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Georgemar V. Arana Jr.

June 20, 2014


Group III Pedia

ANEMIA

1) Iron Deficiency Anemia


a. iron loss exceeds iron intake for a time long enough to deplete the body’s iron stores, insufficient iron is available for normal Hb production.
b. need for iron is increased (e.g., during rapid growth in infancy and childhood, during pregnancy) or when excessive loss of blood has reduced
the body’s reserves of iron (e.g., following repeated hemorrhages, excessive menstruation, or multiple pregnancies).
c. 6 and 24 months old – are particularly susceptible.
i. After the first 4–6 months of life, the iron stores present from birth have been exhausted, and the infant depends on dietary iron.
d. Prolonged treatment of peptic ulcer and acid reflux by H2 blockers and acid pump blockers may cause defective iron absorption.
e. Hemorrhagic lesions, such as benign and malignant tumors, chronic ingestion of some medications, and helminthic infections are common
causes of iron deficiency in males and postmenopausal females.
f. Stages:
i. iron depletion
1. negative iron balance exists - blood loss exceeds absorption
2. Iron is mobilized from stores, storage iron decreases, plasma ferritin decreases,
3. iron absorption and plasma iron binding capacity (transferrin) increases.
ii. Iron deficient Erythropoiesis
1. result of lack of iron for heme synthesis  red cell protoporphyrin increases.
iii. iron deficiency anemia;
1. detectable
2. first is normochromic and normocytic, gradually becomes microcytic, and finally becomes microcytic and
hypochromic.
g. Clinical Features:
i. Paresthesias
ii. atrophy of epithelium of the tongue with burning or soreness
iii. angular stomatitis)
iv. pica – due to chronic gastritis
v. koilonychias
vi. dysphagia
vii. Plummer-Vinson syndrome - combination of glossitis, sore mouth, dysphagia, and iron deficiency
viii. Splenomegaly
h. Laboratory Features
i. Blood - microcytosis, anisocytosis, poikilocytosis (including elliptical and elongated cells), and varying degrees of hypochromia,
elliptocytes (pencil cells)
1. Anisocytosis - ↑ red cell distribution width (RDW).
2. ↓ Reticulocytes, MCV, Hb, Hct, osmotic fragility
3. Platelets - ↑ whether the lack of iron is due to blood loss or dietary deficiency,
a. ↓ in severe anemia
ii. Marrow - Normoblastic hyperplasia, proportion of normoblasts that are sideroblasts is decreased (20%);
1. percent saturation in children is less than in adults; it reaches the adult value between the ages of 15 and 18 years
iii. Serum Ferritin – In adults, the reference values are 12–300 g/L, with higher values in men
2) Thalassemia
a. intestinal blood loss due to cow’s milk protein intolerance in young children when cow’s milk is introduced too early.

Types Features Description Manifestations Diagnostic Treatment

Congenital Diamond Blackfan Most common inherited Infant: pallor –mc CBC PRBC
hypoplasticanemia Syndrome disorder affecting the
RBC 2-6mos: profound anemia HbF Corsticosteroids

Congenital anomalies: Bone marrow bx Bone marrow


Craniofacial anomalies transplant

Fanconi’s Aplastic Fragile anemia Most common aplastic Hyperpigmentation Bone morrow Bx Androgen + low dose
Anemia anemia prednisone
Café au lait spots
BM transplant
Short stature
HPV vaccine
Microcephaly

Skeletal abnormalities

Thumb and Radial


hypo/aplasia
Georgemar V. Arana Jr. June 20, 2014
Group III Pedia
Acquired aplastic Caused by drugs, Pallor CBC – pancytopenia Control bleeding
anemia organophosphates, and normocytic
parvovirus B19 Bruising anemia Bone marrow
transplant
Petichia Normocytic anemia
Mucosal hemorrhages Bone marrow Bx –
hypercellularity,
No hepatosplenomegaly
decreased RBC,
WBC,
megakaryocytes

Transient DDX of blackfan Most common acquired Moderate to severe CBC PRBC if severe
Erythroblastemia of syndrome red cell aplasio normocytic anemia
Childhood Bone marrow bx
Reduced or absent
reticulocytes

