Heme

Question 1

The bone marrow becomes the primary site of hematopoiesis

Answer 1

After 22 weeks

Question 2

Infants with factor 13 deficiency with have Normal ____

Answer 2

PT and PTT

Question 3

Blood volume exchange

Answer 3

Patients HCT- desired HCT/
Patients HCT

x infant blood volume

Question 4

The most prevalent hemoglobin abnormality in the world

Answer 4

Hemoglobin E

Question 5

A hemolytic anemia that is both normocytic and normothermic with an appropriate reticulocute response

Answer 5

Pyruvate kinase deficiency (common in Amish population)

Question 6

Most common site for post nec stricture

Answer 6

Left colon

Question 7

Factor 8 and 9 deficiency inheritance pattern

Answer 7

-x linked recessive

Question 8

Factor 11 and 13 deficiency inheritance pattern

Answer 8

Autosomal recessive

Question 9

Von Willebrand disease inheritance pattern

Answer 9

Autosomal dominant or recessive

Question 10

Blooms syndrome, diamond-black fan syndrome, fanconi’s anemia, and trisomy 21 are all associated with

Answer 10

Increased risk of congenital leukemia

Question 11

Characterized by hypopigmented hyperpimented skin lesions, photosensitive malaria rash, mild craniofacial dysmorphisms, and high pitched cry, increases risk of congenital leukemia

Answer 11

Blooms syndrome

Question 12

Characterized by a congenital macrocyclic hypoplastic anemia and increased risk of congenital leukemia

Answer 12

Diamond- Blackfan syndrome

Question 13

Characterized by short stature, bone marrow failure with cytopenias, radial and other anomalies, and increased risk of congenital leukemia

Answer 13

Fanconi’s anemia

Question 14

In the fetus, EPO is produced primarily by the

Answer 14

Liver

Question 15

Breastfeeding jaundice at 2-4 days of life is caused by

Answer 15

Am increased enterohepatic circulation of bilirubin

Question 16

______amount of ligandin contributes to physiologic jaundice

Answer 16

Decreased

Question 17

Disorders associated with Wilms tumor

Answer 17

Beckwith- weiderman, Denys Dash, Pearlman syndrome, WAGR syndrome

Question 18

Syndrome notable for progressive renal disease, male pseudohermaphroditism, and Wilms timor

Answer 18

Denys Dash (Danni’s Dash wants to be a girl and boy)

Question 19

Fetal gigantism, visceromegaly, abnormal facies, bilateral renal hamartomas, and Wilms tumor secondary to a germ line mutation resulting in an AR overgrowth syndrome.

Answer 19

Pearlman syndrome (Pearl the whale)

Question 20

Wilms tumor, aniridia, genitourinary anomalies and mental retardation.

Answer 20

WAGR syndrome.

Question 21

Central hypoventilation, Hirschsprung’s disease, and neurofibromatisis type 1 have all been associated with

Answer 21

Neuroblastoma

Question 22

Itridiated blood helps prevent ______ and leukodepleted helps reduce ______

Answer 22

Graft vs host disease, CMV transmission

Question 23

10-15 cc/kg PRBC can raise the hemoglobin by

Answer 23

2-3

Question 24

Alpha thalassemia:
1gene affected =
2 gene affected =

Answer 24

Carrier- no clinical manestifation

Trait-low mean corpuscular volume, low mean corpuscular Hgb, normal or mild anemia otherwise normal growth and development

Question 25

Aloha thalessemia
3 genes affected-
4 genes affected

Answer 25

Hemoglobin H disease
Hemoglobin Barts, hydros details/ die in utero

Question 26

Albumin binding capacity may be decreased in the presence of

Answer 26

Sepsis, acidosis, hypoxia, free fatty acids and albumin binding drugs