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PEDIATRICS
CARDIOLOGY
(1)CARDIOLOGY
MYOCARDITIS:
Is an inflammation of the myocardium, caused by: infections, toxins, and autoimmune diseases.
Viral infection ( enteroviruses in particular) the most common cause of myocarditis in children.
Classically occurring in spring and summer, myocarditis develops more often in infants and young
children than in older children and adolescents.
Leads to tissue necrosis, thereby worsening myocardial function, dilating the heart, and increasing end
diastolic volume with resultant pulmonary edema and CHF.
Myocarditis typically presents as a flulike illness: fever, anorexia, lethargy, and irritability followed by
respiratory distress from acute heart failure.
A holosystolic murmur may be identified secondary to dilated cardiomyopathy and the resulting
functional mitral regurgitation.
Hepatomegaly can develop due to passive congestion Other features of heart failure include fatigue,
tachypnea, tachycardia, and decreased perfusion.
Affected children should be monitored in the intensive care unit because of the risk of acute
decompensation and fatal arrhythmias.
The workup for suspected myocarditis includes a (CBC) with differential, ESR and C-reactive protein,
cardiac enzymes, and blood and viral cultures.
A chest radiograph demonstrates cardiomegaly and pulmonary edema.
Electrocardiography most commonly shows low voltage QRS and sinus tachycardia.
Echo.is the best means of evaluating myocardial function and often shows global hypokinesis, left vent.
hypertrophy, left vent. dysfunction & pericardial effusion.
The gold standard for diagnosis is a myocardial biopsy, which also allows for disease stage
classification.
(1)CARDIOLOGY
PEDIATRICS
Is the most common cyanotic congenital disease in children less than 4 years.
Patients often present with cyanotic spells.
The classic tetrad includes:
(1)subpulmonary stenosis (single S2), (2) overriding aorta, (3) ventricularseptal defect (VSD)
(pansystolic murmur), and (4) right ventricular hypertrophy.
Because a large unrestrictive VSD is always present, the right ventricular pressure is the same as the
left ventricular and aortic pressures.
Pulmonary artery pressure and flow are inversely proportional to the degree of subpulmonary
obstruction.
The degree of cyanosis correlates precisely with the degree of pulmonary stenosis.
Untreated patients with TOF are at risk for "hypercyanotic" or "tet" spells.
These spells are often precipitated by exertion (such as feeding) and are characterized by irritability,
cyanosis, and tachypnea.
The exact etiology of these episodes is unclear, it may be predisposed by peripheral vasodilation,
hyperventilation, and/or contractility of the Rt. ventricular outflow tract.
Regardless of the etiology, however, an increased right to left shunt across the ventricular septal defect
develops, causing a decrease in pulmonary blood flow and increased systemic venous return of
deoxygenated blood leading to worsening cyanosis.
If untreated, "tet" spells can lead to syncope or death.
The treatment of a "tet" spell is to place the child in a knee to chest position, which increases systemic
vascular resistance resulting in increased blood flow from the right ventricle to the pulmonary
circulation.
Morphine and an intravenous fluid bolus can also be given to increase pulmonary blood flow.
Oxygen is of little benefit because the abnormality is decreased pulmonary blood flow, not insufficient
oxygenation
A cyanotic heart disease which presents with cyanosis in the first 24 hours of life.
It is commonly seen in infants of diabetic mothers and in males.
The aorta arises from the right ventricle, and the pulmonary artery from the left ventricle.
As a result, deoxygenated blood coming from the body goes to the right atrium and ventricle and is
cycled back to the body through the aorta.
Oxygenated blood from the lungs is returned to the lungs by the left side of the heart through the
pulmonary artery.
During intrauterine life, the foramen ovale and ductus arteriosus provide mixing of the deoxygenated
and oxygenated blood, resulting in an almost normal oxygenation of the fetal circulation.
A patent foramen ovale or ductus arteriosus can maintain life after birth, as seen in a patient with
normal Apgar scores.
The foramen ovale and ductus arteriosus begin to close after birth, leading to decreased mixing and
poor oxygenation.
(1)CARDIOLOGY
As the aorta is located in front of the pulmonary artery, the S2 aortic component is better heard than
the S2 pulmonic valve component and is audible as a single S2.
There is usually no murmur because the foramen ovale and ductus arteriosus have closed.
The neonate then presents with cyanosis and tachypnea within the first few hours of life.
Chest x-ray can be initially normal but show some evidence of increased pulmonary blood flow after 12 weeks.
Echocardiography confirms the diagnosis.
Prostaglandin E1 can be intravenously given to stabilize the neonate by maintaining the patency of the
ductus arteriosus, which is important for survival.
Surgical treatment is definitive.
.N.B:
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A congenital defect characterized by stenosis of the aorta, usually near the ductus arteriosus. present
with hypertension in the upper part of the body (high BP in the arms) and relative hypoperfusion in the
lower part of the body (low BP in the legs).
The prevalence of COA in patients with Turner's syndrome is approximately 7%.
The clues to the diagnosis are occasional headaches and increased BP in both arms, leg muscle
fatigability while climbing stairs (due to lower body hypoperfusion).
And a mild, continuous murmur heard all over the chest (due to the development of collaterals between
the hypertensive and hypoperfused vessels).
Rib notching caused by the dilatation of the collateral chest wall vessels is specific for coarctation.
MITRAL STENOSIS:
also causes a diastolic rumble but does not typically cause a pansystolic murmur at the left lower
sternal border unless there is tricuspid regurgitation as well.
Other features to suggest rheumatic fever such as fever, arthritis, or skin lesions.
TRICUSPID ATRESIA:
(1)CARDIOLOGY
PEDIATRICS
Patients with normal arteries (without TGA) present in the first two weeks of life with progressive
cyanosis, tachypnea, easy fatigability, and poor feeding.
EKG reveals left ventricular hypertrophy, which occurs since the left ventricle handles both pulmonary
and systemic venous returns.
Echocardiography shows a fibromuscular membrane in the place of the tricuspid valve, a variably small
right ventricle, ventricular septal defect, and a large left ventricle.
The treatment generally includes PGE1 (to keep the ductus arteriosus open) and balloon atrial
septostomy (if the ASD is not large enough to allow an adequate flow from the right to left atrium)
POSTPERICARDIOTOMY SYNDROME:
It is reactive pericarditis with a pericardial effusion that present after surgery for congenital heart
disease.
Symptoms usually occur 1-6 weeks after surgery.
Although the cause is not known, it is thought to be an autoimmune response, possibly to a viral
infection.
Most children develop mild symptoms which are self-limited. In infants, pericardial effusions can
present with abdominal pain, vomiting, and decreased appetite.
Older children may complain of chest pain Fever is often present On examination, findings are
consistent with pericardial and/or pleural inflammation, including tachycardia, poor perfusion, pulses
paradoxus, distant heart tones, and jugular venous distention.
If the effusion is large enough to affect the function of the heart , pericardial tamponade can occur.
N.B:
Most cardiac murmurs in childhood are benign (innocent) and require only observation. Know
the following features of a benign murmur:
Asymptomatic patient
Normal S2
No audible clicks
Normal pulses
No other abnormalities.
Pathological murmurs may indicate or lead to congenital heart disease, and are very likely
when the patient has any of the following:
symptomatic patient
Pansystolic murmur
RHEUMATIC FEVER:
Suspect rheumatic fever in a child with sore throat, low-grade fever, pericarditis, arthritis, chorea and
subcutaneous nodules.
Other features include erythema marginatum, elevated ESR and prolonged PR interval on EKG.
Rheumatic fever is caused by is group A streptococcus.
(1)CARDIOLOGY
Diagnosis is made if two major or one major and two minor criteria (Jones criteria) are satisfied, in
addition to evidence of recent streptococcal infection (via detection of antistreptolysin antibodies,
cultures and other various antigen detection kits).
Jones criteria:
The five major criteria are:
Polyarthritis.
Carditis.
Chorea.
Subcutaneous nodules.
Erythema marginatum.
The three minor criteria are:
Fever.
Arthralgia.
Previous rheumatic fever.
The treatment is benzathine penicillin G.
Patients with chorea, pericarditis and arthritis may require additional therapy with anti-epileptics,
salicylates, and codeine, respectively.
JERVELL-LANGE-NIELSON SYNDROME:
Characterized by an accessory pathway between the atrium and ventricle resulting in pre-excitation
and an increased risk for tachyarrhythmias.
WPW is usually seen in otherwise healthy children or adolescents.
Affected patients can either be asymptomatic or present with chest pain, palpitations, syncope, or
cardiac arrest.
In WPW, an accessory pathway conducts antegrade from the atria to the ventricles faster than the
conduction through the AV node, which allows part of the ventricle to depolarize early.
This results in the classic EKG findings of a shortened PR interval, a slurred initial portion of the QRS
(the delta wave), and a widened QRS complex.
If there is retrograde conduction from the ventricles to the atria, supraventricular tachycardia (SVT) can
result.
The EKG features of WPW will not be apparent during episodes of SVT, but the delta wave should be
visible once the rhythm is slowed.
Sudden death can occur from dysrhythmias if WPW is untreated.
(1)CARDIOLOGY
PEDIATRICS
Usually associated with a continuous flow murmur due to constant movement of blood from the highpressure aorta to the low-pressure pulmonary artery.
Small PDAs are often asymptomatic and detected incidentally on routine cardiac auscultation.
Physical examination is often unremarkable in small PDA, although mildly accentuated peripheral
pulses can occur.
(1)CARDIOLOGY
ENT
(2)ENT
EPIGLOTTITIS:
ALLERGIC RHINITIS:
N.B:
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The most common predisposing factor for acute bacterial sinusitis is a viral upper respiratory
infection.
Suspect choanal atresia in an infant who presents with cyanosis that is aggravated by feeding and
relieved by crying.
Failure to pass a catheter through the nose 3-4 cm into the oropharynx is suggestive of the diagnosis.
The diagnosis is confirmed by CT scan with intranasal contrast.
First step in management consists of placing an oral airway and lavage feeding.
Definitive treatment involves repairing the obstruction with surgery or endoscopy.
An acute onset of headaches and focal neurologic symptoms after an episode of acute otitis media or
sinusitis most likely has a brain abscess.
Hypothermia, hypotension.
Ring-enhancing lesions are usually seen on CT or MRI scan.
(2)ENT
PEDIATRICS
Streptococcus pneumonia is the most common cause of acute sinusitis in childhood, followed by
Haemophilus influenza and Moraxella catarrhalis.
Staphylococcus aureus and anaerobes are common etiologic agents of chronic sinusitis, and are rarely
seen in acute cases.
Diagnosis of acute bacterial sinusitis for children <6 years of age should be based on clinical rather
than radiographic criteria.
The treatment is antibiotics, in uncomplicated sinusitis, the treatment of choice is amoxicillin 45-50
mg/kg/day.
CHOLESTEATOMAS:
Cholesteatomas in children can either be congenital or acquired secondary to chronic middle ear
disease.
Congenital cholesteatomas typically found in younger patients around the age of five.
New-onset hearing loss or chronic ear drainage despite antibiotic therapy are typical presenting
symptoms of acquire cholesteatomas, and granulation tissue and skin debris may be seen within
retraction pockets of the tympanic membrane on otoscopy.
Complications of cholesteatomas include hearing loss, cranial nerve palsies, vertigo, and lifethreatening infections as brain abscesses or meningitis.
Should be referred to an otolaryngologist for a dedicated otologic exam, possibly accompanied by a
CT and/or surgical visualization to confirm the diagnosis.
The most common causative organism is Streptococcus pneumoniae (40% of cases), followed by nontypable Haemophilus influenzae & Moraxella catarrhalis.
Acute otitis media should be considered in any patient with symptoms of ear drainage and difficulty
hearing.
Ear pain is also common, but may be absent in young patients.
Nonspecific systemic symptoms: fever, irritability, and diarrhea can sometimes be present.
The first-line treatment is 10 day course of amoxicillin.
Any adolescent with a nasal obstruction, visible nasal mass, and frequent epistaxis is considered to
have a juvenile angiofibroma (JNA) unless proven otherwise.
This is typically found in the back of the nose or upper throat (nasopharynx) of adolescent boys.
It is a benign growth, but is capable of eroding and locally invading.
Removal is often difficult because the tumor is unencapsulated and may be deeply invasive.
Recurrence of the tumor after surgical resection is common.
RETROPHARYNGEAL ABSCESSES:
Present with: fever, irritability, and fatigue along with a sore throat, dysphagia, trismus, a muffled or
"hot potato" voice, and/or neck stiffness.
On examination, unilateral cervical lymphadenopathy and decreased range of motion of the neck are
common.
(2)ENT
The oropharynx may be erythematous and a unilateral pharyngeal bulge may be present if the child is
able to open the mouth.
Respiratory distress, if present, is concerning for airway obstruction.
Diagnosis can be confirmed with either plain X-ray or a CT scan of the neck.
Lateral neck X-ray often show widening of the soft tissue space between the trachea and the spine.
Due to false +ve & -ve results of X-ray, so the diagnosis should be confirmed by CT, which will delineate
the abscess and help distinguish between an abscess and cellulitis.
N.B:
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CROUP:
(2)ENT
PEDIATRICS
OPHTHALMOLOGY
(3)OPHTHALMOLOGY
NEONATAL CONJUNCTIVITIS:
Gonococcal conjunctivitis: A copious amount of purulent drainage in newborns who are 2-5 days old
is most consistent with gonococcal conjunctivitis.
Chlamydia trachomatis conjunctivitis: caused by Chlamydia trachomatis is usually milder and presents
> 5 days after birth.
Chemical conjunctivitis can result from prophylactic silver nitrate eye drops, but it is usually fairly mild.
VIRAL CONJUNCTIVITIS:
CHEMICAL CONJUNCTIVITIS:
It is the most common cause of a red eye that presents within the first 24 hours of life.
Approximately 80% of patients who receive silver nitrate prophylaxis (to prevent gonococcal
conjunctivitis) experience mild conjunctival irritation and tearing that generally resolve within 24 hours.
GONOCOCCAL CONJUNCTIVITIS:
is acquired through contact with infected genital secretions, and occurs 2-5 days after birth.
It presents as a hyperacute and highly purulent conjunctivitis with copious, purulent eye discharge with
swollen eyelids and chemosis.
It is the most destructive neonatal eye infection, since it may result in corneal perforation and
permanent blindness if left untreated.
The treatment is ceftriaxone.
CHLAMYDIAL CONJUNCTIVITIS:
Develops a few days to several weeks after birth ( > 5 days after birth).
Manifests with conjunctival congestion, edema, and mucoid or frank purulent discharge.
Pneumonia usually appears 3 to 19 weeks after birth, and symptoms include cough, tachypnea, and
rales; there is no fever, and wheezing is rare.
The latter two features permit to differentiate chlamydial pneumonia from RSV bronchiolitis.
The treatment is oral erythromycin for 14 days, as topical agents are ineffective.
TRACHOMA:
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(3)OPHTHALMOLOGY
RETINAL HEMORRHAGES:
Common findings in child abuse. Multiple retinal hemorrhages that differ in size and color support the
suspicion of child abuse.
A precipitating factor (stepfather/mother, financial issue) is usually present in the history In addition,
there may be bruises that usually have associated swelling and tenderness.
In this case, nothing in the history and physical findings is suggestive of child abuse; therefore,
reporting to the authorities is not necessary.
ORBITAL CELLULITIS:
Is an infection that is posterior to the orbital septum which is unilateral and more common in children
with paranasal sinus infection.
Patients present with an abrupt onset of fever, proptosis, restriction of extraocular movements and
swollen, red eyelids.
Differentiating preseptal cellulitis and orbital cellulitis is important in any patient who presents with a
swollen and painful eye accompanied by fever.
Pain with eye movement is more suggestive of orbital cellulitis it can occur in preseptal cellulitis as
well.
Both proptosis and decreased visual acuity are almost always seen in orbital cellulitis.
CT can be used to confirm the extent of infection and to identify a potential abscess.
Treatment requires intravenous broad-spectrum antibiotics.
PRESEPTAL CELLULITIS:
Is an infection of the soft tissue anterior to the orbital septum, that is most commonly caused by
contiguous spread of infection from local facial or eye lid trauma.
It is often present with: eyelid swelling, tenderness, erythema or discoloration.
(3)OPHTHALMOLOGY
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PEDIATRICS
The presence of decreased eye movements, decreased visual acuity, proptosis or double vision is
more suggestive of orbital cellulitis.
STRABISMUS:
an esodeviation (medial deviation of the eye), which is the most common type of strabismus,
representing over 50% of all cases.
Strabismus is the most common cause of amblyopia.
The cover test: asking the child to fix his sight on a target as the examiner alternately covers one eye
while observing the movement of the other.
A normal eye keeps the same position and does not move, whereas a misaligned eye shifts to refixate
the object when the normal eye is covered.
The standard of treatment is occlusion of the normal eye.
This procedure forces the affected eye to correct itself in order to be properly functional, thus
stimulating proper visual maturation.
In case the amblyopia is secondary to abnormalities of refraction, appropriate lenses should be
prescribed so as to have a well-formed retinal image.
The normal or less severely affected eye is then covered.
