Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Pediatrics > Hematology > Flashcards

Hematology Flashcards

1
Q

What is neutropenia?

A

low absolute number of neutrophils expressed as absolute neutrophil count (ANC)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

what is the main complication of beta-thalassemia major?

A

hemochromatosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q
  • autosomal recessive
  • oculocutaneous albinism
  • large blue-gray granules in the cytoplasm of neutrophils
  • neutropenia
  • blond or brown hair with silver streaks
A

Chediak-Higashi syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is the main management of iron deficiency anemia ?

A

1) iron supplementation (4-6mg/kg/day)

2) dietary counseling

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

what is the treatment for von Willebrand disease?

A

DDAVP - Desmopressin acetate. Induces release of vWf from endothelial cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is the coagulation defect in von willebrand’s disease?

A

defective platelet function because of decrease or defective von Willebrand’s factor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

what is the management for beta-thalassemia major?

A

lifelong transfusions and often splenectomy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What does ANC stand for?

A

absolute neutrophil count - the percentage of WBCs that are neutrophils, bands, and immature myeloid cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What 2 age groups is nutritional iron deficiency most common

A

1) 9 - 24 months - inadequate intake and iron stores

2) adolescent girls - poor diet, rapid growth, loss of iron from menstruation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What are the laboratory findings in hemophilia A

A
  • prolonged aPTT
  • normal PT, bleeding time, platelet count, platelet function assay
  • low factor 8 protein activity
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is the pathophysiology of PK deficiency?

A

decreased production of pyruvate kinase production leading into leading to ATP depletion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What would you expect the reticulocyte count be in a hemolytic anemia?

A

high

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q
  • hemolytic anemia
  • hepatosplenomegaly
  • bone marrow hyperplasia if untreated
  • delayed growth and puberty
  • hypochromia and microcytosis
  • target cells
  • poikilocytes on blood smear
A

beta-thalassemia major

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q
  • Autosomal recessive
  • frequent and life-threatening pyogenic bacterial infections beginning in infancy
  • ANC usually < 300 cells/mm^3
A

severe congenital agranulocytosis (Kostmann syndrome)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

what are the 2 major causes of macrocytic anemia is children?

A

1) folic acid deficiency

2) b12 deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What are the 2 most common microcytic, hypo chromic anemias in childhood?

A

iron deficiency anemia (most common) and beta-thalassemia trait

17
Q

What is the inheritance patter for hemophilia A?

A

x-linked

18
Q

What are the clinical features of Chronic benign neutropenia of childhood (CBD)?

A

Children with increased incidence of mild infections, such as otitis media, sinusitis, pharyngitis, and cellulitis. Children are otherwise healthy, with normal appearance and growth.

19
Q

What is hemochromatosis?

A

iron accumulation within the heart, liver, lungs, pancreas, and skin

20
Q

What are some clinical features of anemia in infants

A

pale, underweight

21
Q

3 year old female with splenomegaly, pallor, weakness. Elevated reticulocyte count, spherocytes on blood smear, abnormal RBC fragility

A

hereditary spherocytosis

22
Q
  • autosomal recessive
  • short stature
  • immunodefficiency
  • fine hair
  • neutropenia
A

cartilage-hair hypoplasia syndrome

23
Q

What is the inheritance pattern of pyruvate kinase (PK) deficiency?

A

autosomal recessive

24
Q

What is the inheritance pattern of von willebrand’s disease?

A

automsomal dominant

25
Q

What are the clinical features of hemophilia A

A
  • hemarthroses

- central nervous system bleeding

26
Q

What is the difference between aplastic crisis and aplastic anemia?

A

aplastic anemia leads to pancytopenia but aplastic crisis does not

27
Q

What is the coagulation defect in Hemophilia A?

A

defect in factor VIII

28
Q
  • exocrine pancreatic insufficiency w/ malabsorption
  • short stature caused by metaphysical chrondrodysplasia
  • neutropenia
  • failure to thrive
  • recurrent infections common
A

Schwachman- Diamond Syndrome

29
Q

What are some clinical features of anemia in older children

A
  • spoon shaped nails

- diminished attention and ability to learn

30
Q

sideroblastic anemia

A

group of anemias characterized by the presence
of ring sideroblasts in the bone marrow. Ring sideroblasts result
from the accumulation of iron in the mitochondria of RBC precursors

31
Q

What is the treatment for hereditary spherocytosis?

A

1) transfusions

2) splenectomy

32
Q

What is the normal % of RBCs that are reticulocytes?

A

1%

33
Q

A 10 year old Italian boy has had chronic anemia since infancy that is characterized by severe hypochromia and microcytosis. Examination reveals a short child, an enlarged liver and spleen, and prominent facial bones, especially the maxilla, forehead, and cheekbones. What is the diagnosis ?

A

beta-Thalassemia

34
Q

What factor is deficient in hemophilia B?

A

Factor IX

35
Q

What does a low reticulocyte count indicate?

A

bone marrow failure or diminished hematopoiesis

Pediatrics (12 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions