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20 Cards in this Set
- Front
- Back
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Why do diseases of alpha globin manifest themselves earlier than diseases of beta globin?
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Fetal hemoglobin, alpha2gamma2, switches to adult hemoglobin, alpha2beta2.
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Is anemia in infancy pathologic?
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No. Infants naturally experience a dip in hemoglobin in the first few years of life.
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Why are premature babies iron deficient?
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There is a large transfer of iron from mother to child in the 3rd trimester, and premature babies lose some of this trimester.
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When should iron supplementation be given in infants?
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For breast-fed infants, no later than 4 months.
For infants fed with formula, through 12 months. |
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What are the consequences of iron deficiency in children?
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Delays in cognitive and motor development and behavior problems.
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What molecular markers are affected as iron deficiency develops?
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Serum iron falls
TIBC rises Transferrin saturation falls sTfR rises Hemoglobin content of reticulocytes (CHr) falls sTfR-Ferritin index rises |
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What is the treatment for iron deficiency?
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Elemental iron, 3-5 mg/kg/day for 3 months.
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What are Hemoglobin Barts and Hemoglobin H?
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Hemoglobin Barts = gamma4
Hemoglobin H = beta4 |
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What is the difference between B+ thalassemia and B0 thalassemia?
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B+ represents decreased hemoglobin production,
B0 represents absent hemoglobin production. |
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What can mild thalassemia sometimes be confused with?
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Iron deficiency. Both are microcytic anemias.
Thalassemia has normal RDW, iron deficiency has high RDW. |
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How can you differentiate between alpha and beta thalassemia?
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Alpha thalassemia will show Hemoglobin Barts (gamma4) on newborn screen.
Beta thalassemia will show elevated hemoglobin A2 after about 1 year of age. |
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What is anemia of acute infection?
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Mild normocytic anemia can result from infections like URIs and gastroenteritis.
It is self-limited and resolves in 1-2 months. Mediated though hepcidin. |
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What is a neonatal sign of G6PD deficiency?
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G6PD deficiency = significant neonatal jandice.
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How do you diagnose autoimmune hemolytic anemia?
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Dark urine/jaundice,
spherocytes, Direct Coombs positive |
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Why is it easier to make a VIII diagnosis in a newborn than factor IX deficiency?
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All newborns have factor IX levels lower than adult normals.
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What treatment approaches are available to children with hemophilia?
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On-demand (transfusion with cryoprecipitate),
prophylaxis (intravenous recombinant VIII), and ITI (immune tolerance induction) |
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What treatment is given to patients with FVIII inhibitors?
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"Bypass" treatment with activated prothrombin products and
recombinant factor VIIa. |
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What is hemophilic arthropathy?
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Repetitive bleeding into a joint space (i.e. in patients with hemophilia) that causes the synovium to hypertrophy and become prone to more bleeding (a "target joint.")
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How is childhood ITP different from adult ITP?
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As common in females as it is in males,
abrupt onset, not often chronic (80% will resolve in 6 months.) |
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What are the treatment options for childhood ITP?
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No treatment (platelets >10,000),
glucocorticoids, IVIG and anti-D immunoglobulin (WinRho) |