If the amblyopia is caused by an opaque media, surgical removal of the media should be performed
.before occlusion therapy.
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(3)OPHTHALMOLOGY
NEUROLOGY
(4)NEUROLOGY
NIGHT TERRORS:
N.B.:
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MIGRAINE HEADACHES:
are the most common cause of headaches in the pediatric population, occurring in 50% of cases before
the age of 20 years.
The aura can be visual (e g , blurred vision, scotoma, photophobia, distortion of objects) or sensory (e
g perioral paresthesia, numbness of extremities, shoulder pain, abdominal pain, otalgia).
Migraine headache is a clinical diagnosis, and further investigations are not usually indicated.
Neuroimaging is required only when there are behavioral changes, decline in school performance,
decline in growth parameters, early morning headaches, seizures, or abnormal neurologic findings.
In complicated migraines, neurologic signs (e g , basilar signs, ophthalmoplegia, or hemiparesis) may
be present, although the work-up reveals nothing abnormal.
These neurologic symptoms may last only during the headache or persist for several days or weeks.
BRAIN ABSCESSES:
can present with fever, headache, focal neurologic changes, seizure, spasticity, or signs of increased
intracranial pressure.
Congenital heart disease (particularly right-to-left shunts), head trauma, infections of the jaw or mouth,
infections of the face or scalp, meningitis, and cranial instrumentation increase the risk of developing
a brain abscess.
(4)NEUROLOGY
13
PEDIATRICS
Minor motor symptoms are common, but complex automations and clonic activities do not occur.
Best diagnosed with EEG studies.
An EEG with activation procedures (hyperventilation, photic stimulation, sleep) helps in further
diagnosis and classification of seizures.
Ethosuximide is used almost exclusively for childhood absence seizures & valproic acid is the 2nd
choice.
N.B:
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SEIZURES:
TODD'S PARALYSIS:
Represents a postictal condition that usually rapidly improves with restoration of motor function within
24 hours.
May follow generalized as well as focal seizures.
The exact pathophysiology of this condition is not well understood, but most probably involves
alteration in neuronal electrical activity.
It indicates that a structural abnormality underlying the seizure is present.
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(4)NEUROLOGY
Is a subperiosteal hemorrhage; hence, it is always limited to the surface of one cranial bone.
There is no discoloration of the overlying scalp, and swelling is usually not visible until several hours
after birth because subperiosteal bleeding is a slow process.
Most cases do not require any treatment and resorb spontaneously within 2 weeks to 3 months,
depending on the size.
Rarely, phototherapy may be necessary to improve the hyperbilirubinemia.
PARINAUD'S SYNDROME:
Consists of paralysis of vertical gaze that may be associated with pupillary disturbances and eyelid
retraction (Collier's sign).
The lesion is on the rostral midbrain at the level of the superior colliculus and CN III.
It is most commonly caused by germinomas and pinealomas at this region.
The endocrine syndrome results from interruption of hypothalamic inhibiting pathways, and sometimes
beta-hCG secretion and consequent Leydig's cell stimulation.
N.B.:
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In 90% of cases, medulloblastoma arises from the cerebellar vermis, and is not associated
with Parinaud's syndrome.
Neuroblastoma is the third most common malignancy in the pediatric population, It arises
mostly from the adrenal glands and sympathetic paraspinal ganglia.
Although craniopharyngioma can also cause an endocrine syndrome, it results in visual field
defects owing to its sellar location.
(4)NEUROLOGY
15
PEDIATRICS
are episodes of apnea that are sometimes associated with a loss of consciousness, and are
precipitated by frustration, anger, or pain.
These episodes usually occur in children ages 6-18 months.
Classically, the child gets frustrated and holds their breath, which may lead to loss of consciousness
followed by spontaneous resolution.
Unlike a seizure, there is no incontinence or postictal phase associated with breath holding spells.
These spells can be simple, cyanotic, pallid, or complicated.
In a simple breath holding spell, there is no change in circulation or oxygenation.
Cyanotic spells are associated with cyanosis during the spell, and may be associated with hypoxia and
decreased blood flow to the brain.
However, children with cyanotic breath holding spells usually have no long-term adverse effects from
the episode.
Pallid spells usually occur after painful events or minor trauma, and the affected child has pallor.
Complicated breath holding spells are a more severe form of the cyanotic or pallid types with
associated seizure-like activity However, EEG is normal.
Breath holding spells are diagnosed based on the clinical history.
Since iron deficiency anemia may be associated with breath holding spells, patients are generally
tested to rule-out anemia as a predisposing cause.
The most important step in managing affected patients is reassuring the parents that the episodes are
not harmful and will not have any long-term effects.
No treatment is necessary for breath holding spells, and affected children will eventually stop having
episodes.
.Anemic patients, however, should be treated with iron supplementation.
BRAIN TUMORS:
In the pediatric population, CNS tumors are the most common solid tumors and the second most
common malignancies (after leukemias).
Approximately 60% of these tumors are infratentorial, 25% are supratentorial, and 15% arise in the
midline.
Astrocytomas are the most common histologic type for both supratentorial and infratentorial groups.
MEDULLOBLASTOMA:
Is the second most common posterior fossa tumor (after cerebellar astrocytoma) in children.
It is highly radiosensitive and can metastasize through the CSF tract Over 90% of medulloblastomas
develop in the vermis.
This patient has posterior vermis syndrome (truncal dystaxia & horizontal nystagmus).
CRANIOPHARYNGIOMA:
A young boy with symptoms of increased intracranial pressure ( headaches, vomiting), bitemporal
hemianopia, & a calcified lesion above the sella has a craniopharyngioma until proven otherwise.
Craniopharyngiomas are derived from epithelial remnants of Rathke's pouch.
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(4)NEUROLOGY
It is not a true pituitary tumor, and is characterized by nests of squamous cells in a loose stroma,
resembling the appearance of embryonic tooth bud enamel.
Its location is suprasellar and inferior to the optic chiasm, which is why it can cause bi-temporal
hemianopia (by pressing the optic chiasm).
Associated endocrine symptoms such as diabetes insipidus, and growth failure associated with either
hypothyroidism or growth hormone deficiency may occur.
The diagnosis is usually made by (CT) or (MR): Presence of a cystic calcified parasellar lesion on MRI
is almost diagnostic of craniopharyngioma.
Surgical removal is the treatment of choice.
pituitary adenoma may present with similar symptoms; however, the condition is more frequent in
women.
Furthermore, prolactinoma is an important part of the syndrome, and calcification of the gland does not
occur.
NEUROCUTANEOUS SYNDROMES:
Type 1 neurofibromatosis:
Characterized by cafe-au-lait spots, axillary freckles, Lisch nodules of the iris, neurofibromas, and bony
lesions.
Type 2 neurofibromatosis:
Characterized by a congenital unilateral cavernous hemangioma along trigeminal nerve distribution &
intra-cranial calcifications that resemble a tramline in X-ray.
Also present with: focal or generalized seizures, mental retardation, and a port wine stain or nevus
flammeus along the branches of trigeminal nerve.
Other pertinent findings are hemianopia, hemiparesis, hemisensory disturbance, and ipsilateral
glaucoma.
Treatment is aimed at controlling the seizures and reducing intraocular pressure Argon laser therapy
is successful in removing the skin lesions.
Tuberous sclerosis:
N.B.:
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(4)NEUROLOGY
17
PEDIATRICS
STROKES IN CHILDREN:
are uncommon but When they occur, they are often the result of a congenital abnormality, infection, or
systemic illness.
Many childhood strokes are caused by sickle cell anemia, and suspected by (patient's race, history of
dactylitis, and prior pain crisis are all consistent with sickle cell disease).
The mechanism of strokes in children with sickle cell anemia is not fully understood, but RBCs
adherence to the endothelium, activation of van Willebrand's factor, and hyperviscosity are all thought
to contribute.
Children who have trauma to the soft palate with a sharp object can either compress the internal carotid
artery (causing a thrombosis that embolizes to the brain and causes stroke) or dissect the internal
carotid artery (leading to ischemic stroke).
The onset of symptoms can be delayed up to 24 hours after the traumatic episode.
The diagnosis should be suspected clinically and can be confirmed with MRl/MRA of the brain.
Treatment is somewhat controversial and ranges from close observation with supportive care to
aggressive treatment with anticoagulation, with similar outcomes.
EPIDURAL HEMATOMAS:
usually caused by injury to meningeal blood vessels & do not usually cross suture lines because of the
better dural attachment at the sutures.
The classic history of an epidural hematoma consists of a direct head injury followed by a lucid interval
and then rapid neurologic deterioration with headache, vomiting, seizures, confusion, and lethargy.
Affected patients can progress to coma and death due to herniation if not treated.
Infants with open fontanelles may present with bulging fontanelles, irritability, seizures, and hypotonia.
Older children and adolescents usually present with more classic symptoms of headache, vomiting,
and deteriorating mental status.
CT scan of the head is the diagnostic test of choice --> a biconvex mass.
The key to evaluating a child with an epidural hematoma is to search for the presence of clinical
features that necessitate an emergent craniotomy. which include any of the following: GCS < 8, signs
of increased ICP, pupillary abnormalities, hemiparesis, or cerebellar signs.
FEBRILE SEIZURE:
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(4)NEUROLOGY
A complex febrile seizure is diagnosed if the seizure is focal, lasts >15 minutes, or occurs more than
once with a total duration of seizure activity >30 minutes.
Children with complex febrile seizures are at increased risk for future febrile or afebrile seizures,
especially if there is family history of epilepsy in a parent or sibling.
Febrile seizures are associated with viral or bacterial infections (e.g. otitis media) However, serious
intracranial infections (e.g. meningitis) must be ruled out.
Once an intracranial infection has been ruled out, the patient can be treated for the fever and underlying
infection.
The patient can then be discharged home with parental education about the use of antipyretics and
seizure precautions.
CEREBRAL PALSY:
HYDROCEPHALUS:
GUILLAIN-BARRE SYNDROME:
Suspect Guillain-Barre syndrome in a child who presents with an ascending polyneuropathy one week
after an apparent viral infection (e.g. diarrhea).
The underlying pathology involves mainly the peripheral motor nerves, although sensory and
autonomic nerves may also be affected.
FRIEDREICH ATAXIA:
an autosomal recessive condition and genetic counseling is recommended for prenatal diagnosis for
parents with one affected child.
FA is associated with necrosis and degeneration of cardiac muscle fibers leading to myocarditis,
myocardial fibros is and cardiomyopathy.
(4)NEUROLOGY
19
PEDIATRICS
Cardiac arrhythmia and congestive heart failure contribute to a significant number of deaths.
KLUMPKE'S PARALYSIS:
is a brachial palsy that occurs in newborns following excessive traction on the arm.
It consists of hand paralysis and ipsilateral Homer's syndrome (ptosis and miosis).
It is secondary to injury to seventh and eighth cervical nerves and first thoracic nerve.
Confirmation MRI, which demonstrates nerve root avulsion or rupture.
Treatment depends on the severity of the injury and, generally, it consists of partial immobilization and
appropriate positioning to prevent contractures.
Gentle massages and range-of-motion exercises can be started by 7-10 days of age.
If by 3-6 months there is no improvement, neuroplasty, neurolysis, end-to-end anastomosis, and nerve
grafting may be attempted.
ERB-DUCHENNE PALSY:
is another type of brachial palsy in which the injury involves the fifth and sixth cervical nerves.
It presents with: absent Moro reflex and intact grasp reflex of the affected arm.
Patients present with a characteristic position, which consist of adduction and internal rotation of the
arm with pronation of the forearm.
N.B:
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20
Dandy-Walker anomaly and Chiari malformation will both reveal CT findings consistent with
obstructive or noncommunicating hydrocephalus
. Dandy-Walker Anomaly will demonstrate a cystic expansion of the fourth ventricle, and
Chiari malformation will reveal protrusion of the structures of the posterior fossa through the
foramen magnum.
(4)NEUROLOGY
HEMATOLOGY
(5)HEMATOLOGY
FANCONI'S ANEMIA:
are more common in children, and Fanconi anemia (FA) is the most common congenital cause.
It is an autosomal recessive or X-linked disorder associated with the clinical manifestations
summarized below.
Most patients with FA are diagnosed by the age of 16 years and have a predisposition for developing
cancer.
Numerous genes, all believed to involve DNA repair, have been implicated.
Bone marrow: Aplastic anemia and progressive bone marrow failure.
Appearance: Short stature, microcephaly, abnormal thumbs, upper limb anomalies, renal
malformations and hypogonadism.
Skin: Hypopigmented/hyperpigmented areas, cafe au lait spots, and large freckles.
Eyes/ears: Strabismus, low-set ears, and middle ear abnormalities (e.g. hemorrhage, incomplete
development, chronic infections, deafness, etc ).
Diagnosis of FA is made by chromosomal breaks on genetic analysis combined with the clinical
findings.
The patient likely has bleeding secondary to thrombocytopenia, fatigue from macrocytic anemia, and
pounding in his ears from possible conduction defects or chronic hemorrhage.
The definitive treatment for aplastic anemia is hematopoietic stem cell transplantation.
Blood counts start to decrease at the age of 4-12 years.
Initial manifestations: Thrombocytopenia followed by neutropenia then anemia.
(5)HEMATOLOGY
21
PEDIATRICS
Acquired red cell aplasia which occurs in healthy children between 6 months and 5 years.
Gradual onset of symptoms as pallor and decreased activity.
Lab. --> Normocytic normochromic anemia with Hb level 3-8 mg/dl and extremely low reticulocytic
count.
The most common RBC enzyme deficiency causing anemia is (G6PD) deficiency.
In affected patients, oxidant drugs (e g , antimalarials and sulfas) and infection can cause episodic
hemolysis.
THALASSEMIAS:
22
(5)HEMATOLOGY
Characterized by chronic hemolysis of sickled cells, leading to a high RBC turnover and anemia.
Patients with sickle cell anemia will have sickle cells and reticulocytes on their peripheral blood smear.
Reticulocytes are present because the bone marrow is attempting to compensate for the chronic
hemolysis characteristic of sickle cell anemia.
Painful crisis, the most common manifestation of sickle cell anemia.
These episodes due to intermittent vaso-occlusion in connective tissue & muscle, are characterized by
severe acute pain & tenderness, fever, tachycardia, and anxiety.
Hemolysis is mainly extravascular and leads to reticulocytosis, hyperbilirubinemia and elevated serum
LDH with low serum haptoglobin.
The hematocrit is generally 20-30 percent, owing to the decreased RBC volume.
Iron deficiency anemia is not common in SCD as the hemolysis of RBC is extravascular, and the iron
is usually restored and reused; however, iron deficiency secondary to increased utilization or urinary
iron loss can be present in up to 20% of patients.
Megaloblastic anemia occurs in vitamin B12 or folic acid deficiency, which is not usually seen with
SCD; although folate deficiency secondary to increased folate utilization can be seen in some
individuals.
Anemia of chronic disease may occur in SCD if iron reuptake and use is decreased due to infection,
inflammation or some chronic illness.
Iron deficiency, folate deficiency, and anemia of chronic disease occur less frequently in SCD patients.
Sideroblastic anemia is seen in inherited or acquired defects affecting the biosynthesis of heme within
red cell precursors not related to SCD.
Salmonella and Staph aureus are the most common cause of osteomyelitis in patients with sickle cell
disease.
Pneumococcal vaccination plus penicillin prophylaxis can prevent almost all cases of pneumococcal
sepsis in patients with sickle cell anemia.
Those with sickle cell trait have some protection against malaria.
Sickle cell disease is a chronic, well-compensated hemolytic anemia with appropriate reticulocytosis.
An acute drop in hemoglobin is can occur secondary to a hyperhemolytic crisis, splenic sequestration,
or an aplastic crisis.
An aplastic crisis: a transient arrest of erythropoiesis that results in a severe drop in hemoglobin and virtual
absence of reticulocytes on peripheral smear (reticulocytes < 1%).
A hyperhemolytic crisis: is a rare complication of sickle cell disease that is characterized by sudden, severe
anemia accompanied by an appropriate reticulocytosis.
Splenic sequestration: occurs in sickle cell patients who have not yet developed auto-splenectomy.
This is caused by vasoocclusion and pooling of red cells within the spleen, which may lead to severe
hypotensive shock.
It is characterized by a dramatic drop in hemoglobin concentration that is accompanied by persistent
reticulocytosis.
The classic physical examination finding is a rapidly enlarging spleen.
(5)HEMATOLOGY
23
PEDIATRICS
Splenic sequestration carries a mortality of 10-15% and a 50% chance of recurrence, so splenectomy
is usually recommended after the first episode.
Acute chest syndrome: is characterized by the presence of fever, chest pain, and an infiltrate on chest X-ray.
The etiology of acute chest syndrome is thought to be multifactorial and related to pulmonary infarction
and infection.
While a drop in hemoglobin may be seen, severe anemia without associated reticulocytos is is not
seen in acute chest syndrome
A vaso-occlusive (painful) crisis: is characterized by acute onset of pain and is caused by vasoocclusion
secondary to the sickling of red blood cells.
N.B:
-
patient has sickle cell trait, which is characterized by a Hb S concentration ranging from 3540% (sickle cell disease Hb S > 40%).
