Heme

Question 1

Normal coagulation studies but prolonged bleeding after circ

Answer 1

Factor 13 deficiency

Question 2

Low baby plt ct
Normal mom plts

Answer 2

Neonatal alloimmune

Question 3

Decreased maternal and baby plts

Answer 3

Neonatal autoimmune due to maternal ITP
Occurred with moms who have ITP, lupus or other autoimmune conditions

Question 4

Blood cell formation in bone marrow starts at

Answer 4

22 wks

Question 5

Polycytthemia associations

Answer 5

Placental insufficiency
Smoking
HTN
Trisomy’s and beckwith
Maternal diabetes and thyrotoxicosis

Question 6

Decreased NADPH levels

Answer 6

G6PD def

Question 7

Amish hemolytic anemia

Answer 7

Pyruvate kinase def

Question 8

Blood production in bone marrow begins at what age

Answer 8

22 wks

Question 9

Tests if there is baby blood in maternal blood

Answer 9

Kleihauer-betke

Question 10

1% fetal hemoglobin =

Answer 10

50ml of fetal blood

Question 11

Detects maternal blood in bb

Answer 11

Apt test

Used to detect if blood in aspirated or stool is maternal in origin

Question 12

Macrocytic anemia with reticulocytopenia, wbc and plts normal. Abnormal thumbs

Answer 12

Diamond- blackfan

Question 13

Blood group antigens that cause hemolysis

Answer 13

Kell , Duffy (Fya), Kidd, c and e

Question 14

Antigens that don’t cause hemolysis

Answer 14

Lewis, anti I , anti Fyb

Question 15

Most frequently inherited enzyme defect
X linked recessive
may have false neg test results during acute hemolysis /reticulocytosis
Decreased NADPH levels
Hemolysis during times of stress , flava beans and sulfa drugs

Answer 15

G6PD

Question 16

Lack of ATP generation causes chronic anemia and increase 2, 3DPG
European ancestry
Ar

Answer 16

Pyruvate kinase deficiency

Question 17

Allo antibodies from Mom directed towards paternally inherited platelet antigens on baby platelets

Answer 17

Neonatal alloimmune thrombocytopenia

Reoccurrence is 90%
Known mom treated with ivig/steroids

Baby -give plts , matched plts or ivig

Question 18

Mother has normal platelet count baby has low platelets

Answer 18

Neonatal alloimmune thrombocytopenia

Question 19

Coagulation factors that are normal at birth

Answer 19

5,8,13, vwf

Question 20

Reasons for elevated platelet counts

Answer 20

Normal reaction in preterm infants at 4-6 weeks

Iron deficiency
Vit E deficiency
Trisomy 21
Infection

Question 21

Increased risk of leukemia

Answer 21

Fanconi anemia, diamond-blackfan, trisomy 21

Question 22

Most common solid tumor in the neonatal period

Answer 22

Teratoma

Sacrococcygeal

Question 23

Second, most common tumor

Answer 23

Neuroblastoma

Question 24

Components of ffp

Answer 24

All clotting factors
Fibronectin
IgG
Albumin
Plasma proteins

Question 25

Cryo

Answer 25

8, vWf, fibrinogen , 13, fibronectin

Question 26

BilliRubin is converted by

Answer 26

Glucuronosyltransferase

Question 27

Enzyme that allows for bili to be reabsorbed during entrohepatic circulation

Answer 27

Glucurinidase

Question 28

Bili that is excreted by kidneys

Answer 28

Urobilinogen

Question 29

Bili excreted by intestines

Answer 29

Stercobilinogen

Question 30

Increased risk of Wilms tumor

Answer 30

Beckwith-wiedemann Denys-drash syndrome Pearl man syndrome WAGR

Question 31

Polycythemia causes

Answer 31

Low glucose calcium and plts

Question 32

G6pd

Answer 32

Most common inherited defect Heinz bodies Prolonged hyperbili Decreased nadph

Question 33

BM and iron

Answer 33

Breast milk is low in iron, but the iron has high bio availability thanks to lactoferrin

Question 34

Changes to stored red cells

Answer 34

Stack , sticky Increased viscosity Vasoactive NO scavenger Higher affinity for o2 Cytokine release

Question 35

Kleihauer Betke

Answer 35

# fetal cells/ # maternal cells x 100 For every 1% = 50mls of fetal blood loss

Question 36

Pathological jaundice

Answer 36

1st 24 hrs More than 0.5 rise Last more than 8 days in term or 14 in preterm

Question 37

Most common tumor in Neonate Infant Childhood

Answer 37

Teratoma Neuroblastoma Neuroblastoma