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18 Cards in this Set

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6th position has a valine instead of a glutamic acid in this hereditary disease
Sickle cell anemia, (HbS)
Percentage of fetal Hg after one year
10%
Hemoglobin (Hb) composition in adults:
A1
A2
F
Hb A1: - > 96%
A2: ~1.5-3%
F: < 2%
The fetus gets iron stores from maternal supply, how long does this store last after birth?
4-6 months
Lower limit of the infant at 2-3 months is 9.4, why does this level not need treatment?
High Hb at birth, erythropoiesis then stops due to its new oxygen rich environment (physiological anemia)
Why is it advisable that kids get off the bottle by 12 to 14 months?
They can fill up on milk and become iron deficient
These are possible causes of?

Merckel diverticulum
Epistaxis
Ulcer
Inflammatory bowel disease
Intestinal polyps
Iron Deficiency Anemias
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
No specific treatment for this disease
Diagnosis important to avoid iron deficient treatment for a patient w/o iron deficiency
Thallassemia
-Hg as low as 3g/dL
-Requires transfusion
-Hepatosplenomegaly
-maxillary hyperplasia
Beta thalassemia major
-Hemoglobin disease almost exclusively in Asians
-Chronic hemolytic anemia
-Splenomegaly
Hemoglobin H disease
Hb disease - Tetrad of gamma chains in newborns
Bart's disease - Hydrops fetalis

Catastrophic anemia, death
Can be caused by Spectrin + Ankryin deficiency, or only Spectrin deficiency
Hereditary spherocytosis
Osmotic fragility test positive
Hereditary spherocytosis
Spleen helps protect against infection from what type of organisms?
Encapsulated
* Haemophilus influenzae type B (Hib)

* Streptococcus pneumoniae (pneumococcus)

* Neisseria meningitides (meningococcus)

* Group B streptococcus (GBS)

* Salmonella typhi
Polysaccharide encapsulated bacteria
Splenectomy in patients older than five years helps prevent RBC destruction in this disease
Hereditary spheroctosis

Requires prior immunization for encapsulated organisms and peniceillin prophylaxis
Consider this treatment option when Hg < 10, Retic < 10%, or h/o aplastic crisis
Splenectomy