Individuals with sickle cell trait have a heterozygous genotype (AS) and are generally
asymptomatic.
Symptoms occur very rarely, and the most common of these is painless hematuria.
In a patient with a chronic hemolytic anemia and compensatory reticulocytosis, increased
demand for folic acid can lead to folic acid deficiency,
For this reason, patients with sickle cell anemia should be on folic acid supplementation
.vitamin B12 is also required However, in contrast to folic acid, B12 stores can be adequately
maintained by most every patient via diet alone.
HEREDITARY SPHEROCYTOSIS:
24
(5)HEMATOLOGY
Laboratory studies typically show a microcytic, hypochromic anemia with a low reticulocyte
count, high ROW, low serum iron, high TI BC, and low serum ferritin.
This type of anemia can be normocytic or microcytic, and laboratory studies will show a low
serum iron and low TIBC.
In hemolytic anemia (spherocytosis), the reticulocyte counts are much higher (sometimes as high as 9%)
Spherocytosis is unlikely with just 3% reticulocyte count.
N.B.:
-
Approximately 5%- 15% of the patients with thymic tumors have pure red cell aplasia, This
finding is most common in older women.
Sugar water test is done for the diagnosis of Paroxysmal Nocturnal Hemoglobinuria (PNH).
ANEMIA OF PREMATURITY:
is the most common anemia in premature and low birth weigh infants.
The pathology involves a combination of diminished RBC production, shortened RBC life span, and
blood loss.
Iron supplementation does not prevent falling hemoglobin levels, and iron deficiency is not the cause
of anemia of prematurity.
The treatment involves iron supplementation, periodic hemoglobin checking and blood transfusion, if
needed. Erythropoietin is not routinely used.
is common in infants and toddlers who drink excessive amounts of cow's milk.
Empiric oral iron therapy is appropriate for children with microcytic, hypochromic anemia.
Term infants usually have adequate iron stores to sustain them for the first six months of life.
(5)HEMATOLOGY
25
PEDIATRICS
After six months, dietary factors (insufficient intake, malabsorption) become the most important cause
of iron deficiency.
Early introduction to or excessive intake of cow's milk is of particular concern because cow's milk is
low in iron and can cause occult intestinal blood loss in infants.
Children should not be started on cow's milk until one year of age,
MICROCYTIC ANEMIA:
caused by iron deficiency can often be differentiated from thalassemia by an elevated RDW, which is
typically greater than 20% in iron deficiency.
The reticulocyte count is low in patients with iron deficiency due to decreased erythropoiesis.
These bodies are nuclear remnants within RBCs that are typically removed by the spleen.
They are present on peripheral blood smear as single, round, blue inclusions on Wright stain.
Their presence indicates physical absence of spleen or functional hyposplenism due to splenic
autoinfarction, infiltrative disorders of spleen, or splenic congestion.
LEUKEMIA:
Leukemic patients can present with numerous infections, diarrhea and failure to thrive.
Although such patients present with more serious bacterial and fungal infections.
Hepatomegaly and splenomegaly may be found.
Laboratory studies reveal: leukocytosis, thrombocytopenia and anemia. Other findings may include
hyperuricemia,hyponatremia, elevated LDH and hypokalemia.
26
(5)HEMATOLOGY
The most common presenting sign is Painless, firm, cervical or supraclavicular adenopathy.
Depending on the extent and location of nodal and extranodal disease, there maybe symptoms and
signs of airway obstruction, pleural or pericardial effusion, hepatocellular dysfunction, or bone marrow
infiltration.
Although signs and symptoms are similar to ALL, presence of lymphoblasts makes ALL most probable.
BURKITT LYMPHOMA:
They are clonal stem cell disorders which may progress to acute leukemias.
These are usually seen in elderly patients, and are characterized by pancytopenia.
HENOCH-SCHONLEIN PURPURA:
(5)HEMATOLOGY
27
PEDIATRICS
Renal involvement relatively mild disease characterized by asymptomatic hematuria & proteinuria with
a normal or slightly elevated plasma creatinine; however,
More marked findings may occur including the nephrotic syndrome, hypertension, & acute renal failure
(treatment includes steroids and monitoring of renal function).
Other organs as the lung and central nervous system, may be involved.
Confirmation of the diagnosis requires evidence of tissue deposition in the skin or kidney of lgA by
immunofluorescence microscopy.
N.B.:
-
Minimal change disease: electron microscopy usually shows Podocyte fusion in the
glomerulous.
Membranous nephropathy: electron microscopy shows Glomerular basement thickening & is
considered the most common cause of adult nephrotic syndrome.
Focal segmental glomerulosclerosis: electron microscopy shows Focal and segmental
sclerosis.
Goodpasture's syndrome: electron microscopy shows Linear deposition of lgG on the
basement membrane is seen.
LYMPHADENITIS IN CHILDREN:
usually have fever, chills, headache, and malaise in addition to the lymphadenopathy.
In addition, tularemia is a zoonosis and presents after contact with an infected animal (e g rabbits,
hamsters, or blood-sucking arthropods).
Peptostreptococcus is an anaerobic bacteria that can cause acute, unilateral lymphadenitis However,
are one cause of unilateral subacute-chronic lymphadenopathy Affected children are usually less than
5 years old and
present with firm, nontender lymphadenopathy that is usually less than 4 cm in size.
The skin over the lymph node often thins and develops a violaceous color.
Fever and tenderness are unusual with this infection.
28
(5)HEMATOLOGY
Patients with genetic B-cell deficiencies begin to develop recurrent infections after passing 6 months
(when the newborn's levels of maternal antibodies drop).
The deficient humoral immune response in these patients impairs the body's ability to destroy
encapsulated organisms.
Hence, recurrent sinopulmonary infections with H. influenzae and S. pneumoniae are common.
Lack of lgA also predisposes to Giardia infection.
Characterized by high levels of lgM with deficiency of lgG, lgA and poor specific antibody responses
to immunizations.
HIM presents with recurrent sinopulmonary infections and Pneumocystis jiroveci pneumonia.
The unique susceptibility to opportunistic infections and neutropenia, with high lgM levels, distinguishes
HIM from XLA or other hypogammaglobulinemias.
(5)HEMATOLOGY
29
PEDIATRICS
Characterized by decreased lgG levels, normal lgA levels, and variable lgM levels.
These immunoglobulin levels all normalize by 6-11 months of age.
IgA DEFICIENCY:
Selective lgA deficiency is characterized by decreased lgA levels (less than 50mcg/ml) with normal
serum concentrations of other immunoglobulins, although lgG2 subclass deficiency may occur.
There is no other evidence of any immune defects.
Patients commonly present with respiratory, gastro intestinal, and genitourinary infections + transfusion
reaction.
Recurrent sinopulmonary (Streptococcus & Hemophilus) and GI infections (Giardia), a consequence
of lgA's role in mucosal barrier protection.
Patients with lgA deficiency may also form antibodies to lgA, predisposing them to anaphylactic
transfusion reactions.
DIGEORGE SYNDROME:
Is a defect of phagocytic cells due to dysfunction of the NADPH oxidase enzyme complex (impaired
oxidative metabolism within the phagocytes) leading to:
30
(5)HEMATOLOGY
An X-linked disorder characterized by the triad of thrombocytopenia, eczema, and recurrent bacterial
infections.
Recurrent infections are usually due to Streptococcus pneumoniae, Neisseria meningitidis, and
Haemophilus influenzae.
The thrombocytopenia is caused by decreased platelet production, and the few platelets that exist are
typically quite small.
Platelet counts in patients with WAS are typically less than 50,000/mm3 and the mean platelet volume
is also usually reduced.
Decreased platelet production is the primary pathophysiologic cause of thrombocytopenia in patients
with WAS.
An immune-mediated disorder caused by antibodies that bind to platelets and facilitate their removal
from the circulation.
Affected children typically present with easy bruisability or bleeding.
(5)HEMATOLOGY
31
PEDIATRICS
POLYCYTHEMIA:
PURPURA IN CHILDREN:
The common causes of purpura in children: thrombocytopenia, clotting factor deficiencies, and
vasculitis.
Nearly 2.5% of the normal population have a platelet count of< 150,000/?L, and clinical manifestations
of thrombocytopenia (e g petechiae, purpura) usually do not occur unless the platelet count is <
100,000/?L.
Common clotting factor deficiencies causing purpura in children: van Willebrand disease, hemophilia
A (factor VIII deficiency) & hemophilia B (factor IX deficiency).
These conditions do not usually cause abdominal pain and other renal findings (proteinuria).
If there is no thrombocytopenia (paltelets > 100.000), then vasculitis should be high in suspecious,
especially if there is no abdominal pain or renal affection.
Henoch Schonlein purpura (HSP): a small vessel vasculitis that usually presents with the classic tetrad
lower extremity palpable purpura, arthritis/arthralgias, abdominal pain, and renal disease.
HSP is the most common form of systemic vasculitis in children.
50% of patients develop HSP after upper respiratory infection & have other nonspecific symptoms as
subcutaneous edema, joint pains, bloody stools, and scrotal edema.
Small bowel intussusception is a common complication of HSP.
32
(5)HEMATOLOGY
Laboratory testing is usually normal (including a normal platelet count), although hematuria or
proteinuria may be found.
Diagnosis is usually based on clinical findings, and skin biopsy is rarely necessary.
Treatment is usually supportive, but NSAIDS are sometimes given to abdominal pain.
seen with bacterial infections (e.g. N. meningitidis, S. pneumoniae) However, patients are
usually very ill with fever, hypotension, and evidence of DIC.
IMMUNE THROMBOCYTOPENIA:
HEMOPHILIA:
Presence of spontaneous hemarthrosis and soft tissue hematomas raise the suspicion for hemophilia,
for which factor VI 11 assay is diagnostic.
Spontaneous hearthrosis is very unlikely with any other form of bleeding disorder, including Von
Willebrand's disease.
The best next step is a complete set of coagulation studies followed by factor VIII and IX levels.
Prolonged PTI, normal prothrombin time, normal bleeding time, normal fibrinogen level and low serum
factor VIII activity are the typical lab findings.
The standard treatment for hemophilia is to replace the factor VIII.
However, mild hemophilia may be treated with desmopressin (DDAVP), which causes release of factor
VIII from the endothelial cells.
An immune deffeciency disease present with: recurrent bacterial infections and necrotic periodontal
infection.
Delayed separation of the umbilical cord (>3 weeks) is characteristic and presents an important clue
to the correct diagnosis.
The leukocytes fail to express some adhesion molecules on their surface.
The leukocyte number is increased, but the inter-leukocyte communication is defective; therefore,
chemotaxis and cytotoxicity are impaired.
Normal lymphocyte count and gamma globulin concentration help to differentiate this condition from a
variety of cell and/or humoral immune defects.
(5)HEMATOLOGY
33
PEDIATRICS
It is usually preceded by an acute diarrheal illness due to the pathogens Escherichia coli serotype 0157
H7, Shigella, Salmonella, Yersinia, and Campylobacter.
It is less commonly preceded by an upper respiratory infection.
GI bleeding is common, Physical examination frequently reveals purpura and hypertension.
The hallmark finding is microangiopathic hemolytic anemia.
Other typical features: acute renal failure, fever, oliguria (or anuria) and thrombocytopenia
The peripheral smear reveal schistocytes (which represent fragmented RBCs) and giant platelets.
lntravascular hemolysis results in elevated levels of (LDH) and indirect bilirubin, as well as reticulocyte
count BUN and creatinine levels are markedly elevated.
Moderate leukocytosis may be present.
The urine contains hemoglobin, hemosiderin, albumin, RBCs, WBCs, and casts.
Suspect HUS when: a diarrheal illness and presents with acute R.F., microangiopathic hemolytic
anemia, fever, thrombocytopenia and peripheral smear of schistocytes.
N.B:
-
34
Howell-Jolly bodies: are nuclear remnants of RBCs present in sickle cell patients & are
generally removed by a functioning spleen.
Their presence in peripheral smear suggests functional asplenia in sickle cell patients (Sickle
cell patients usually have infarcted spleens by the first 18 to 36 months of life).
Heinz bodies: are aggregates of denatured hemoglobin & are commonly seen in patients with
hemolysis due to G6PD deficiency and thalassemia
Helmet cells are fragmented red blood cells, their presence is suggestive of traumatic
hemolytic conditions such as DIC, HUS and TIP.
Basophilic stippling are ribosomal precipitates which appear as blue granules & seen with
thalassemias, as well as lead or heavy metal poisoning.
(5)HEMATOLOGY
PULMONOLOGY
(6)PULMONOLOGY
PSEUDOMONAS AERUGINOSA :
Is a common cause of severe pulmonary infection in patient with cystic fibrosis (CF).
So the best treatment for a patient with severe exacerbation of cystic fibrosis is combination of
aminoglycosides (Tobramycine) and antipseudomonas penicillines (piperacillin).
N.B.: Staphylococcal aureus is a frequent cause of mild pulmonary infection in patient with cystic fibrosis SO;
when pulmonary infection is mild (cough and mucous production only) oral anti-staphylococcal antibiotic is
preferred (Dicloxacillin).
N.B.:
-
Mothers with gestational diabetes mellitus (GDM) usually give birth to large babies with the
most common complications:
Clavicular fracture which is usually greenstick fracture and heal rapidly without complications.
The characteristic laboratory findings of:
o Iron deficiency anemia are: decreased serum iron, decreased
percent of saturation (serum iron/ TIBC) & increased TIBC.
o Sideroblastic anemia: is characterized by increased serum iron
levels and normal TIBC.
o In dimorphic anemia: two distinct forms of red cells are circulating
o Megaloblastic anemia is characterized by an elevated MCV,
elevated MCH, and normal MCHC.
o Anemia of chronic disease is associated with decreased TIBC.
CYSTIC FIBROSIS:
thick, viscous secretions in the lungs, pancreas, reproductive tract, and liver.
Young child presents with recurrent upper respiratory tract infection, persistent nasal discharge and
bilateral nasal polyps.
Also can be present with recurrent upper respiratory tract infections, bronchiectasis and failure to thrive.
The patient may have pulmonary manifestations, which begins as a persistent, productive cough, and
progresses to obstructive airway disease.
A deficiency of pancreatic enzymes leads to fat and protein malabsorption, thereby causing bulky,
greasy, foul-smelling stools and subsequent deficiency of fat-soluble vitamins (A, D, E and K).
Vitamin A deficiency presents as dry skin, and Vitamin K deficiency presents as epistaxis.
Vitamin K is an important cofactor of several coagulation factors factors II , VII , IX, and X as well as
proteins C and S.
Failure to thrive is the result of chronic malabsorption from pancreatic insufficiency.
(6)PULMONOLOGY
35
PEDIATRICS
PERTUSSIS:
36
(6)PULMONOLOGY
Most often due to a double aortic arch, right-sided aorta, pulmonary sling, or anomalous innominate or
left carotid artery, causing tracheal compression.
Patients present with signs of chronic upper airway obstruction including stridor, wheezing, coughing,
and shortness of breath.
Symptoms are often worse while supine and relieved with neck extension.
Patients are commonly misdiagnosed as having reactive airway disease but will not respond to inhaled
bronchodilators and corticosteroids.
The treatment for severe disease is surgery.
ASTHMA:
N.B.
-
Tracheostomy is a surgical procedure when the upper airways are obstructed. It is nearly
always an elective procedure, and never done to relieve asthma.
Racemic epinephrine may help a little in moderate asthma, but in severe cases, its
administration will just delay the initiation of mechanical ventilation.
Racemic epinephrine has a better role in the management of croup.
Antibiotics are not indicated if there is no evidence of infection.
KARTAGENER'S SYNDROME:
N.B:
A large thymic shadow is a normal chest radiograph finding in children less than 2 years old.
Because the thymus is a soft organ, it can appear as a scalloped border.
It can also have a straight inferior border which usually appears on the right side and is called a "sail"
sign because it resembles the sail of a ship.
(6)PULMONOLOGY
37
PEDIATRICS
Occurs primarily in premature infants who are less than 28 weeks gestational age (the incidence of
RDS is inversely proportional to the gestational age).
Other risk factors: male sex, cesarean section without labor, perinatal asphyxia, and maternal diabetes.
Maternal diabetes increases the incidence of RDS in several ways Most importantly, maternal diabetes
delays the maturation of surfactant production in the lungs.
The mechanism is that fetal hyperinsulinism antagonizes the actions of cortisol and may delay the lung
maturation process.
The decreased production and secretion of surfactant is the 1ry cause.
In the absence of surfactant, atelectasis ensues and results in perfused but non-ventilated alveoli, and
consequently hypoxia.
HMD presents as: tachypnea, prominent grunting, intercostal and subcostal retractions, nasal flaring,
and duskiness within a few minutes after birth.
Breath sounds may be normal or diminished with a harsh tubular quality, & on deep inspiration, fine
rales may be heard, especially over the lung bases posteriorly.
The characteristic chest-x ray: fine reticular granularity of the lung parenchyma.
Treatment includes early mechanical ventilation and surfactant administration.
TRANSIENT TACHYPNEA:
Should be suspected in all term and post-term infants with cyanosis with or without fetal distress.
