia
(b) Aromatic aminoaciduria
(c) Phenylketonuria
(d) Intrauterine infections
169. The urine of newborn infants may show:
(AIIMS 91)
(a) Glycosuria
(b) Transient proteinuria
(c) Symptomatic bacteriuria
(d) Microscopic hematuria
170. In neonate with respiratory distress with multiple cavitary lesions on X-ray chest is:
(AIIMS 92)
(a) Diaphragmatic hernia
(b) Multiple congenital cysts
(c) Pneumatocoeles
(d) Obstructive emphysema
171. An infant with dyspnoea and multiple translucencies of whole left hemithorax is suffering from: (AIIMS 92)
(a) Pneumatocoele
(b) Multiple congenital lung cyst
(c) Diaphragmatic hernia
(d) Cystic fibrosis
172. In an infant exclusively fed on breast milk with pallor, hepatomegaly but no splenomegaly, investigation of
choice is:
(AIIMS 92)
(a) Serum iron levels
(b) HbF levels
(c) Serum B12 levels
(d) Serum folic acid levels
173. Taste perception of baby develops at:
(TN/99)
(a) Birth
(b) 4 months
(c) 6 months
(d) 8 months
174. Deficiency of which vitamin is not known in newborn:
(AIIMS 92)
(a) C
(b) D
(c) E
(d) K
175. Artificial feeding causes following except:
(AIIMS 91)
(a) Eczema
(b) Late onset tetany
(c) Haemorrhagic disease
(d) Necrotising enterocolitis
176. Which is true in growth of children:
(AIIMS 92)
(a) Should not be < 5 cm/year in prepubertal age (b) Growth is by obesity
(c) Prepubertal growth depends
on basal GH level
(d) Gonadal growth parallels somatic growth
177. Newborn child not passed urine for 36 hours, investigation method of choice:
(AIIMS 95)
(a) Ultrasound of kidney and bladder
(b) X-ray pelvis
(c) Cystoscopy
(d) CT scan
178. Following are true about Lawrence-Moon-Biedl syndrome except:
(AIIMS 95)
(a) Mental retardation
(b) Digital anomalies
(c) Hypogonadism
(d) Asthenic build
156 A
169 A
157 A
170 B
158 B
171 B
159 D
172 A
160 D
173 A
161 D
174 C
162 D
175 B
163 D
176 C
164 C
177 A
165 D
178 D
166 C
167 B
168 B
180 A
193 C
181 A
194 D
182 B
195 B
183 A
196 A
184 C
197 B
185 C
186 C
187 B
188 B
189 B
190 D
191 B
Self-assessment / 126
Soft tissue sarcomas: (Rhabdomysarcoma)arises from
Unsegmented mesoderm.
M.C. age group affected is 2-6 yr abd adolescent.
Most common type is of embryonal type and have best prognosis.
M.C. 40% arise in head and neck and 30% in urogenital region.
M.C. site in males is bladder or prostate.
Lymph node metatases are extremely common.
Racquet shaped cells are found.
Most valuable for certain identificationcross striations; desmin and myoglobin.
Treatment is surgical resection of tumor mass plus chemotherapy and radiotherapy.
Commonly used drugs in chemotherapy is vincristine, actinomycin D and cyclosphosphamide.
198. Neonatal herpes is caused by:
(PGI 2000)
(a) HSV-2
(b) HSV-1
(c) HPV
(d) HHV-8
199. If a 2-year-old child develops leukemia, which one of the following radiological findings is likely to occur:
(PGI 2000)
(a) Osteoporosis of long bones
(b) Periosteal new bone formation
(c) A radiolucent rim near the epiphysis
(d) Osteolytic lesions
200. Production of adult hemoglobin in the fetus occurs from:
(PGI 2000)
(a) 5th week
(b) 10th week
(c) 11th week
(d) 20th week
(e) 25th week
201. Which of the following is true regarding larynx of neonates:
(PGI 2000)
(a) Larynx is present at the level of C4,5,6
(b) Epiglottis is large and leafy
(c) Glottis is the narrowest
part
(d) Tongue is small compared to the oral cavity
202. As per the WHO, for infant less than 6 months, the Hb cut off level is:
(AIIMS/NOV/01)
(a) 100 g/L
(b) 115 g/L
(c) 105 g/L
(d) 110 g/l
203. Breathing movements and swallowing movement seen in the foetus by:
(AIIMS/NOV/01)
(a) 13-14 weeks
(b) 18-24 weeks
(c) 15-18 weeks
(d) > 30 weeks
204. A child with recent onset of URTI after 2 days presents with acute onset of breathlessness, cough and fever. All
of the following can be given except:
(AIIMS/MAY /01)
(a) Antibiotics
(b) Antipyretics
(c) O2 inhalation
(d) Morphine
205. If in a community a high proportion of newborn babies suffers from methaemoglobinaemia, then which of the
following inferences would be most relevant:
(UPSC/01)
(a) The community is exposed to industrial smoke
(b) The local water supply is very rich in nitrites
(c) The babies are suffering from the effects of mercury poisoning
(d) The hemoglobin of the babie's blood
is in a very reduced state
206. Most common cause of death in children< 5 year age is:
(UPSC/01)
(a) Respiratory infection
(b) Diarrhoea
(c) Prematurity
(d) Accidents
207. Water content in infant:
(Orissa 01)
(a) 60-70%
(b) 75-80%
(c) 80-90%
(d) > 90%
208. Gender of external genitalia of foetus becomes clearly distinguished by:
(Orissa 01)
(a) 10 weeks
(b) 16 weeks
(c) 12 weeks
(d) 20 weeks
209. which one of the following pairs of diseases and features is not correctly matched?