Normal or increased
platelet count

Normal or slightly reduced


neutrophils

Bone marrow erethroid


hypoplasia

Physiologic Anemia of Decreased erethrocyte CBC Iron therapy


infancy production

Decreased EPO production

Shortened RBC life span

Shift of fetal to adult Hgb

Rapid body growth

Iron deficiency Anemia Mc hematologic disease Mild to moderate: 6-10g/dl, Serum ferritin Iron: 3mkd-6mkd
of infancy irritable, pagophagia
Plasma iron Increase diet of heme
MC nutritional anemia Severe: < 5g/dl, irritable, iron from meat and
anorexia, restlessness, RBC iron shell fish and non-
tachycardia, cardiac heme iron foods such
dilatation, systolic murmur, as cereals, pasta,
cardiomegaly, widened legumes and
diploe potatoes.

PRBC

Megaloblastikanemia Due to inadequate Failure to thrive, slow CBC Vit B12: 0.2ug/kg
nutrients, defect in mental development,
absorption, defect in anorexia, weakness, Serum vitamin B12 If with neurologic
transport, defects in glossitis, pallor, sclera manifestations
Schilling’s test
metabolism icterus

Anemia of Decrease production of Self limited CBC


inflammation RBC

Hemolyticanemia More on African Intrinsic cellular defects: Anemiaunresponsisve to CBC


americans and G6PD Membrane defects – hematinics
Asians. spherocytosis and
elliptocytosis Intermittent bouts of
hyperbilirubinemia
Enzyme defects – G6PD
Slpenomegally
Hemoglobin disorder
Chronic leg ulcers
Extrinsic defects:
Georgemar V. Arana Jr. June 20, 2014
Group III Pedia
acquires, immunologic, Multiple gall stones
microangiopathic and
toxic

Hereditary Autosomal dominant Most common familial Neonate: anemia, Osmotic fragility test Splenectormy
spherocytosis and congenital hyperbilirubinemia,
abnormality of the cell jaundice PBS Folic acid
membrane
Children: anemia, jaundice, Vaccination: PCV,
splenomegaly (hallmark) meningococcal, Hib

Paroxysmal nocturnal Acquire disorder, Intravascular haemorrhage Acid serum sucralose Prednisone
hemoglobinuria mutation I PIG-A – dark urine lysis test
Anticoagulation
Infections Flow cytometry
Iron
Venous thrombosis
DOC: Eculizumab
Defective haematopoiesis

Malignancy transition

G6PD deficiency X-link recessive Diminished reductive Acute haemolytic anemia CBC Prevention
ability of RBC =
hemolysis Chronic non spherocytic NBS BT
haemolytic anemia
Treat complications
Neonatal jaundice

Favism

Sickle cell anemia Substitution of valine Mc cause of avascular Vasso-occlusive sickle cell PBS Immunization
instead of glutamic acid necrosis crises: hallmark
Electrophoresis Painful crisis
Hand and foot syndrome management
Reticulocyte count
Acute chest syndrome: 2nd Transfusion
mc cause of death Bone marrow Bx
Replacement of
Skeletal Xray defective gene

Beta thalassemia Cooley’s thalassemia Imbalance of globin Infants: pallor, poor growth PBS Transfusion
major synthesis abdominal enlargement,
hepatosplenomegally Bilirubin Chelation

Children: HbF(20-100), CBC Allogenic bone


HbA2(2-7%), HbA(0-80%), marrow transplant
Very high HbF in RBC
splenomegaly, low
Vitamin
reticulocyte count, severe Urinalysis supplementation
microcytic RBC.
Gene therapy
Marked erythroid
hyperplasia

Hemophilia A: 80-85% - classic MC severe inherited Hemarthrosis: hallmark PTT Mild: desmopresin
haemophilia: factor VIII congenital bleeding
deficiency disorder Musculoskeletal bleeding Specific factor assay Life threatening:
factor VIII and IX
B: 10-15% - Christmas hemorrhage Genetic testing replacement
factor deficiency
Test for inhibitory FFP
C: factor IX deficiency antibodies to factor
VIII and IX Antifibrinolytic
therapy