Persistence of fetal circulatory pattern of right-to-left shunting through the PDA and foramen ovale after
birth is due to very high pulmonary vascular resistance.
The associated hypoxia is universal and unresponsive to 100% oxygen.
The chest-x ray may be normal or may show parenchymal opacification in the chest, depending on the
etiology.
Although the clinical presentation of PPHN is indistinguishable from HMD, the x-ray findings can
differentiate.
38
(6)PULMONOLOGY
Usually occurs in term or post-term infants, either in utero or more often with the first breath.
Thick & particulate meconium is aspirated into lungs, resulting in small airway obstruction &
consequent respiratory distress, that present within the 1st hour of birth.
Partial obstruction of some airways may lead to pneumothorax or pneumomediastinum.
Patchy infiltrates, coarse streaking of both lung fields, increased anteroposterior diameter, and
flattening of the diaphragm characterize the typical chest-x ray
INTRACRANIAL HEMORRHAGE:
In neonates classically presents as periods of apnea, pallor or cyanosis, poor suckling, abnormal eye
signs, high-pitched, shrill cry, muscular twitching, convulsions, decreased muscle tone or paralysis,
metabolic acidosis, shock, and a decreased hematocrit.
The fontanel may be tense and bulging.
The condition is rarely present at birth and is diagnosed on the basis of the history, clinical
manifestations, and trans-fontanel cranial (CT).
BRONCHIOLITIS:
is defined as the first episode of wheezing associated with an upper respiratory tract infection.
The infection is usually caused by respiratory syncytial virus.
It affects 50% of children in the 1st 2 years of life, especially those who are prone to airway reactivity,
& there is increased incidence of asthma later in life.
The WBC count is normal, and the chest x-ray may show air trapping or atelectasis.
The treatment is supportive care with humidified oxygen and bronchodilators.
Ribavirin may be administered in high- risk children.
(6)PULMONOLOGY
39
PEDIATRICS
RHEUMATOLOGY
(7)RHEUMATOLOGY
SEPTIC ARTHRITIS:
Septic arthritis can occur following skin or upper respiratory tract infections.
Arthrocentesis is both diagnostic and therapeutic, and is mandatory in all suspected cases.
Blood cultures and synovial fluid cultures must be obtained prior to administering the antibiotics.
Empiric antibiotic therapy ( IV nafcill in or IV cefazolin) should be started with pending synovial fluid
cultures.
Presents with equinus and varus of the calcaneum and talus, varus of the midfoot, and adduction of
the forefoot.
This is a common foot deformity and may be congenital, teratologic, or positional (in utro).
Initial treatment involves, stretching and manipulation of the foot, followed by serial plaster casts,
malleable splints, or taping.
Because conservative treatment corrects the majority of cases, so Surgical treatment is indicated if
conservative management gives unsatisfactory results, and is preferably performed between 3 and 6
months of age.
Remember that the treatment of clubfoot should be started immediately.
SPONDYLOLISTHESIS:
HEMOPHILIC ARTHROPATHY:
patients with coagulopathies (Hemophilia) --> Recurrent hemarthroses may then lead to a joint injury
called "hemophilic arthropathy".
Although the exact mechanism underlying the joint damage is not known, iron deposition and synovial
thickening with fibrosis are characteristic.
Iron deposition and cytokines released from the blood stimulate synovial proliferation, fibrosis and
cartilage injury.
40
(7)RHEUMATOLOGY
TRANSIENT SYNOVITIS:
Is a frequent cause of hip pain in children, typically occurring in male children ages 3-10 years old.
The cause of transient synovitis is unknown, but the condition usually follows mild trauma or viral
infection.
Transient inflammation of the joint synovium leads to pain and decreased range of motion.
Patients usually have a history of a recent viral infection and present with a limp and/or unilateral hip
or knee pain A low grade fever may be present, but patients are generally afebrile.
On examination, the affected hip will be flexed, slightly abducted, and externally rotated, There is mild
limitation of motion.
The patient may complain of pain with palpation or passive motion.
Laboratory findings characteristic of transient synovitis include a slightly elevated white blood cell count
and erythrocyte sedimentation rate.
Four clinical criteria have been shown useful in differentiating septic arthritis from transient synovitis;
white blood cell count > 12,000/mm3; temperature > 39C (102F); erythrocyte sedimentation rate >
40 mm/h; and refusal to bear weight.
If at least three of these four criteria are met, further work-up is indicated to rule out septic arthritis.
Radiographs of the hip should be done to exclude bony lesions and Legg-Calve-Perthes disease.
Widening of the joint space or an enhanced pericapsular shadow may be seen with transient synovitis.
Additional work-up is not usually necessary, unless the diagnosis is uncertain.
Treatment consists of rest and non-steroidal anti-inflammatory medications (NSAIDs).
Most patients experience resolution of symptoms within several days, although transient synovitis may
contribute to the development of arthritis later in life.
COMPARTMENT SYNDROME:
(7)RHEUMATOLOGY
41
PEDIATRICS
N.B.:
The United States Preventive Services Task Force (USPSTF) recommends vision screening for children aged
0-5 years, primarily to identify those with strabismus, amblyopia, and refractive errors.
Early diagnosis and treatment are being emphasized, because poor visual acuity resulting from the
above conditions may impair a child's future academic performance and self image, and may even
lead to blindness.
Lead toxicity causes microcytic anemia and permanent neurologic damage, so identification of affected patients
is desirable at an early stage.
Risk factors for environmental lead exposure include minority race or ethnicity, low socioeconomic
status, pre-1950 housing, occupational exposure from parents, and recent immigration.
The USPSTF recommends no screening for asymptomatic children aged 1-5 years at average (no)
risk of lead toxicity.
The Centers for Disease Control (CDC) recommend beginning the meningococcal vaccination series between
11-12 years.
Children who are at high risk of the disease (i.e , asplenic) may be vaccinated as early as 2 years of
age.
The CDC recommends administering the rotavirus vaccine between 2 and 8 months.
Is the earliest manifestation of vaso-occlusion in sickle cell anemia, so needs a complete workup for
previously asymptomatic sickle cell patients.
Patients usually present at the age of 6 months to 2 years with an acute onset of pain and symmetric
swelling of the hands and feet.
Although only soft tissue swelling is initially seen, fever is sometimes present.
The pathophysiology of dactylitis involves vascular necrosis of the metacarpals and metatarsals, which
may be seen on plain radiographs as osteolytic lesions.
EWING'S SARCOMA:
A highly malignant tumor that is found in the lower extremity more than the upper extremity.
The most common sites are the metaphysis and diaphysis of the femur, followed by the tibia and
humerus.
It is very aggressive and metastasizes early to the lungs and lymph nodes.
Patients are typically white males in their first or second decade of life.
Presents with pain and swelling for weeks or months, Erythema and warmth of the local area are
sometimes seen.
Patients may initially be diagnosed with osteomyelitis, due to the misleading presentation of intermittent
fevers, leukocytosis, anemia, and increased ESR.
42
(7)RHEUMATOLOGY
But, Ewing's sarcoma is characterized radiologically with its lamellated appearance or "onion skin"
periosteal reaction.
This lesion is usually lytic, central, and accompanied by endosteal scalloping
The "onion-skin" appearance is often followed with a "moth-eaten" or mottled appearance and
extension into soft tissue.
The treatment includes surgery, radiation, and multi-drug chemotherapy.
OSTEOGENESIS IMPERFECTA: Extremely high yield question for USMLE step-1 and step-2.
An autosomal dominant disease characterized by mutations in the gene coding for type I collagen.
There are four types of (OI). All types can have joint hyperlaxity, hypotonia, wormian bones, and early
hearing loss.
Type II is the most severe form and is usually lethal within the first year of life.
Types I, III , and IV can all be further categorized by the presence or absence of dentinogenesis
imperfecta.
Dentinogenesis imperfecta is a disorder of tooth development The teeth are usually discolored (bluish
gray or yellow-brown), translucent, and weak.
Both primary and permanent teeth are affected.
Patients with the severe type II form of this disease typically expire in utero due to multiple intrauterine
and/or perinatal fractures.
Since type I collagen is an important structural protein that is present in the skin, sclera, bone, tendon
and ligament, patients with this disorder present with multiple recurrent fractures, blue sclera, hearing
loss, joint laxity, short stature, and scoliosis.
Clinical findings include limb deformities, growth retardation, multiple fractures and blue sclerae.
Cardiac anomalies such as aortic root dilatation are not usually seen in (OI). Aortic root dilatation can
be seen in disorders such as Marfan syndrome.
Horseshoe kidneys are not a feature of (OI). Patients with Turner syndrome can have horseshoe
kidneys
Patients with (OI) have normal intelligence not mental retardation.
Ash leaf macules are hypopigmented macules seen in patients with tuberous sclerosis.
(7)RHEUMATOLOGY
43
PEDIATRICS
ASEPTIC NECROSIS OF THE FEMORAL HEAD: Extremely high yield question for USMLE!!!
Clavicular fracture presents with irregularity, crepitus, and fullness over the fracture site, and decreased
movements of the arm.
Predisposing factors are shoulder dystocia, traumatic delivery, and large size of the infant.
Generally, no specific treatment is required (reassurance).
44
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A congenital foot deformity, which is most frequent in first-born infants & is attributed to the molding
effect of the primigravid uterus.
Approximately 10% of patients also have an associated acetabular dysplasia; hence, careful hip
examination is required.
Metatarsus adductus is subdivided into three types:
Type I metatarsus adductus:
characterized by deformity of the feet, specifically adduction of the anterior aspect of the foot with a
convex lateral border and concave medial border.
The ankle movements are normal, and passive and active movement of the foot overcorrects the
deformity into abduction.
AP radiographs reveal mild adduction of the metatarsals at the tarsometatarsal articulation, and an
increased angle between the 1st and 2nd metatarsals.
These cases tend to spontaneously correct by themselves; therefore, treatment is not necessary
(reassurance).
Type II metatarsus adductus:
characterized by feet that correct to the neutral position with passive and active movements; this is
managed with orthosis or corrective shoes, and sometimes plaster casts if initial treatment gives no
results.
Type III metatarsus adductus: is characterized by rigid feet and do not correct; these are managed with
serial casts.
Surgical treatment may be required if there is significant residual metatarsus adductus in children 4
years of age.
OSTEOMYELITIS:
Staphylococcus aureus is the most common cause of osteomyelitis in both infants and children.
2nd common: Group B streptococcus and Escherichia coli in infants, and Streptococcus pyogenes in
children.
Salmonella and Staph aureus are the most common cause of osteomyelitis in patients with sickle cell
disease.
Staphylococcus epidermidis is a frequent cause of osteomyelitis associated with prosthetic devices.
SEPTIC ARTHRITIS:
Like osteomyelitis in a child, a septic joint is usually hematogenous in origin and frequently follows an
upper respiratory tract infection.
The most common organisms causing septic arthritis in children are Staphylococcus aureus and
streptococcus.
It is characterized by severe pain, leukocytosis and an elevated ESR.
The typical patient generally refuses to walk, and keeps the affected limb externally rotated (the
external rotation position allows for maximal pus accumulation).
Ultrasound guided aspiration of the synovial fluid should be performed immediately.
The synovial fluid white cell count of more than 100,000/ul indicates a definite septic arthritis.
An x-ray is often normal.
Empiric antibiotics: in infants a combination of antistaphylococcal agent (nafcillin or vancomycin) and
third generation cephalosporin and for children over age 5 years antistaphylococcal agent (nafcillin or
vancomycin) alone is a reasonable empiric antibiotic therapy.
(7)RHEUMATOLOGY
45
PEDIATRICS
Septic joint in a child is a true surgical emergency and needs immediate drainage.
A delay of even 4-6 hours can lead to avascular necrosis of the femoral head.
LYME ARTHRITIS:
occurs as a result of untreated Lyme infection. It is caused by Borrelia burgdorferi, a spirochete carried
by the deer ticks.
It spreads via blood to various areas of the body, including the joints.
The early symptoms are mild and are easily overlooked, arthritis is the hallmark of late disease.
It tends to involve the knee in most patients Warmth, swelling from effusion and restriction of
movements differentiate it from arthralgias, which occur early in the disease.
The first episode occurs within six months of having erythema migrans, the episodes gradually
disappear in about ten years, if left untreated.
In United States, Lyme disease is prevalent in: Connecticut, Rhode Island, New York, Pennsylvania,
New Jersey, Delaware, Maryland and Wisconsin.
SUPRACONDYLAR FRACTURES:
46
(7)RHEUMATOLOGY
should be suspected in a child under the age of 5 who presents with proximal muscle weakness
(difficulty climbing stairs),
Gower's sign (use his hands to "walk up" his legs in order to assume an upright position), diminished
reflexes, and pseudohypertrophy of the calf muscles.
Mental retardation.
Cardiomyopathy.
Serum CK (and aldolase) levels are used for screening the muscular dystrophies.
Muscle biopsy can confirm the diagnosis in most cases.
The gold standard is genetic studies, which is required in atypical cases.
Prognosis: most of patients are wheelchair bound by age of 12 years.
NURSEMAID'S ELBOW:
occurs when infants or children are lifted or pulled by the hand or arm.
This pulling injury occurs when the radial head slips through parts of the annular ligament.
The child typically keeps the hand in a pronated position, and refuses (cries out in pain) attempted
forearm supination
Rotating the hand and forearm to a supinated position with pressure over the radial head usually
reduces the annular ligament with an audible click, and restores full, normal use of the extremity.
The response to reduction of the displaced ligament is diagnostic.
(7)RHEUMATOLOGY
47
PEDIATRICS
GASTROENTROLOGY
(8)GASTROENTROLOGY
Diagnosis Congenital abdominal wall defects can be made antenatally with maternal serum-AFP
measurements and ultrasonography.
The two types of abdominal wall defects are described in the table below:
Gastroschisis:
Bowel protrudes through a defect on the right side of the umbilical cord.
Bowel is not covered by a protective membrane.
Bowel looks "angry" and matted.
Not typically associated with any other abnormalities outside the GI tract.
If the diagnosis was made antenatally, management would begin with planned spontaneous vaginal
delivery at a tertiary neonatal surgical unit,
Caesarean section is performed only in the presence of the usual obstetric indications.
After delivery, the next step in management is to immediately wrap the exposed bowel with sterile
saline dressings and
Cover with plastic wrap in order to prevent insensible heat and large fluid losses.
The neonate is then maintained in a thermo-neutral environment.
Further steps in the management of gastroschisis include inserting an orogastric tube (to decompress
the stomach), establishing peripheral intravenous access (to provide fluids), and administration of
broad-spectrum antibiotics.
Definitive treatment is surgical; however, surgery should be preceded by the above measures.
Primary closure is successful in 70% of neonates, but if it is unsuccessful, staged closure with Silastic
silo can be used.
Omphalocele:
Intra-abdominal contents protrude through the umbilical ring The abdominal herniation usually includes
small bowel and may include large bowel and liver.
Bowel is covered by an amnioperitoneal membrane.
Can be associated with other congenital abnormalities ( e g , heart, kidney).
The management of omphalocele is similar to that of gastroschisis:
Delivery is also planned at a tertiary care center, and caesarean section is reserved for the usual
obstetric indications, (This is based on the lack of evidence that caesarean delivery improves the
outcome of patients with uncomplicated omphalocele ).
Immediate care of the newborn with omphalocele also involves, sterile wrapping of the bowel, insertion
of an orogastric tube, stabilizing the airway (to ensure adequate ventilation), and establishing
peripheral intravenous access.
Small defects (< 2 cm) can be repaired with primary closure, but most defects will require a staged
procedure with Silastic silo.
48
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Is due to an imperfect closure or weakness of the umbilical ring, it is especially common in low birth
weight, female, and black infants.
It appears as a soft swelling covered by skin that protrudes during crying, coughing or straining, and
can be reduced easily through the fibrous ring at the umbilicus.
The hernia consists of omentum or portions of the small intestine.
Most umbilical hernias disappear spontaneously by 1 year of age.
Surgery is advised if the hernia persists to the age of 3-4 years, exceeds 2 cm in diameter, causes
symptoms, becomes strangulated, or progressively enlarges after the age of 1 to 2 years.
NECROTIZING ENTEROCOLITIS:
Suspect necrotizing enterocolitis in any preterm or low birth weight infant with fever, vomiting,
abdominal distension, bloody diarrhea, leukocytosis & pneumatosis intestinalis.
These symptoms usually occur after the introduction of formula feeding.
The radiologic finding of pneumatosis intestinalis (i.e. intramural air) in infants is diagnostic.
Increased gastric residual volume in a preterm neonate is highly suspicious for necrotizing enterocolitis.
The condition usually presents at 3-10 days after birth, and is due to bowel wall injury resulting from
perinatal asphyxia.
Preterm neonates are at high risk for other complications such as hyaline membrane disease, which
presents with respiratory distress and ground glass appearance of the lungs on chest x- rays.
N.B:
Trachea-esophageal fistula: presents with excessive drooling, choking, coughing, and cyanosis due to
aspiration of feedings.
Duodenal atresia: Bilious vomiting without abdominal distention is the cardinal sign for duodenal
atresia.
