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ANEMIA
Congenital Diamond Blackfan Most common inherited Infant: pallor –mc CBC PRBC
hypoplasticanemia Syndrome disorder affecting the
RBC 2-6mos: profound anemia HbF Corsticosteroids
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
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
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
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
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
Exertionaldyspnea (+)
OrthopenEa (-)
Tachycardia (+)
S3 gallop (-)
Cental venous pressure greater than 16cm water (-) NOT DONE
MINOR CRITERIA
Tachycardia (+)
Dizziness(+)
Syncope(-)
Signs and symptoms Left Sided Heart Failure Right Sided Heart Failure