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18 Cards in this Set
- Front
- Back
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6th position has a valine instead of a glutamic acid in this hereditary disease
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Sickle cell anemia, (HbS)
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Percentage of fetal Hg after one year
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10%
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Hemoglobin (Hb) composition in adults:
A1 A2 F |
Hb A1: - > 96%
A2: ~1.5-3% F: < 2% |
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The fetus gets iron stores from maternal supply, how long does this store last after birth?
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4-6 months
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Lower limit of the infant at 2-3 months is 9.4, why does this level not need treatment?
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High Hb at birth, erythropoiesis then stops due to its new oxygen rich environment (physiological anemia)
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Why is it advisable that kids get off the bottle by 12 to 14 months?
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They can fill up on milk and become iron deficient
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These are possible causes of?
Merckel diverticulum Epistaxis Ulcer Inflammatory bowel disease Intestinal polyps |
Iron Deficiency Anemias
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Most frequent sign of iron deficiency anemia
Usually diagnosed in screening |
Palor:
-can be seen in conjunctiva -when finger tips are extended - dark colored creases abscent |
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No specific treatment for this disease
Diagnosis important to avoid iron deficient treatment for a patient w/o iron deficiency |
Thallassemia
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-Hg as low as 3g/dL
-Requires transfusion -Hepatosplenomegaly -maxillary hyperplasia |
Beta thalassemia major
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-Hemoglobin disease almost exclusively in Asians
-Chronic hemolytic anemia -Splenomegaly |
Hemoglobin H disease
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Hb disease - Tetrad of gamma chains in newborns
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Bart's disease - Hydrops fetalis
Catastrophic anemia, death |
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Can be caused by Spectrin + Ankryin deficiency, or only Spectrin deficiency
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Hereditary spherocytosis
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Osmotic fragility test positive
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Hereditary spherocytosis
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Spleen helps protect against infection from what type of organisms?
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Encapsulated
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* Haemophilus influenzae type B (Hib)
* Streptococcus pneumoniae (pneumococcus) * Neisseria meningitides (meningococcus) * Group B streptococcus (GBS) * Salmonella typhi |
Polysaccharide encapsulated bacteria
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Splenectomy in patients older than five years helps prevent RBC destruction in this disease
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Hereditary spheroctosis
Requires prior immunization for encapsulated organisms and peniceillin prophylaxis |
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Consider this treatment option when Hg < 10, Retic < 10%, or h/o aplastic crisis
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Splenectomy
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