Meconium aspiration syndrome: Clinical features of meconium aspiration syndrome include thick,
greenish amniotic fluid in the mouth and throat at birth, accompanied by cyanosis and respiratory
distress.
Pyloric stenosis is characterized by projectile nonbilious vomiting and palpation of an olive-shaped
mass immediately after the infant has vomited.
CELIAC DISEASE:
The presence of dermatitis herpetiformis and chronic non-bloody diarrhea in a child of 12-15 months
is suggestive of celiac disease.
The child has Kwashiorkor-like clinical features associated with dermatitis herpetiformis (erythematous
vesicles symmetrically distributed over the extensor surfaces of elbows and knees).
This scenario is suggestive of celiac disease, which is also called gluten-sensitive enteropathy.
It is due to abnormal hypersensitivity to gluten and presents characteristically at 12-15 months of age,
when gluten-containing foods such as wheat, rye or barley are introduced into the child's diet.
(8)GASTROENTROLOGY
49
PEDIATRICS
PYLORIC STENOSIS:
Is a common congenital anomaly typical presentation is non-bilious projectile vomiting which gradually
becomes more frequent and forceful in a 4-8 week old infant Pertinent physical findings are a palpable
abdominal mass and visible peristaltic waves in the upper abdomen.
Hypokalemic alkalosis occurs due to the frequent episodes of vomiting.
Abdominal ultrasound is the most useful tool for confirming the diagnosis, and shows a thick
hypoechogenic ring in the pyloric region.
Additionally, the loss of large volumes of fluid via vomiting leads to contraction alkalosis due to the
action of aldosterone.
In order to compensate for the metabolic alkalosis, the respiratory system responds via hypoventilation
to create a secondary respiratory acidosis. i.e. have a primary metabolic alkalosis with respiratory
compensation.
This is characterized by a plasma pH greater than 7.45, PaC02 greater than 40 mm Hg and HC03greater than 24 mEq/L.
Pyloric stenosis is treated surgically; however, surgery should be undertaken only after the infant's
hydration status and electrolyte levels have been stabilized.
MECKEL'S DIVERTICULUM:
N.B.:
Peptic ulcer disease may present in preschool children with epigastric pain related to meals with a
positive family history.
Anal fissure is a painful condition associated with constipation it may occur in infancy, and presents
with blood streaked stools.
Intussusception may present in an infant or a young child with irritability, vomiting, red currant-jelly
stools and abdominal mass.
Inflammatory bowel disease may present in preschool children with diarrhea, abdominal pain, lowgrade fever and malaise.
CHOLEDOCHAL CYST:
a congenital abnormality of the biliary ducts characterized by the dilatation of intra or extra-hepatic
biliary ducts or both.
It has a multifactorial origin, but most of the cases are related to an anomalous pancreaticobiliary
junction, which leads to weakness and dilatation of the biliary wall due to the reflux of alkaline
pancreatic secretions into the biliary tree.
Various types of choledochal cysts are:
Type 1: Most common type, features the dilatation of the entire common hepatic and common
bile ducts or segments of each.
Type 2: Relative isolated protrusions or diverticulae from the common bile duct wall.
50
(8)GASTROENTROLOGY
PSEUDO-PANCREATIC CYST:
INTESTINAL ATRESIA:
MIDGUT VOLVULUS:
usually presents in a child less than one month old with bilious vomiting, abdominal distension and
passage of bloodstained stools.
Volvulus is associated with malrotation of the gut, and can be complicated by perforation and
peritonitis.
INTUSSUSCEPTION:
is the most common cause of intestinal obstruction in the first two years of life.
The classic presentation: is an infant with colicky abdominal pain, vomiting, and red currant jelly stools.
The currant jelly stools are caused by bowel ischemia, which leads to bleeding and mucus production.
Between episodes of abdominal pain, the child appears well.
(8)GASTROENTROLOGY
51
PEDIATRICS
On physical examination, the abdomen is usually soft, nontender, and nondistended, A sausageshaped mass is often palpated.
An abdominal radiograph may show air fluid levels due to obstruction.
An ultrasound commonly shows a targetoid lesion in which one bowel segment is nested inside another
bowel segment.
An air contrast enema is both diagnostic and therapeutic in the majority of cases.
Emergent laparotomy is indicated for the treatment of intussusception when air contrast enema is
unsuccessful.
However, laparotomy becomes the treatment of choice if the child presents with peritoneal signs.
The most common type of intussusception is ileocolic.
HIRSCHSPRUNG'S DISEASE:
usually presents with failure to pass meconium within the first 24 hours.
In this disease, an aganglionic segment of the colon causes the intestinal obstruction.
CYCLICAL VOMITING:
Recurrent self-limiting episodes of vomiting and nausea in children in the absence of any apparent
cause.
The etiology of this condition is unclear; however, its incidence is high in children whose parents have
a history of migraine headaches.
Complications that may arise from the condition are anemia and dehydration.
Treatment consists of anti-emetics and reassurance of the parents.
The child typically has frequent vomiting, but maintains adequate weight gain.
Symptoms usually begin in the first few months of life, peak around 4 months, and generally resolve
by 1 year
Conservative therapy is first-line management in these infants, and make sure that the feedings are
the appropriate volume and are given at regular intervals.
Breast milk or formula can be thickened with rice cereal, which can decrease the frequency of vomiting.
The infant should also be positioned to help decrease intraabdominal pressure by keeping the child's
head elevated or laying the child prone when awake.
Parents should be reassured that most children will have resolution of their symptoms by 12 months.
If infants develop respiratory symptoms due to GERO such as stridor, apnea, or failure to thrive, a
more detailed evaluation is necessary including an upper gastrointestinal series, pH probe monitoring,
or endoscopy.
These children may require pharmacotherapy if conservative treatments do not improve their
symptoms.
52
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Batteries lodged in the esophagus on x-ray should be removed immediately under endoscopic
guidance to prevent mucosal damage and esophageal ulceration.
Batteries located distal to the esophagus pass uneventfully in most cases and need only to
be observed with stool examination and/or follow up x-rays to confirm excretion.
TURCOT'S SYNDROME:
GARDNER'S SYNDROME:
is autosomal dominant where colonic polyps are seen with prominent extraintestinal lesions.
Includes: desmoid tumors, sebaceous or epidermoid cysts, lipomas, osteomas (especially mandible),
supernumerary teeth & juvenile nasopharyngeal angiofibroma.
PEUTZ-JEGHERS SYNDROME:
ESOPHAGEAL ATRESIA:
The most common esophageal anomaly is esophageal atresia with a tracheoesophageal fistula.
This is characterized by an atretic esophageal pouch that communicates distally with the trachea just
above the carina.
This anatomic arrangement allows air to enter the stomach, and this leads to gastric distention.
The discontinuous esophagus prevents the infant from completely swallowing, thereby resulting in
drooling or regurgitation during feeding.
In addition, gastric fluid ascends into the distal esophagus through the fistula, into the trachea and
lungs, thereby producing aspiration pneumonia Pneumonitis and atelectasis occur frequently, and
rattles are heard during breathing.
The inability to pass a feeding tube into the stomach is suggestive of esophageal atresia with or without
tracheoesophageal fistula.
(8)GASTROENTROLOGY
53
PEDIATRICS
INFANTILE COLIC:
is defined as excessive crying in an otherwise healthy infant for more than 3 hours a day, more than 3
days a week, and more than 3 weeks in a month.
Colic usually presents within the 1st 3 weeks of life and resolves spontaneously by four months of age.
Most parents report that the crying occurs at the same time of day, typically in the early evening.
The crying begins suddenly, and the child generally cannot be comforted.
The cause of colic is not known, it maybe due to a digestive tract origin (eg, trapped gas) leading to
abdominal pain.
Because the etiology is not known, there is no accepted treatment (resolves spontaneously by four
months of age).
Proposed treatments: soothing measures, simethicone (which decreases the surface tension of gas
bubbles), or probiotics.
The first step is immediate placement of an orogastric tube & connecting it to a continuous suction, to
prevent bowel distension and further lung compression.
Endotracheal intubation and mechanical ventilation are also priorities for all infants with severe CDH
who present in the first hours of life.
54
(8)GASTROENTROLOGY
NEONATOLOGY
(9)NEONATOLOGY
The Apgar score is measured in newborns at 0 and 5 minutes of life. It has five components, and is
scored as follows:
Heart rate
0 heart shows no activity
1 HR <100 beats/min
2 HR > 100 beats/min
Tone/ Activity:
limp
1 some flexion of extremities
2 active flexion of extremities.
Respirations:
0 completely absent
1 slow and irregular
2 good respiratory effort.
BREAST MILK:
The protein in human milk is 70% whey and 30% casein, and the protein content is highest at birth and
decreases over the first month of life.
Whey is more easily digested than casein and helps to (improve gastric emptying).
Human milk also contains lactoferrin, lysozyme, and secretory immunoglobulin A proteins that confer
improved immunity to the infant.
The main carbohydrate in both human milk and standard infant formulas is lactose.
(9)NEONATOLOGY
55
PEDIATRICS
Although calcium and phosphorus content is significantly lower in human milk when compared to
formula, these minerals are better absorbed from human milk.
Breast milk has an inadequate supply of vitamin D and exclusively breast-fed infants must receive
supplemented vitamin D.
BREAST FEEDING:
contraindications to breastfeeding:
Infants with certain inborn errors of metabolism such as galactosemia, phenylketonuria, and urea cycle
defects.
Other contraindications: maternal use of certain medications such as radioactive isotopes,
antimetabolites, and chemotherapeutic agents.
Mothers using drugs of abuse should be counseled to quit using these drugs and should not breastfeed
if they are continuing to abuse drugs.
Breastfeeding is also contraindicated with some maternal infections including herpes simplex (if there
are lesions on the breast), active tuberculosis, and HIV.
Tobacco smoking, hepatitis C infection, mastitis nor maternal alcohol use.
NEONATAL SEPSIS:
Group B streptococcus is the most common cause of neonatal sepsis, it is part of the normal vaginal
flora of women and is transmitted to infants during passage th rough the birth canal.
In neonates, infection presents either as early-onset sepsis or late-onset sepsis.
Early sepsis presents within the first week of life and usually involves several organs Symptoms
include:
Respiratory failure, meningitis, DIC, acute tubular necrosis and peripheral gangrene.
It is usually refractory to therapy and quickly evolves to shock, coma and death.
Late-onset GBS infection is usually a focal infection, most frequently meningitis (75%), although
arthritis, osteomyelitis, cellulitis and urinary tract infection may also occur.
Meningitis presents with fever, lethargy, poor feeding, hypotonia, seizures and a bulging fontanel.
The diagnosis is confirmed by positive blood or cerebrospinal fluid cultures, although all newborns with
fever and sepsis should have full workup for sepsis, including urine cultures.
If the mother has already received antibiotics, culture results may be negative and a latex agglutination
test may be more helpful.
N.B.
Escherichia coli meningitis is less frequent than GBS meningitis It is the second most common cause
of neonatal meningitis.
Listeria is the third most common cause of neonatal meningitis It also tends to result in multiple
abscesses and pneumonia, in addition to meningitis.
56
(9)NEONATOLOGY
Congenital
toxoplasmosis
classically
presents
with
microcephaly,
microphthalmia,
hepatosplenomegaly and chorioretinitis.
Herpes simplex virus encephalitis usually presents with focal neurologic signs. Herpes involves
temporal lobes, so patients may present with seizures.
NEONATAL SEPSIS:
Sepsis in the neonate often presents with fever or hypothermia, jaundice, lethargy, and poor feeding.
Infants with a serious bacterial infection such as meningitis rarely present with classic findings such as
neck stiffness or Kernig's or Brudzinski's signs.
As a result, all infants with a suspected diagnosis of sepsis should be evaluated with blood cultures
and a lumbar puncture
Jaundice in the newborn has a broad list of differential diagnoses including sepsis, breast milk jaundice,
and breastfeeding jaundice.
Temporary cessation of breastfeeding is the most appropriate next step in an infant with suspected
breast milk jaundice.
Breast milk jaundice, which is a caused by a factor in human breast milk, results from increased
intestinal absorption of bilirubin and generally peaks around two weeks of age.
Liver function tests (Albumin, AST, ALT, alklaline phopshatase) should be obtained in infants with a
direct or conjugated hyperbilirubinemia, to assess for conditions such as biliary atresia.
Suspected in infant with vomiting, bloody diarrhea, RBCs and eosinophils in stool, and there may be a
family history of an atopic disorder.
It is a hypersensitivity reaction to cow's milk proteins, and therefore usually occurs in babies who are
fed with cow's milk; however, it may occur in breastfed babies, as the mother's milk may contain
proteins from ingested cow's milk.
.N.B.:
-
It should be done to all pregnant women in the first trimester to prevent possible transmission
to the infant.
Persistent oral thrush, lymphadenopathy and hepatosplenomegaly & maybe intractable
diarrhea, refractory infections and failure to thrive the presenting symptoms.
In neonates, serologic testing is not useful for HIV diagnosis because of the passage of
maternal antibodies to the fetal circulation through the placenta.
PCR, viral culture and p24 antigen testing are more appropriate; HIV infection is confirmed if
at least two of these tests are positive.
(9)NEONATOLOGY
57
PEDIATRICS
CONTRAINDICATIONS TO BREASTFEEDING:
-
Certain active maternal infections (HIV, tuberculosis, herpes simplex lesions on or near the
nipple, malaria, sepsis, typhoid fever),
Eclampsia, nephritis, substance abuse, and breast cancer.
Transmission of HIV by breastfeeding is well documented; therefore, the presence of maternal
HIV infection is a contraindication to breastfeeding.
Mastitis --> we encourage breastfeeding since this prevents engorgement, along with other subsequent
symptoms such as fissures and cracking of the nipple.
Maternal rubella infection --> there is currently insufficient evidence regarding the associated risks.
Breast milk jaundice --> Mothers of such infants are instructed to switch their babies to formula-feeding
for 1-2 days.
This allows the increased indirect bilirubin levels of the infant to return to normal values. After 2 days,
the mother resumes breastfeeding, and further hyperbilirubinemia usually does not recur.
Hemolytic disease of the newborn (erythroblastosis fetalis) --> breastfeeding because antibodies in the
mother's milk are inactivated in the intestinal tract and do not contribute to further hemolysis of the
infant's RBCs.
PHYSIOLOGICAL JAUNDICE:
BREASTFEEDING JAUNDICE:
58
(9)NEONATOLOGY
NEONATAL TETANUS:
Is generally seen in infants born to unimmunized mothers, frequently following umbilical stump
infection.
Affected infants initially present in the first two weeks of life with poor suckling and fatigue, followed by
rigidity, spasms and opisthotonus.
MONGOLIAN SPOT:
The classic description (ie , well-demarcated, flat blue/gray lesion on the sacral or presacral area) of a
Mongolian spot.
This lesion is seen more commonly in dark skinned children.
It is caused by entrapment of melanin-containing melanocytes during their migration from the neural
crest into the epidermis in fetal development. and usually disappears in the first few years of life, most
cases do not require any treatment.
Mostly located at the base of the spine, lower back, and buttocks, it can also be seen in other parts
such as the shoulders, arms, wrists, legs, ankles or abdomen.
The face, palms and soles are usually spared
N.B.:
Cafe-au-lait spots are well-demarcated, tan or light brown flat lesions that can vary in number, size, and
distribution.
This finding may indicate neurofibromatosis if the diameter is larger than 0.5 mm and if more than 5
lesions are found.
(9)NEONATOLOGY
59
PEDIATRICS
Cutis Marmorata appears as a lace-like pattern on the skin in response to cold or stress.
It can persist in some diseases such as Down's syndrome and trisomy 18.
Salmon Patch is a flat salmon-colored lesion commonly seen over the glabella, eyelids, and neck.
BREAST-MILK JAUNDICE:
Appears in the second week of life; however, the hyperbilirubinemia is indirect (unconjugated).
The unconjugated bilirubin levels may rise as high as 10-30 mg/dl Levels drop rapidly as soon as
breastfeeding is stopped, and the infant is given milk formula for 1-2 days.
After 2-3 days the breastfeeding can be resumed, and usually there is no return of the
hyperbilirubinemia.
Although it is a benign condition in some instances, phototherapy may be indicated.
PHYSIOLOGIC JAUNDICE:
Consists of moderate unconjugated hyperbilirubinemia (< 12-14 mg/dL) appears after the first 24 hours
of life and resolves before the end of the first week.
It is a benign condition and is more common in preterm infants, infants of diabetic mothers, and infants
of Asian or Native American descent.
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(9)NEONATOLOGY
ERYTHROBLASTOSIS FETALIS:
CARE OF A NEONATE:
Early neonatal care in an uncomplicated pregnancy include: initial physical assessment, removal of
airway secretions, drying the infant & keeping him warm, & early preventive measures (gonococcal
ophthalmia prevention, vitamin K supplementation).
(9)NEONATOLOGY
61
PEDIATRICS
10
It is very important to differentiate between precocious puberty that is caused by premature activation
of the hypothalamus-pituitary-gonad (HPG) axis, and precocious pseudo-puberty that is caused by a
gonadotropin-independent process, typically an excess of sex steroids.