(UPSC/01)
(a) Acute glomerulonephritishypertension
(b) Nephrotic syndromepneumococcal peritonitis
(c) Kwashiorkor pulmonary oedema
(d) Hepatic encephalopthyhypoglycemia
210. Consider the following intracranial haemorrhages:
(UPSC/01)
1. Intraventricular
2. Subdural
3. Subarachnoid
Which of these types of haemorrhages in a newborn are due to anoxia?
(a) 1, 2 and 3
(b) 1 and 2
(c) 2 and 3
(d) 1 and 3
198 A
199 B
200 E
201 C
202 B
203 C
204 D
205 B
206 A
207 B
208 A
209 C
210 D
POINTS TO PONDER
In born Errors of Amino Acid Metabolism Associated with Abnormal Odor
Inborn Error of Metabolism
Urine Odor
Characteristics
1.
2.
3.
4.
Loss
Loss
Loss
Loss
211 D
212 C
of
of
of
of
213 B
214 D
215 A
216 D
217 B
218 D
Self-assessment / 128
Paralysis About 12 to 21% of the cases suffer from the post-diphtheretic paralysis somewhere
between first and sixth weeks of illness. The following are the various types seen:
a. Pharyngeal and palatal paralysis are manifested by nasal voice, dysphagia, nasal regurgitation,
and failure to life the palate.
b. Ocular paralysis occurs late and is second in frequency. It is manifested in the form of diplopia,
squint, ptosis, ophthalmoplegias, etc.
c. General paralysis occurs quite late. There may be quadriplegia, paralysis of the neck muscles
and respiratory embarrassment, as a result of involvement of the diaphragm from phrenic nerve
paralysis. Even CSF protein may be elevated. This complication closely simulates GuillainBarr syndrome.
Vasomotor disturbances in the form of hypotension and cardiac failure 2 to 3 weeks after onset of
the disease occur rarely.
Gastritis
Hepatitis
World Health Organization (WHO) Duidelines for Identifying Severe Malaria
More than 2% erythrocytes infected with malarial parasites
Asexual parasite count 100,000/mm3 of blood
High fever with body temperature 105F (40.5C) and above
Severe anemia with hemoglobin under 5 g%
Hematocrit (PCV) 30%
Blood urea 55 mg
Acute hepatopathy and clinically detectable jaundice
Cerebral malaria (headache, mental disturbance, neurologic signs, convulsions, delirium, coma)
Renal complications like acute renal failure, acute tubular necrosis, dark urine, etc.