Von willebrand’s Type I: autosomal Disorder of protein vWF Epistaxis Immunoassay of Desmopressin
disease dominant –mc, responsible for platelet vWF
decreased vWF adhesion. Ecchymosis Factor 8 for acute
Ristocetin co- factor bleeding
Georgemar V. Arana Jr. June 20, 2014
Group III Pedia
decreased Menorrhagia Platelet function Plasma derived vWF
analyser for type III
Type II: autosomal Post operative bleed
recessive, abnormal vWF Multimer analysis
quality Rare: hemarthrosis
Bleeding time:
Type III: absent vWF Prolonged

Immune Antibody mediated Mc cause of Sudden bruising and CBCP: low platelet Prevent CNS bleed
thrombocytopenic destruction: Thrombocytopenia Petechiae count
purpurea Platelet <10k: IV Ig
Acute: occurs in 90%, DDX: dengue fever Bleeding manifestations in Bone marrow and steroids,
spontaneous within a child who is otherwise in aspiration
6mos, lasts for 1- excellent health 10-20k
2weeks, mortality rare, hospitalization, 20-50:
good prognosis Offen with viral illness Oral steroids/IV
history or immunization
Chronic: persist >6mos,
occurs in 10%, smaller
probability of
spontaneous resolution

CONGESTIVE HEART FAILURE

Signs and symptoms Patient’s symptoms

Exertionaldyspnea (+)

OrthopenEa (-)

Actue pulmonary edema (-)

Chest pain/pressure and palpatations (-)

Tachycardia (+)

Fatigue and weakness (+)


HEART FAILURE CRITERIA
Nocturia and oliguria (-)
Table 1 Framingham criteria for diagnosis for the presence of either 2 major
Anorexia, weight loss, nausea (+) criteria or 1 major criteriaand 2 minor criteria

Exophthalmos and or visible eye pulsation (-) CRITERIA PATIENT’S


SYMPTOMS
Distended neck veins (-)
MAJOR
Weak, rapid and thready pulse (-)
Paroxysmal nocturnal dyspnea (-)
Rales, wheezing (-)
Weight loss of 4.5kg in5 days in response to (-)
S3 gallop and pulsusalterans (-) treatment
Increased intensity of P2 heart sound (-) Neck vein distension (-)
Hepatojugular reflux (-) Rales (-)
Ascitesm hepatomegaly and anasarca (-) Acute pulmonary edema (-)

Central or peripheral cyanosis (-) Hepatojugular reflux (-)

S3 gallop (-)

Cental venous pressure greater than 16cm water (-) NOT DONE

Circulation time of 25 secs


Georgemar V. Arana Jr. June 20, 2014
Group III Pedia
Radiographic cardiomegaly (+) Bilateral ankle edema (-)

Pulmonary edema, visceral congestion, (+)


cardiomegaly

MINOR CRITERIA

Nocturnal cough (-)

Dyspnea on ordinary exertion (+)

Decrease in vital capacity by one third the maximal NOT DONE


value recorded

Pleural effusion (-)

Tachycardia (+)

Right Sided Heart Failures Left Sided Heart Failure

Edema(-) Tachypnea (+)

Ascites(-) Respiratory Distress, and wheezing


(cardiac asthma)

Jugular Vein distention (NMT 8- Easy fatigue(+)


10cm)(-)

Signs of renal and Hepatic Failure(-) Exercise intolerance(+)

Dizziness(+)

Syncope(-)

Signs and symptoms Left Sided Heart Failure Right Sided Heart Failure

Pitting Edema Mild to moderate(-) Moderate to severe(-)

Fluid Retention Pulmonary edema and Abdomen ascites (-)


pleural effusion (-)

Organ Enlargement Heart(-) Liver. Severe Jugular


Venous Pressure (JVP),
Neck veins visibly
distended(-)

Shortness of breath Prominent dsypnea(+), Dyspnea present but not as


Paroxysmal nocturnal prominent
dyspnea

Gastrointestinal Present but not as Loss of appetite(+),bloating,


prominent constipation,
Georgemar V. Arana Jr. June 20, 2014
Group III Pedia

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