In case of precocious pseudo-puberty: there is signs of severe androgen excess (i. e. severe cystic
acne, significant growth acceleration) which suggests precocious pseudo-puberty It can be caused by
late-onset congenital adrenal hyperplasia (21-hydroxylase deficiency).
Hypothalamic dysfunction leading to precocious puberty is usually less dramatic in presentation
Sequential development of testicular enlargement, penis enlargement, pubic hair growth, and then
growth spurt is typically present.
Severe hypothyroidism is a rare cause of precocious puberty, and is characterized by slowing (not
acceleration) of growth.
Klinefelter's syndrome may present with a height that is higher than normal, but signs of androgen
excess are not typical.
47, XYY karyotype may manifest as severe acne, but precocious puberty is not characteristic.
characterized by the isolated appearance of axillary hair before the age of six years.
This change results from premature androgen secretion of the adrenal glands.
The condition is generally benign and has no clinical significance.
On the other hand, premature pubarche (pubic hair growth before the age of 8 years) is more alarming,
as it is associated in 50% of cases with a CNS disorder.
62
Thyroid dysgenesis is the most common cause of congenital hypothyroidism in United States.
Iodine deficiency or endemic goiter is the most common cause of congenital hypothyroidism worldwide,
but is essentially not seen in the United States.
.N.B:
Mammary gland enlargement and non-purulent vaginal discharge are common findings in newborn
infants.
These are transitory physiologic events due to infant's physiologic target-organ response to
transplacentally acquired maternal hormones (estrogens).
Therefore, such infants only require observation and routine care.
PRIMARY AMENORRHEA:
The most common cause of primary amenorrhea is Turner syndrome, a 45,XO karyotype chromosomal
disorder.
It may also result from anatomic abnormalities (imperforate hymen, absent uterus),
hypothalamic/pituitary dysfunction, hyperandrogenism or pseudohermaphroditism.
Turner syndrome is characterized by short stature, webbed neck, hypogonadism (streak ovaries),
lymphedema, high-arched palate, congenitally bicuspid aortic valves, and coarctation of the aorta.
Most commonly, ovarian failure in Turner syndrome manifests with delayed puberty, though at least
1/5 of patients will have a normal puberty followed by early menopause.
The decreased femoral pulses may indicate aortic coarctation, which occurs in approximately 10% of
patients with Turner syndrome.
This clinical diagnosis should be confirmed by karyotype analysis.
.N.B.:
-
MCCUNE-ALBRIGHT SYNDROME:
is a rare condition characterized by precocious puberty, cafe au lait spots and multiple bone defects
(polyostotic fibrous dysplasia).
It is responsible for 5% of the cases of female precocious puberty, and may be associated with other
endocrine disorders, such as hyperthyroidism, prolactin- or GH-secreting pituitary adenomas, and
adrenal hypercortisolism.
McCune-Albright syndrome is sporadic and has been recently attributed to a defect in the G-protein
cAMP-kinase function in the affected tissue, thereby resulting in autonomous activity of that tissue.
Remember the 3 P's of McCune-Albright syndrome precocious puberty, pigmentation (cafe au lait
spots) and polyostotic fibrous dysplasia.
63
PEDIATRICS
.N.B.: Adrenal tumors result most commonly in heterosexual precocious puberty (i.e , premature development
of male secondary sexual characteristics in a female).
CRI-DU-CHAT SYNDROME:
GAUCHER'S DISEASE:
NIEMANN-PICK DISEASE:
Types A and B result from the deficient activity of sphingomyelinase. this is a fatal disorder of infancy
Clinical manifestations include failure to thrive, hepatosplenomegaly, and a rapidly progressive
neurodegenerative course that eventually leads to death by age 2-3 years.
BECKWITH-WIEDEMANN SYNDROME:
The exact cause is unknown, but sometimes associated with duplication of chromosome 11 p.
This region contains the gene encoding for IGF-2, which may explain the macrosomia.
Present with macrosomia, macroglossia, visceromegaly, omphalocele, hypoglycemia,
hyperinsulinemia, prominent eyes, prominent occiput, ear creases & pancreatic hyperplasia.
It is usually sporadic, but occasional cases have familial inheritance.
Sometimes, the hypoglycemia may be severe and intractable, and subtotal pancreatectomy may be
needed.
Patients have an increased risk of neoplasms such as Wilms' tumor, hepatoblastoma, and
gonadoblastoma
LESCH-NYHAN SYNDROME:
64
Is a sporadic disorder, in 50-70 % of cases, there is a deletion in the long arm of chromosome 15.
PWS displays a particular type of genetic transmission called genomic imprinting, wherein the
phenotype expression depends on whether the genetic defect is inherited from the mother or the father
presentation: severe hypotonia at birth or infancy, hyperphagia, obesity, short stature and mental
retardation.
The typical craniofacial features are narrow bifrontal diameter, diamond-shaped eyes and a small,
down-turned mouth.
Hypothalamic dysfunctions (GH deficiency and hypogonadotropic hypogonadism) may be present.
The associated short stature, obesity and hypotonia usually respond to GH administration; however,
the implication of GH deficiency accompanying PWS in the development of these features is still
controversial.
N.B:
-
If you see a vignette with a child that is >2-years-old, it is unlikely that the diagnosis is Patau's
or Edward's syndrome.
Important differ. diag. of Beckwith-Wiedemann syndrome; but here infants do not present with
dysmorphic features as omphalocele, prominent occiput & macroglossia.
Moreover, the prenatal and birth histories of the patient maternal D.M.
The common congenital problems that in an 'infant of diabetic mother:
Caudal regression syndrome
Transposition of great vessels.
Duodenal atresia and small left colon.
Anencephaly and neural tube defects.
WAGR SYNDROME:
GALACTOSEMIA:
65
PEDIATRICS
Other common manifestations: aminoaciduria, hepatic cirrhosis, hypoglycemia, and mental retardation.
Such patients are at increased risk for E.coli neonatal sepsis.
Galactosemia is NOT a self-limiting condition.
Early diagnosis and treatment by elimination of galactose from the diet are mandatory.
PHENYLKETONURIA:
CONGENITAL HYPOTHYROIDISM:
The most common cause is thyroid dysgenesis (ie , aplasia, hypoplasia, or ectopic gland).
Other causes: inborn errors of thyroxin synthesis, and transplacental maternal thyrotropin- receptor
blocking antibodies.
Infants initially appear normal at birth, but gradually develop apathy, weakness, hypotonia, large
tongue, sluggish movement, abdominal bloating & umbilical hernia.
Other signs: pathologic jaundice, difficult breathing, noisy respiration, hypothermia, and refractory
macrocytic anemia.
Infants initially appear normal due to the presence of moderate amounts of maternal hormones in the
infant's circulation.
So, screening is mandated in all states at birth to allow for the early detection, treatment, and
consequent improvement of the prognosis.
Screening is done by measuring serum T4 and TSH levels.
The treatment is levothyroxine.
FRAGILE X SYNDROME:
Male infant with a characteristic appearance (la rge head, long face, prominent forehead and chin,
protruding ears), joint laxity, and large testes.
Behavioral abnormalities: hyperactivity, short attention span, and autism, are common.
It results from a full mutation in the FMR1 gene caused by an increased number of CGG trinucleotide
repeats accompanied by aberrant methylation of the FMR1 gene.
66
TURNER SYNDROME:
Presentation: short stature, short webbed neck and broad chest with widely spaced nipples and 45 XO
karyotype.
Other features: a low posterior hairline, gonadal dysgenesis, infertility and primary amenorrhea, which
may occur as the child grows.
A higher incidence of cardiac anomaly: coarctation of the aorta (COA) and a bicuspid aortic valve.
Physical findings of COA include upper extremity hypertension and a delay in the radial-femoral pulse.
Congestive heart failure may be seen in infants with severe cases.
The diagnosis is made with an echocardiogram and the treatment is surgery.
There is higher risk of renal abnormalities, particularly horseshoe kidney, and should have a screening
ultrasound after the diagnosis is made.
Swollen hands and feet due to congenital lymphedema, which is common in patients with Turner
syndrome due to abnormal development of the lymphatic network.
Edema that is due to lymphedema is generally nonpitting on physical exam.
Patients are prone to osteoporosis which increases the risk of bone fracture, this is due to low estrogen
levels from gonadal dysgenesis.
EDWARD'S SYNDROME:
trisomy 18.
The features: micrognathia, microcephaly, rocker bottom feet, overlapping fingers and absent palmar
creases.
Closed fists with index finger overlapping the 3rd digit and the 5th digit overlapping the 4th, and rocker
bottom feet.
Congenital heart disease occurs in greater than 50 % of affected patients; ventricular septal defect is
the most common one.
80% of affected children die in the first month, 90% die by 1 year and remaining 5-10% are mentally
retarded.
67
PEDIATRICS
They have normal serum calcium, normal serum PTH and normal serum alkaline phosphatase and
normal levels of serum 25-hydroxy vitamin D.
There may also be a functional defect in the activity of 1-hydroxylase and as a result serum levels of
calcitriol may below.
In type II vitamin D dependent rickets, there is mutation of vitamin D receptor.
Therefore, these patients have normal serum levels of calcitriol but it is ineffective and as a result
osteomalacia occurs.
DOWN SYNDROME:
Patients with Down syndrome may have a wide range in severity of manifestations.
The most common congenital heart defect is an endocardial cushion defect of the atrioventricular (AV)
canal.
Other congenital heart defects: PDA & ventricular septal defect (congenital heart defects are the most
common cause of death in childhood).
AV canal defects are diagnosed with echo.
The left to right shunt due to the (AV) defect can develop into pulmonary hypertension, that manifested
as a loud P2 on auscultation & has a high morbidity.
AV canal defects may be partial or complete, but both need early surgical correction to prevent
pulmonary hypertension.
Patients with Down's syndrome have a high predilection for duodenal atresia.
Other GIT anomalies: Hirschsprung's disease, Esophageal atresia, Pyloric stenosis, Malrotation of the
bowel.
Atlantoaxial instability is commonly seen in Down syndrome, and most commonly occurs due to
excessive laxity in the posterior transverse ligament, which causes increased mobility between the
atlas (C1) and the axis (C2).
Symptoms of atlantoaxial instability: behavioral changes, torticollis, urinary incontinence, and
vertebrobasilar symptoms as dizziness, vertigo, and diplopia.
Also upper motor neuron symptoms such as leg spasticity, hyperreflexia, a positive Babinski sign, and
clonus.
Patients with Down syndrome are normally hypotonic, and they may remain hypotonic or have
increased tone due to atlantoaxial instability.
It is diagnosed by lateral radiographs of cervical spine in flexion, extension & in a neutral position Open
mouth radiographs can also visualize the odontoid.
Treatment: surgical fusion of the first cervical vertebrae (C1) to the second (C2).
MARFAN SYNDROME:
MSF is an autosomal dominant disorder that results from the mutations of the fibrillin-1 (FBN1) gene.
It presents with tall stature, long and emaciated extremities, arachnodactyly, hypermobility of the joints,
upward lens dislocation, and aortic root dilation.
68
HOMOCYSTINURIA:
GLUCOSE-6-PHOSPHATASE DEFICIENCY:
69
PEDIATRICS
NEPHROLOGY
(11)NEPHROLOGY
11
Positive anti-Smith antibodies and/or anti-double stranded DNA antibodies is specific and confirmatory
for the diagnosis of systemic lupus erythematosus
TRANSIENT PROTEINURIA:
A urine dipstick can be positive in up to 10% of school-aged children. Proteinuria in children can be
transient (intermittent), orthostatic, or persistent.
Transient proteinuria is the most common cause of proteinuria and can be caused by fever, exercise,
seizures, stress, or volume depletion.
Orthostatic proteinuria is very common in adolescent boys and is defined as increased protein when
the patient is in an upright position that returns to normal when the patient is recumbent.
If the urinalysis shows no hematuria and is otherwise normal, the urine dipstick should be repeated on
at least two additional specimens.
If these subsequent tests are negative for protein, the diagnosis is transient proteinuria that are usually
benign conditions that require no further evaluation.
BLADDER EXSTROPHY:
Results from a ventral defect of the urogenital sinus, which causes separation of the pubic rami,
external rotation of the hips, and separation of the rectus abdominis muscles.
The resulting ventral midline defect allows protrusion of a malformed bladder.
This condition is associated with epispadias, recurrent urinary tract infections, and urinary
incontinence.
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(11)NEPHROLOGY
is a congenital abnormality where the penile urethra opens on the ventral surface of the penis rather
than at the tip.
It results from incomplete fusion of the urethral folds on the ventral penis.
URACHUS:
is a tubular extension of the allantois that extends from the bladder to the umbilicus.
It is normally obliterated during fetal development A patent urachus can result in an urachal fistula, cyst
or sinus.
None of these conditions would interfere with urine drainage from the bladder.
CRYPTORCHIDISM:
is a failure of one or both testes to descend from the abdomen through the inguinal canal(s) into the
scrotum.
Wilms tumor is usually diagnosed between the ages of two and five years.
It is the most common primary renal neoplasm of childhood.
80% of the cases have an asymptomatic abdominal mass that is usually detected by the
mother/caretaker while bathing the child.
The mass may be bilateral, symptoms may include hypertension, hematuria, abdominal pain, and
vomiting.
In a few cases, it may present with lung metastases.
It is associated with Beckwith-Wiedemann and Denys-Drash syndromes.
The treatment is nephrectomy.
If treated in the early stage, majority of the patients have a long-term survival.
It arises from metanephros, which is the embryologic precursor of the renal parenchyma.
NEUROBLASTOMA (NBL):
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71
PEDIATRICS
The tumor arises from neural crest cells, which are also the precursor cells of the sympathetic chains
and adrenal medulla.
For this reason, NBL may arise from the adrenal gland or any location along the paravertebral
sympathetic chains.
The most common site involved is the abdomen, either from the adrenals or retroperitoneal ganglia.
The mass is usually firm and nodular in consistency Calcifications and hemorrhages are seen on plain
x- ray and CT scan.
Up to 70 % of patients have metastatic disease at time of presentation, and the most common
metastatic sites are: long bones, skull, bone marrow, liver, L.N. & skin.
The levels of serum and urine catecholamines and their metabolites (i.e. HVA and VMA) are usually
elevated; however, patients do not present with fainting spells, sweating, palpitations and hypertension,
as in pheochromocytoma.
Is a normal anion gap metabolic acidosis caused by a defect in the ability of the renal tubules to
reabsorb bicarbonate or excrete hydrogen.
There are three types of RTA:
These patients are acidotic, hypokalemic, and have an elevated urinary pH In children, type 1 RTA is
often a genetic disorder.
Patients commonly develop nephrolithiasis.
Type 2 RTA is caused by decreased bicarbonate reabsorption in the proximal tubule. Fanconi syndrome is a
common cause in children.
Type 4 RTA is caused by a defect in the sodium/potassium exchange in the distal tubule which results in
hyperkalemic, hyperchloremic acidosis.
In children, obstructive uropathy, renal disease, or multicystic dysplastic kidneys are common causes.
Renal tubular acidosis can present as growth failure and should be considered in the differential
diagnosis for failure to thrive.
Screening labs will show a low bicarbonate level with an increase in chloride, producing a normal anion
gap metabolic acidosis.
The most common cause of nephrotic syndrome in patients younger than 16 years old.
Light microscopy and immunofluorescence does not usually reveal any change in kidney architecture.
Electron microscopy shows diffuse effacement of foot processes of podocytes.
Minimal change disease is a highly steroid-sensitive condition and is the most common cause of
nephrotic syndrome in children.
For these reasons, empiric steroid therapy is indicated in any child with a clinical presentation
suggestive of nephrotic syndrome
Steroids are the treatment of choice.
N.B.:
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(11)NEPHROLOGY
Renal biopsy is commonly used in adult patients presenting with nephrotic syndrome.
Diffuse thickening of glomerular basement membrane & subepithelial spikes are
characteristic for membranous glomerulonephritis, which is the most common cause of
nephrotic syndrome in adults, it is relatively uncommon in younger patients.
Crescent formation is a typical finding for rapidly progressive glomerulonephritis.
Common in pre-pubertal children, Present with acute or chronic vaginal discharge, foul-smelling odor,
and vaginal bleeding.
If the object is large or has sharp edges, the patient may also complain of pain.
The vaginitis produced by a foreign body can cause the child to have urinary complaints as well.
The most common vaginal foreign body found in children is toilet paper.
Other items: toys or coins that the child has placed into her vagina.
Any child with vaginal discharge should have a vaginal examination, which is usually performed by
placing the child in a knee to chest or frog-legged position.
The external genitalia should be examined as well as the vaginal introitus.
Foreign bodies can often be seen if the child is asked to valsalva during the examination.
If needed, a nasal speculum may be used.
If a foreign body is seen, irrigation with warmed fluid should be performed in an attempt to flush out the
foreign body.
If irrigation is unsuccessful, examination and foreign body removal should be done with sedation or
general anesthesia.
NOCTURNAL ENURESIS:
N.B.:
-
Bedwetting is considered normal before the age of 5 years and no investigations or medical
treatment should be prescribed for it.