Hypoglycemia
Noncardiogenic pulmonary edema, shock lung syndrome
Hypovolemia, hypotension, feeble and rapid pulses, pale and clammy skin
Cardiac dysrhythmias
Secretory diarrhea and dysentery
Splanchnic capillary blockade (melana)
Other complications: Rupture of spleen, pyogenic pneumonia, miliary tuberculosis, septicemia,
symptoms resembling hemolytic anemias
Self-assessment / 130
2.6%
1.0%
5 to 10%
25%
25%
Upto 75%
20%
Organism
Staphylococcus
Pseudomonas
Listeria
Streptococcus group B
Defects/Deficiencies
Inheritance
Manifestations
MPS IH (Hurlers
syndrome)
-iduronidase
Recessive
MPS IS
(Scheie syndrome)
-iduronidase
(partial)
Recessive
MPS II (Hunters
syndrome)
Iduronosulfate
sulfatase
X-linked
recessive
Heparin
sulfate sulfatase
Recessive
N-acetyl-D-glucosaminidase
Recessive
MPS IV
(Marquio syndrome)
Excess production
of keratosulfate
Recessive
MPS VI
(Maroteaux-Lamy
syndrome)
Maroteaux-Lamy
corrective factor
Recessive
MPS VII
(Sly syndrome)
-glucuronidase
Recessive
None
Self-assessment / 132
Neonatal screening (Infant heel puncture blood sample) includes
Tests for
1. Hypothyroidism
2. Phenylketonuria
3. Galactosemia
4. Maple syrup urine disease
5. Homocystinuria
6. Biotinidase deficiency
7. Adrenal hyperplasia
8. Hemoglobinopathy
9. Cystic fibrosis
10. Tyrosinemia
11. Aminoacidopathies
Conditions associated with antinuclear antibodies
Systemic lupus erythematosus
Drug induced lupus
Juvenile arthritis
Juvenile dermatomyositis
Vasculitis syndrome
Scleroderma
Infectious mononucleosis
Chronic active hepatitis
Hyperextensibility
Autoantibodies found in systemic
Antibody
1. Coombs antibodies
2. Antiphospholipid antibodies
3. Lupus anticoagulant
4. Antithyroid antibodies
5. Antiribosomal P antibody
lupus erythematosus
Manifestation
Hemolytic anemia
Antiphospholipid antibody syndrome
Coagulopathy
Hypothyroidism
Lupus cerebritis
219. Child with fever, convulsion CSF-protein-150 mg, glucose-40 mg%, chloride-520, lymphocytosis; diagnosis is:
(AIIMS 93)
(a) Tubercular meningitis
(b) Viral enchephalitis
(c) Pyogenic meningitis
(d) Viral meningitis
220. All of the following are neural tube defects; except:
(a) Myelomeningocele
(b) Anencephaly
(c) Encephalocele
(AI/04) (NEL/1983)
(d) Holoprosencephaly
219 A
220 D
(AI/04)
(d) Congenital adrenal
222 D
Non-malignant
1.
2.
3.
(AI/04)
Self-assessment / 134
3. Ewings sarcoma
4. Rhabdomyosarcoma
5. Teratomas
4. Others
1. Breast carcinoma
2. Bronchial carcinoma
4.
3. Congenital neutropenia
4. Glanzmanns thromboasthenia
5. Osteopetrosis
Non-hematological genetic disorders
1. Mucopolysaccharaidosis
2. Leukodystrophy
3. Lysosomal disorders
4. Other metabolic disorders
(AI/04)
(c) Chromosomal breakage
Ataxia Telangiectasia
It is an AR inherited disease
Clinical manifestations:
Progressive cerebellar ataxia
Oculocutaneous telangiectasia
Choreoathetosis
Pulmonary and sinus infections
Thymic hypoplasia with cellular and humoral (Ig A and IgG2) immunodeficiencies
Endocrine disorders
Lymphoreticular malignancies
Begins beween 12-15 months
Progresses relentlessly; child wheel chair bound by 10-12 years
Neuroimagingcerebellar atrophy
224. A 12-year-old girl with tremors and emotional lability has a golden brown discoloration of Descemets membrane.
The most likely diagnosis is:
(AI/04)
(a) Fabrys disease
(b) Wilson disease
(c) Glycogen storage disease
(d) Acute rheumatic fever
225. An infant develops cough and fever. The X-ray examination is suggestive of broncho-pneumonia. All of the
following viruses can be the causative agent except:
(AI/04)
(a) Parainfluenza viruses
(b) Influenza virus A
(c) Respiratory syncytial virus
(d) Mumps virus
226. A two-year-old boy has vitamin D resistant rickets. His investigations revealed serum calcium 9 mg/dl,
phosphate 2.4 mg/dl, alkaline phosphatase 1041-IU, normal intact parathyroid hormone and bicarbonate 22
mEq/L. Which of the following is the most probable diagnosis?
(AIIMS/04)
(a) Distal renal tubular acidosis
(b) Hypophosphatemic rickets
(c) Vitamin D dependent rickets
(d) Hypoparathyroidism
223 B
224 B
225 D
226 B
(1) Daptomycin
(2) Gemifloxacin
(3) Nitazoxanide
(4)
(5)
(6)
(7)
Sertaconazole
Alefacept
Aripiprazole
Atomoxetine
(8) Memantine
(9) Enfurvirtide
(10) Aprepitant
(11) Palonosetron
(12) Bortezomib
(13) Tositumomab
and Iodine I 131
(14) Geftinib
(15) Rosuvastatin
(16) Pegvisomant
(17) Adalimumab
227 A
228 A
229 B
231 A
232 A
Self-assessment / 136
(18)
(19)
(20)
(21)
(22)
(23)
(24)
Lumiracoxib
Teriparatide
Ibandronate sodium
Abarelix
Vardenafil
Tadalafil
Epinastine