FIBROMUSCULAR DYSPLASIA:
(11)NEPHROLOGY
73
PEDIATRICS
Is the retrograde flow of urine from the bladder to the ureter and renal pelvis.
Reflux is a risk factor for UTI & Repeated attacks of UTI can lead to progressive renal scarring, which
is the major cause of end stage renal disease and hypertension in children.
It is a risk factor for UTI as it facilitates the transport of bacteria from bladder to the UPPER URINARY
TRACT, this explains why VUR is the most likely cause of acute UTI in children.
VUR is present in 35-40 o/o of children with UTI.
The diagnosis is made with voiding cystourethrogram (VCUG) followed by renal imaging.
Diagnosis of VUR is best made with a voiding cystourethrogram or a radionuclide cystogram (RNC).
Renal ultrasonography is less sensitive in detecting reflux nephropathy.
The American Academy of Pediatrics recommends that all children aged 2 to 24 months with a first
UTI should undergo a VCUG or RNC to detect the presence VUR.
N.B:
-
ACUTE PYELONEPHRITIS:
usually present with fever, chills, nausea, vomiting and flank or suprapubic pain.
Physical examination shows costovertebral angle tenderness.
Urinalysis shows bacteriuria and pyuria.
Therapy should be started with empiric antibiotics after withdrawal blood & urine for culture and
sensitivity and before its results are available.
Severe disease with systemic manifestations warrants intravenous (IV) antibiotics.
If patient is vomiting and hypotensive; therefore, IV antibiotics should be administered.
74
(11)NEPHROLOGY
INFECTIOUS DISEASE
12
(12)INFECTIOUS DISEASE
MEASLES (RUBEOLA):
It is caused by Paramyxovirus.
Characterized by a prodrome of non-productive cough, coryza, non-purulent conjunctivitis, followed by
Koplik's spots (pathognomonic): red spots with bluish specks over the buccal mucosa, opposite the
premolar tooth & sometimes on the inner conjunctivae & vaginal mucosa
And maculopapular rash initially appearing on the face then spread to involve the entire body.
Lab. findings: leukopenia, lymphopenia and Proteinuria may be seen.
Diagnosis is mostly clinical and can be supported by a fourfold rise in hemagglutination inhibition
antibody titer.
Leukopenia (T-cell cytopenia) and thrombocytopenia can be seen with measles infection.
Vitamin A has been shown to reduce the morbidity and mortality rates of patients with measles through
immune enhancement.
It also helps the gastrointestinal and respiratory epithelium to regenerate.
Occurs in persons who have previously received inactivated measles vaccine (was available
in the 1960's), (the measles vaccine available now is a live one).
This form is potentially life-threatening, and characterized by atypical rash (not
maculopapular), the absence of Koplik spots, arthritis, hepatitis and lung involvement Edema
of the hands and feet may occur.
RUBELLA:
characterized by low-grade fever, lymphadenopathy (sub-occipital and posterior auricular) and rash.
The rash is erythematous, maculopapular and classically begins on the face, spreading subsequently
down the body.
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75
PEDIATRICS
Features of CRS: IUGR, deafness, cardiac malformations (e g , patent ductus arteriosus, atrial septal
defects), microphthalmia, cataract, retinopathy, hepatosplenomegaly, thrombocytopenia, and CNS
involvement.
Chronic infection may result in growth retardation, radiolucent bone disease, jaundice,
hepatosplenomegaly, thrombocytopenia and purple skin lesions ("blueberry muffin spots").
Maternal infection is confirmed by the presence of lgM to rubella virus, or a fourfold increase in lgG on
two serum samples obtained two weeks apart.
In the infant, the diagnosis is confirmed by the presence of lgM or persistence of lgG beyond the age
of 6 months.
Prevention of congenital rubella is primarily achieved by administration of rubella vaccine to all females
of childbearing age.
If the immunologic status of a pregnant woman is unknown, rubella titers should be obtained in the first
trimester.
Immunization should not be performed in pregnancy because of a theoretical risk for the fetus, and
such women should be advised to avoid anyone with possible rubella infection.
Classic triad of congenital rubella syndrome (CRS) - sensorineural deafness, cardiac malformations (e
g , PDA and ASD), and cataracts.
ROSEOLA INFANTUM:
The clinical manifestations of chickenpox in healthy children generally develop within fifteen days after
the exposure.
Include a prodrome of fever, malaise, or pharyngitis, followed by the development of a generalized
vesicular rash, usually within 24 hours.
The lesions are commonly pruritic and appear as successive crops of vesicles over a three to four day
period.
Patients typically have lesions in different stages of development on the face, trunk and extremities.
New lesion formation generally stops within four days, and most lesions are fully crusted by the 6th
day in normal hosts.
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(12)INFECTIOUS DISEASE
Is a superficial skin infection with multiple vesiculopustules on the exposed areas of the face and
extremities.
Is a contagious disease caused by Staphylococcus aureus, Streptococcus, or both.
It presents as an erythematous macule, which rapidly evolves into vesicles and pustules.
The pustules later rupture and leave honey-colored, crusted exudates.
There are 2 forms of impetigo vesiculo-pustular and bullous types Bullous type is caused by
Staphylococcus.
These vesiculopustules eventually rupture, and then appear encrusted with a characteristic goldenyellow color.
A history of skin trauma or insect bite is common, and local lymphadenopathy can be present.
Impetigo is most commonly seen in children, and is associated with post-streptococcal
glomerulonephritis.
The etiologic agent is either group-A beta-hemolytic streptococci (GABS) or S. aureus, the clinical
presentations of these organisms are almost similar.
Factors predispose to impetigo are warm and humid climate, poverty, crowding, poor personal hygiene,
and carriage of GABS or S . aureus.
Nasal carriage of Staphylococci can cause recurrent impetigo.
Topical mupirocin is the treatment of choice or oral erythromycin.
Erythema, fissuring, and crusting of the lips, strawberry tongue, and diffuse mucosal
injection of the oropharynx.
Cervical lymphadenopathy.
Kawasaki disease is one of the most common causes of generalized vasculitis in children.
It is usually self-limited, although it can be fatal because of giant aneurysm formation, thrombosis or
rupture of the coronary arteries, leading to MI.
2D echocardiography is used to assess cardiac function and coronary vasculature (Coronary artery
aneurysms are the most serious complication of Kawasaki disease).
A baseline echocardiography is to be performed within 7 days of the disease onset, then repeated 6
to 8 weeks later.
The laboratory of Kawasaki's disease: increased (ESR) and C-reactive protein, leukocytosis,
normochromic-normocytic anemia, and thrombocytopenia.
Thrombocytopenia is a non-specific finding and does not characterize this illness.
Fever in Kawasaki's disease poorly responds to acetaminophen.
All patients with suspected Kawasaki disease should be hospitalized and treated with intravenous
immune globulin ( IVIG) and high-dose aspirin.
The mainstays of systemic treatment of Kawasaki's disease are intravenous immunoglobulin (IVIG)
and aspirin.
Aspirin is useful for fever and arthritis.
This is one of the conditions in which aspirin is recommended in children despite concerns about
Reye's syndrome.
(12)INFECTIOUS DISEASE
77
PEDIATRICS
Influenza vaccination is recommended in patients with Kawasaki's disease taking life long aspirin
therapy.
IVIG has been shown to reduce the incidence of coronary artery aneurysms and other complications.
If untreated, up to 25 % of these patients may develop coronary artery aneurysms.
SCARLET FEVER:
HERPANGINA:
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(12)INFECTIOUS DISEASE
N.B.:
Group A beta-hemolytic streptococci (not Group B) is responsible for 20-25% cases of pharyngitis in
children.
Group B streptococcus infections in the adult are serious. these are common in diabetics and those
with peripheral vascular disease.
Complications include endocarditis, arthritis, pneumonia, empyema and meningitis.
It does not present with a rash after taking amoxicillin.
is another form of cutaneous hypersensitivity that is sometimes considered to be a variant of StevensJohnson syndrome.
Most cases are secondary to medications, such as sulfa drugs, anticonvulsants, and NSAIDs.
Similar to staphylococcal-scalded syndrome, it presents with extensive erythema, tenderness, and
blister formation, followed by denudation of the epidermis.
Mucous membranes are severely affected, and shedding of the nails may occur.
STEVENS-JOHNSON SYNDROME:
RABIES:
Fatal disease that is transmitted to humans by contact with saliva or nervous tissue from an infected
animal through a bite, open wound, or mucus membrane contact.
Bats are a major reservoir for the rabies virus, Bat bites are often so quick and minor that the victim is
not even aware.
For this reason, any direct contact between bats and humans requires post-exposure rabies
prophylaxis.
People bitten by domestic animals suspected of being rabid or not available for observation, or by wild
carnivores like raccoons, skunks, and foxes should also receive prophylaxis.
Rabbits and small rodents like squirrels, chipmunks, and rats are rarely infected with rabies, so Postexposure prophylaxis following bites is not routine.
(12)INFECTIOUS DISEASE
79
PEDIATRICS
VIRAL MENINGITIS:
BACTERIAL MENINGITIS:
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(12)INFECTIOUS DISEASE
N.B.:
-
CSF in tuberculous meningitis also shows a mildly elevated white cell count with lymphocyte
predominance; the protein will be very high and glucose will be low.
However, the presentation tends to be subacute rather than acute.
The long-term neurologic sequelae associated with bacterial meningitis are:
hearing loss
loss of cognitive functions (due to the neuronal loss in the dentate gyrus of the
hippocampus)
seizures
mental retardation
spasticity or paresis
TORCHS INFECTIONS:
Toxoplasmosis, rubella, CMV, HSV and syphilis. cause a syndrome characterized by microcephaly,
hepatosplenomegaly, deafness, chorioretinitis, and thrombocytopenia.
Standard prenatal care for high risk women (immigrants) in USA includes: screening for infection with
syphilis, chlamydia, gonorrhea, and HIV, as well as rubella and hepatitis B immunity.
N.B.:
Folic acid supplementation prior to conception is important to prevent neural tube defects such as spina
bifida and anencephaly.
Zidovudine treatment early in pregnancy in women with confirmed HIV infection can reduce the risk of
vertical transmission of HIV to the fetus.
Also Caesarian delivery also reduces vertical transmission.
Malaria during pregnancy, causes maternal anemia, also increases the risks of fetal loss and IUGR.
Microcephaly is not associated with malaria during pregnancy.
Smoking during pregnancy carries a risk of intrauterine growth retardation, as well as neurologic and
developmental defects. Microcephaly is not a feature.
CONGENITAL SYPHILIS:
Presents early on with hepatosplenomegaly, cutaneous lesions, jaundice, anemia, and rhinorrhea.
Metaphyseal dystrophy and periostitis may be seen on radiography.
Late manifestations: frontal bossing, high arched palate, Hutchinson teeth, interstitial keratitis, saddle
nose, and perioral fissures.
These late manifestations can be prevented with early treatment of the infant
Serologic testing: initial screening (VDRL), rapid plasma reagin (RPR), (EIA); confirmatory testing is
with treponemal tests as the (FTA-ABS) or (TPPA).
Parenteral penicillin G is the treatment of choice for syphilis.
CONGENITAL TOXOPLASMOSIS:
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81
PEDIATRICS
MUMPS:
ECZEMA HERPETICUM:
is a form of primary herpes simplex virus infection that is usually superimposed on healing atopic
dermatitis lesions after exposure to herpes simplex virus.
Numerous umbilicated vesicles over the area of healing atopic dermatitis are typical.
It is frequently accompanied with fever and adenopathy.
In infants, the infection may be life-threatening, and acyclovir treatment should be initiated as soon as
possible.
ERYTHEMA TOXICUM:
The healthy appearance of the neonate, the evanescent nature of the rash, and the distinctive red halo
surrounding the lesions support the diagnosis of erythema toxicum.
The presence of numerous eosinophils in the pustules is diagnostic.
Erythema toxicum is a benign, self-limited condition usually found in newborns after the first 2 days of
birth.
Treatment is not necessary.
N.B.:
-
82
Milia are small pearly white cysts, and are distinct from the rash of erythema toxicum.
Sebaceous hyperplasia presents as little yellowish papules and are commonly found on the
face.
Vaccination against hepatitis B decreases the incidence of hepatocellular carcinoma,
especially, in regions with high levels of hepatitis B infection such as Asia (china) and Africa.
The vaccination schedule for preterm infants should be conformed to the child's chronologic
age, not the gestational age.
Because prematurity does not markedly change the immune response to vaccines, and the
risk of infection and complications in preterm infants is greater than term.
The exception is that children should be 2 kg prior to receiving the first hepatitis B vaccine
(which is at birth).
Small for gestational age infants have a weight under the 10th percentile for gestational age
at birth
May have complications such as hypoxia, polycythemia, hypoglycemia, hypothermia, and
hypocalcemia.
(12)INFECTIOUS DISEASE
LYME DISEASE:
The causative agent is the spirochete Borrelia burgdorferi, which is transmitted to humans by the bite
of an infected tick of the Ixodes species.
The first clinical manifestation is the typical annular rash, named erythema migrans, at the site of the
bite.
The rash may be uniformly erythematous, or it may appear as a target lesion with central clearing.
Erythema migrans may be associated with systemic features: fever, myalgia, headache or malaise.
Individuals who are exposed to tick-infested areas should wear light-colored clothing so that ticks can
be spotted more easily and removed before getting attached.
Wearing long-sleeved shirts and tucking pants into socks or boot tops can help keep ticks from
reaching the skin.
Applying insect repellents containing DEET to clothes and exposed skin, and applying permethrin to
clothes, which kills ticks on contact, also reduce the risk of tick attachment
TINEA CORPORIS:
REYE SYNDROME:
(12)INFECTIOUS DISEASE
83
PEDIATRICS
N.B:
-
Newborns of mothers with active hepatitis B infection should be passively immunized at birth
with hepatitis B immune globulin (HBIG) followed by active immunization with recombinant
HBV vaccine.
INFANT BOTULISM:
C. botulinum is the most common etiologic agent and is usually transmitted through food, especially
honey, which is the carrier in 1/3 of cases.
In infantile form of botulism, the causative organism gains entry through the food and produces toxin
in the GIT, which subsequently triggers the symptoms.
It is a protease that blocks acetylcholine release.
Infants typically present between 2 weeks and 9 months with constipation and poor feeding.
This is followed by progressive hypotonia, weakness, loss of deep tendon reflexes, cranial nerve
abnormalities (impaired gag reflex), and respiratory difficulties.
Signs of autonomic dysfunction such as hypotension and neurogenic bladder can occur early in the
course of the disease.
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13
Healthy, full term, and appropriate for gestational age infants may lose up to 10% of their birth weight
in the first week of life.
The fetus exists in a state of relative total body water and extracellular fluid excess.
After birth, excretion of this excess water accounts for the majority of the weight loss.
The birth weight should be regained by 10 days of life.
Management of dehydration:
The initial step in managing children with dehydration is to determine its severity.
The ideal method of assessing dehydration is to determine the measured change in weight because 1
kg of acute weight loss equals 1 L of fluid loss.
A child's weight, however, changes constantly, making it difficult to obtain an accurate recent "well"
weight.
So, the degree of dehydration often has to be determined by the clinical history and physical
examination & can be divided into:
Mild dehydration: presents with a history of decreased intake or increased fluid loss with
minimal or no clinical symptoms.
Moderate dehydration; decreased skin turgor, dry mucus membranes, tachycardia, irritability,
a delayed capillary refill (2-3 seconds),& decreased urine output.
Severe dehydration: cool, clammy skin, a delayed capillary refill (>3 seconds), cracked lips,
dry mucous membranes, sunken eyes, sunken fontanelle (if still present), tachycardia,
lethargy, and minimal or no urine output, Patients can present with hypotension and signs of
shock when severely dehydrated.
Oral rehydration therapy should be the initial treatment in children with mild to moderate dehydration
Children with moderate to severe dehydration should be immediately resuscitated with intravenous
fluids to restore perfusion and prevent end organ damage.
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Isotonic crystalloid is the only crystalloid solution recommended for intravenous fluid
resuscitation in children.
REHYDRATION:
The signs and symptoms of hypernatremia are mainly neurologic and include lethargy, altered mental
status, irritability, and seizures.
Hypernatremia can also cause muscle cramps, muscle weakness, and decreased deep tendon
reflexes.
Hypovolemic hypernatremia develops secondary to renal losses (eg, diuretic use, glycosuria) or
extrarenal losses (eg, gastrointestinal upset, excessive sweating).
Hypervolemic hypernatremia occurs due to exogenous sodium intake or mineralocorticoid excess (eg,
hyperaldosteronism).
When treating a patient with hypernatremia, the sodium must be slowly returned to normal.
In case of hypernatremia and dehydration, the initial goal is to stabilize the infant with fluid resuscitation
as needed.
When giving intravenous fluid boluses, only isotonic solutions such as normal saline or lactated
Ringer's should be used.
N.B:
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Half normal saline (45%) and 5% dextrose are hypotonic solutions, they should never be used
for initial resuscitation. because they quickly exit the intravascular system and lower the
sodium too rapidly Precipitating drop in sodium levels can cause cerebral edema.
Multiple studies have demonstrated that the expensive colloid solutions are no better than
crystalloids at fluid resuscitation.
DEVELOPMENTAL PROCESSES:
Fine Motor: Tow finger pincer grasp & turns several pages of a book at a time.
Gross Motor: Walks without assistance & - Waves bye & Climbs up on furniture.
Language: Says 2-3 words & - Says "mama" and "dada" games (can identify each parent).
. Social: Imitates actions & - Plays reciprocal games (peek-a-boo) & indicates wants.
Age: 12 months:
Age: 2 year:
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Fine Motor: Copies a circle uses utensils to feed self & stacks 9 blocks.
Gross Motor: climbs stairs with alternating feet, rides a tricycle & kicks a ball.
Language: uses 3 word sentences, stats first name & 3/4 (75%) of speech is intelligible.
Social: wash/dry hands, helps with simple household tasks & group play.
Age: 3 years:
Age: 4 years:
Fine Motor: Copies a cross, draw a person, begins to use scissors & holds a crayon with a tripod grasp.
Gross Motor: Hops on one foot without losing balance & jumps over objects.
Language: Counts to 10, tells stories & use plurals and prepositions.
Social: Cooperative play, has imaginary friends & imitate adults role.
LANGUAGE DEVELOPMENT:
-
SOCIAL DEVELOPMENT:
-
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Developmentally, a 12 month old should be able to walk holding onto a hand or object. He
should have a neat pincer grasp that allows him to grab small objects.
Language development at 1 year old consists of mama and dada said specifically to his
parents, and at least one other word.
Socially, a 12 month old can roll a ball and makes postural adjustments when being dressed
N.B:
TOILET TRAINING:
This condition usually presents after several months of being Vitamin D-deficient.
The typical pathology is - defective mineralization of growing bone or osteoid tissue
The typical patients are low-birth weight infants, unsupplemented dark-skinned infants, infants with
inadequate sun exposure, and solely breastfed infants.
Early manifestations: craniotabes (ping-pong ball sensation over the occiput or posterior parietal
bones), rachitic rosary, and thickening of the wrists and ankles.
Other findings: Harrison groove and a large anterior fontanelle.
Diagnosis is confirmed by obtaining the serum calcifediol level (decreased), alkaline phosphatase level
(increased), and observing the characteristic radiologic changes(cupping and fraying of the distal ends
of long bones, and double contour along the lateral outline of the radius).
Treatment includes oral Vitamin D administration and adequate sunlight (or artificial light) exposure.
The current recommendation to prevent rickets is oral Vitamin D supplementation, beginning during
the first 2 months of life and continuing until early adolescence.
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CONDUCT DISORDER:
Characterized by disruptive behavioral patterns that violate basic social norms for at least one year in
patients less than 18 years old.
It includes aggressive behavior towards others or animals, behaviors resulting in property damage,
theft, or violations of societal rules.
Constitutional growth delay is the most common cause of short stature and pubertal delay in
adolescents.
Affected individuals have a normal birth weight and height, but between 6 months to 3 years of age,
the height growth velocity slows, and they drops percentiles on their growth curve.
Around 3 years of age, the child regains a normal growth velocity and follows the growth curve at the
5th to 10th percentile.
Puberty and the adolescent growth spurt are delayed, but eventually occur.
The child will have a normal growth spurt and reach a normal adult height.
Bone age radiographs show a bone age that is delayed compared to the chronological age.
SEPARATION ANXIETY:
Separation anxiety is a normal behavior in young children between 9-18 months. usually manifests
when parents leave the child or at night when the child is put to bed.
The child responds by crying, clinging to the parent, and becoming upset Separation anxiety usually
lasts 2-4 months and gradually resolves as the child begins to understand that his parents will return.
The initial step in the management of children with speech delay is an audiology evaluation especially
when there is history of recurrent otitis media.
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AUTISM:
patient is usually less than 3 years old and presents with impairment in social interactions and
communication, delayed language development, repetitive activities, and stereotypical behaviors.
Treatment include special education and behavioral modification techniques, No pharmacological
agent has been found to be useful in autism.
ASPERGER SYNDROME:
These kids are usually more socially aware and communicative. Although patients may have features
of stereotypical movements and self-injurious behavior, their language development is normal.
Children with ADHD have poor impulse control, inattentiveness, and motor overactivity.
Multiple factors contribute to development of ADHD: genetics, pregnancy or birth complications,
maternal drug use, abnormal brain development or traumatic brain injury, and psychological stressors.
There are three subtypes of ADHD - predominantly inattentive, predominantly hyperactive-impulsive,
or combined.
To meet the diagnostic criteria for ADHD, a child must exhibit six or more symptoms of inattention or
hyperactivity- impulsivity before age 7 years, with the symptoms present for at least 6 months.
The symptoms must cause significant impairment in functioning and must occur in at least two different
settings
Many children with ADHD have a family history of ADHD. However, this is not necessary for the
diagnosis.
Educational testing may be appropriate, as many children with ADHD have coexistent learning
disabilities however.
Teacher evaluations must be obtained first to establish the diagnosis of ADHD.
Children with ADHD can have sleep problems, which should be addressed if the diagnosis of ADHD is
confirmed by teacher evaluations.
Inattention Symptoms
Hyperactivity-impulsivity Symptoms
No attention to detail; makes careless mistakes.
Interrupts others when they are speaking.
Cannot pay attention while completing tasks or has a problem while waiting their turn. playing
(e.g while playing a board game).
Answers question before the person finishes the entire question.
Does not appear to listen when spoken to.
Talks constantly.
Does not follow instructions with regard to school work chores etc.
Physically active all the time.
Has a hard time organizing tasks ,including fun tasks.
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Attention deficit hyperactivity disorder (ADHD): However, poor language development and social
isolation are not features of ADHD, and a hearing impairment should be ruled out before a diagnosis
of ADHD is considered.
Autism: However,
. Repetitive behaviors, poor eye contact, and impaired social interactions are not seen in hearing
impairment.
. In addition, features of autism generally appear before age three and social isolation is more
prominent than in hearing impairment.
The initial management is directed towards maintaining airway patency, The absence of any added
breath sounds and presence of air movement bilaterally in this patient ensures patent airways.
Next step, upper GI endoscopy is recommended in the first 24hrs to assess the extent of the injury and
to dictate further management.
Barium swallow is not recommended due to the associated difficulty in determining the extent of the
injury with this procedure.
Steroids are not recommended because these may increase the chance of perforation Furthermore,
giving steroids have not been proven to provide ,any benefit in preventing strictures in such cases.
Nasogastric lavage and oral antidotes such as vinegar are not recommended since these may increase
the extent of injury.
Remember attempting to neutralize the alkali with vinegar or lavage is dangerous!
Sun exposure is associated with skin photo-aging and several types of skin cancer, including
melanoma.
Encouraging photo-protection is the leading preventive measure in skin care, and sun avoidance is the
best method of photo-protection.
Patients are instructed to avoid outdoor activities in the middle of the day (between 10 am and 4 pm),
which is the period of peak sun radiation intensity.
Sunscreens are useful adjuncts to photo-protection, but offer insufficient protection from ultraviolet
radiation (UVR) when used alone.
Overdependence on sunscreens may sometimes even increase or encourage outdoor exposure
Sunscreens should be applied 15-60 min prior to sun exposure to allow enough time for protective film
development.
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Currently, the available data shows little to no protection against melanoma with the use of sunscreen
lotions with SPF 15 - 30; however, these can protect from non-melanoma skin cancers such as
squamous cell carcinoma.
Resting under umbrellas decreases UVR exposure by 70%, but does not affect reflected radiation.
Shade from trees provides insufficient UVR protection .
Clothing is considered to be an effective tool for sun protection; however, it does not supersede sun
avoidance.
IRON POISONING:
The patient's symptoms and history of spilled multivitamin pills are highly suggestive of iron poisoning.
This is one of the most common causes of death by poisoning Elemental iron, when ingested in large
amounts, has a corrosive effect on the GI mucosa, leading to abdominal pain, nausea, vomiting,
diarrhea and hematemesis.
Iron also accumulates in the mitochondria and tissue, thus impairing energy production and resulting
in cellular damage and systemic toxicity.
Hypotension then occurs due to the increased capillary permeability and venodilation.
The resulting peripheral hypoperfusion and mitochondrial damage lead to accumulation of lactic and
citric acid, and consequent metabolic acidosis.
Other complications include liver necrosis, coagulopathy, drowsiness, seizures and coma. Long-term
sequelae include gastric scarring and pyloric stenosis.
Since iron is radio-opaque, the tablets can be seen in the stomach on abdominal x-ray.
The diagnosis is confirmed by measuring serum iron levels.
The treatment depends on the severity of the poisoning: Ipecac syrup to induce emesis is used in
asymptomatic patients with an intact gag reflex.
Intravenous deferoxamine, an iron chelator, is used in moderate to severe cases of intoxication.
Activated charcoal is not effective, and gastric lavage is not recommended in young children.
Is usually the result of a suicide attempt especially if symptoms of depression (isolative behavior,
decreased appetite, poor sleep) are present.
Acute iron intoxication has five phases:
1. GIT phase --> 30 minutes to six hours after ingestion & is due to direct mucosal damage,
patient experience nausea, vomiting, hematemesis, melena, and abd. pain.
2. Latent phase --> 6 to 24 hours after ingestion, and is largely asymptomatic.
3. Shock and metabolic --> acidosis may occur 6 to 72 hours following the ingestion.
4. Hepatotoxicity --> 12 to 96 hours.
5. Bowel obstruction --> secondary to mucosal scarring can develop several weeks postingestion
The diagnosis of acute iron intoxication is made by checking the serum iron concentration.
Levels > 350 mcg/dL are consistent with toxicity.
Treatment of iron poisoning involves deferoxamine, which binds ferric iron, allowing urinary excretion.
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LEAD POISONING:
Risk for lead poisoning: live in an old house, especially if there is peeling paint or during renovation
projects.
Other risk factors: lead pipes, living near a battery recycling plant, having a parent who works with
batteries or pottery, or having a playmate or sibling with a history of lead poisoning.
Capillary blood specimens (using a fingerstick) are widely used in childhood screening for lead
poisoning.
Since false positive results are common, the first step after an abnormal fingerstick lead level is to
confirm the diagnosis with a serum (venous blood) lead level.
If the serum lead level is >10 ?g/dL, then intervention is needed.
Removing the child from the house would be the most important step in reducing exposure if the
diagnosis of lead poisoning is confirmed.
Chelation therapy with either dimercaprol or dimercaptosuccinic acid (DMSA, succimer) is typically
used when lead levels are extremely elevated (>45 ?g/dL).
Rechecking the lead level in one month is appropriate for children with mildly elevated lead levels (<20
?g/dL) after a thorough history is taken and counseling is done about removing the child from any
potential lead exposure.
VACCINATION ADVERSE REACTIONS: Extremely high yield question for the USMLE!!!
The adverse reactions to the DTaP vaccine are usually attributed to the pertussis component of the
vaccine.
Mild reactions consist of local redness and swelling, irritability , and fever < 105F (40 6C).
Less common reactions include inconsolable crying for > 3 hours and a temperature of > 105F (40
6C).
An immediate anaphylactic reaction, an encephalopathy, or any CNS complication within 7 days of
administration of the vaccine is a contraindication for further administration of DTaP.
In these instances, DT should be substituted for DTaP since the adverse reactions are usually
attributed to the pertussis component of the vaccine.
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VARICELLA VACCINE:
Guidelines recommend active immunization with varicella vaccine for healthy adults & children
exposed to varicella, ideally within the first 3- 5 days of exposure. because efficacy is decreased
beyond this period.
Because this is a live virus, the vaccine is only recommended for immunocompetent individuals.
Postexposure prophylaxis with varicella-zoster immune globulin product is indicated in susceptible
high-risk persons (immunocompromised patients, pregnant women) exposed to varicella within 96
hours (preferably 72 hours) of exposure, it does not prevent infection but effectively reduces disease
severity. administration of any of these vaccines after these periods it will ineffective.
CAT BITES:
Are of significant concern because these often result in deep puncture wounds, also infection of such
wounds with Pasteurella muftocida tends to develop quickly.
And is associated with considerable pain, erythema, and swelling Localized cellulitis can develop
subacutely.
In some cases, systemic effects (e g , fever and lymphadenopathy) may arise.
Prophylactic treatment is thus recommended for such cases. For treatment of minor cat bite wounds,
amoxicillin/clavulanate for five days is recommended.
ANABOLIC STEROIDS:
INFORMED CONSENT:
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persistence of arterial canal), multiple joint anomalies, prematurity, growth retardation & mental
retardation.
It is sometimes clinically difficult to differentiate it from Down's syndrome (trisomy 21 ) since similarities
include facial dysmorphism and heart malformations; however, the normal karyotype in this case rules
out the latter.
Fetal alcohol syndrome is the most common cause of mental retardation in children.
Withdrawal symptoms are usually not seen unless the mother was drinking just prior to delivery.
The opiates that infants are most commonly exposed to are heroin and methadone.
Methadone is prescribed for pregnant women who are addicted to heroin to prevent uncontrolled
withdrawal in the fetus.
Heroin and methadone are not associated with dysmorphic features or congenital anomalies in the
fetus, but prenatal exposure can lead to increased risk of intrauterine growth retardation,
macrocephaly, sudden infant death syndrome, and neonatal abstinence syndrome (NAS).
NAS: presents in the 1st few days of life & characterized by irritability, a high-pitched cry, poor sleeping,
tremors, seizures, sweating, sneezing, tachypnea, poor feeding, vomiting, and diarrhea.
Withdrawal usually presents within 48 hours after birth for heroin withdrawal and between 48 and 72
hours for methadone.
The treatment for NAS: symptomatic care to calm the infant & help the infant sleep, such as swaddling,
providing small frequent feeds, & keeping the infant in a low stimulation environment.
Pharmacologic treatment used when supportive treatment does not control it; Morphine can be
administered & systematically weaned to help control opiate withdrawal.
N.B.:
-
Prenatal exposure to cocaine can result in jitteriness, excessive sucking, and a hyperactive
Moro reflex.
Withdrawal symptoms are usually not as severe as with opiates; Long-term effects on
behavior, attention level, and intelligence may be seen.
It most commonly occurs in young children following the treatment of viral infections with antibiotic.
May occur 1-2 weeks after administration of certain drugs, such as penicillin, amoxicillin or cefaclor in
the setting of a viral illness.
Prominent symptoms: fever, urticarial rash, polyarthralgia and lymphadenopathy.
It derives its name from its similarities to true serum sickness, an immune-complex mediated
hypersensitivity reaction to non-human proteins.
Serum-sickness like reaction is a clinical diagnosis, and should resolve with withdrawal of the offending
agent; it does not represent a true drug allergy.
leads to pellagra, which is characterized by diarrhea, dermatitis, dementia, and if severe, death.
Pellagra is common in 3rd world countries where the main diet consists of cereal or corn, but can be
seen in people with bowel disease that interferes with vitamin absorption.
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Present with GIT complaints (nausea, abdominal pain, or epigastric discomfort) along with glossitis
and watery diarrhea.
Dermatitis seen in pellagra occurs in sun exposed areas and resembles a sunburn; it is also typically
bilateral and symmetric.
As the rash progresses, the skin becomes hyperpigmented and thickened.
Mental status changes can range from poor concentration to irritability, aggressiveness, and dementia.
Death can occur in severe niacin deficiency if untreated.
Treatment of pellagra is niacin replacement.
N.B:
Deficiency of riboflavin (vitamin B2) can lead to cheilosis, glossitis, seborrheic dermatitis (often
affecting the genital areas), pharyngitis, and edema and/or erythema of the mouth (Pure riboflavin
deficiency is has been documented in regions of the world with severe food shortages).
Pyridoxine (vitamin B6) deficiency causes irritability, depression, dermatitis, and stomatitis.
It can also cause an elevated serum homocysteine concentration, which is a known risk factor
for venous thromboembolic disease and atherosclerosis.
Deficiency of cyanocobalamin (vitamin 812) causes macrocytic anemia and peripheral neuropathy.
VITAMIN A DEFICIENCY:
is the leading cause of mortality in infants aged between 1 month and 1 year in the United States.
It is defined as the sudden, unexpected death of an infant that cannot be explained by the history and
a thorough post-mortem examination.
It is recommended that infants be placed in a supine position while sleeping to reduce the risk of SIDS.
ANAPHYLAXIS:
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RISPERIDONE:
ATOPIC DERMATITIS:
In infancy presents with pruritus and skin lesions typically distributed symmetrically over the face, scalp,
chest and extensor surfaces of the extremities.
The diaper region is typically spared.
usually have a prior dermatological condition such as psoriasis, atopic dermatitis or mycosis fungoides
(Sezary syndrome).
INTRAOSSEOUS ACCESS:
Whenever intravenous access cannot be obtained in emergent pediatric cases, intraosseous access
should be attempted next.
Intraosseous cannulation can be performed quickly in the ED, this technique does not require the
precision required for cannulation of small vessels& provides a cannula large enough to deliver
adequate fluids.
This route can be used in emergencies for 24 to 48 hours, at which point another intravenous route
should have been obtained.
Associated complications such as osteomyelitis are rare.
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