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SBL Clin > Pediatric Cases > Flashcards

Pediatric Cases Flashcards

1
Q

How does the concentration of 2,3-DPG change with anemia?

A

It increases within the RBC

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2
Q

The oxygen curve shifts right or left in anemia?

A

To the right - oxygen is easily detached from hemoglobin to deposit into tissue

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3
Q

What is the first test you want to order if you suspect anemia?

A

Hemoglobin and hematocrit

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4
Q

What is the next test after the H/H to order/look at?

A

MCV, MCH, MCHC

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5
Q

After you look at MCV, what do you look at next?

A

Reticulocyte index (<2 is normal)

Less than normal would indicate bone marrow production problem
Greater than normal would indicate increased hemolysis

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6
Q

When is the physiologic nadir of H/H in infants?

A

2 months old

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7
Q

What is an important congenital infection that can cause hemolytic anemia?

A

Parvovirus B19

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8
Q

What is diamond blackfan syndrome?

A

Pure red cell aphasia - macrocytic anemia with low reticulocyte count

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9
Q

What it’s the most common inherited form of aplastic anemia?

A

Fanconi anemia

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10
Q

What can fanconi anemia progress to ?

A

Pancytopenia around age 10

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11
Q

What will the peripheral smear show for iron deficiency anemia?

A

Hypochromic microcytes

Target cells

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12
Q

What is the mentzer index used for?

A

To differentiate iron deficiency anemia from beta thalassemia

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13
Q

What does a mentzer index of >13 indicate?

A

Iron deficiency anemia

<13 - beta thalassemia is more likely

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14
Q

What does basophilic stippling on peripheral smear indicate?

A

Lead poisoning

Microcytic hypochromic

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15
Q 
What is the inheritance of: 
G6PD 
HS 
Thalassemia 
SCD
A

X linked
AD
AD
AR

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16
Q

What does HUS present after?

A

Acute gastroenteritis

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17
Q

What is the classic triad for HUS?

A

MAHA
Thrombocytopenia
Acute renal failure

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18
Q

what is the most common cause of isolated thrombocytopenia in otherwise well children?

A

Acute immune thrombocytopenia (ITP)

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19
Q

What is the platelet count in ITP?

A

<20,000

20
Q

Are the PT/PTT in ITP increased or decreased?

A

Normal !

21
Q

What is the treatment of acute ITP?

A

Prednisone

22
Q

What is kasabach-merritt syndrome?

A

Thrombocytopenia and hypofibrinogenemia secondary to giant hemangioma and associated intravascular coagulation

23
Q

What are B symptoms?

A

Unexplained fever
Weight loss >10%
Drenching night sweats

24
Q

What is the triad for wiscott Aldrich syndrome? (X linked recessive)

A

Recurrent sino-pulmonary and ear infections
Severe atopic dermatitis
Bleeding

25
Q

What trisomy is found in AML and ALL ?

A

Trisomy 21

26
Q

What is the absolute neutrophil count equation (ANC)?

A

((%neutrophils+%bands)x (WBC))/100

27
Q

What happens if the ANC is <500?

A

The risk of severe infections is high

28
Q

What is Kostmann syndrome?

A

Severe congenital neutropenia

29
Q

What are the clinical manifestations of Kostmann syndrome?

A

Life threatening pyogenes infections in infancy

30
Q

What causes Kostmann syndrome?

A

Impaired myeloid differentiation caused by maturational arrest of neutrophil precursors

31
Q

What is the inheritance of Kostmann syndrome?

A

Autosomal recessive

32
Q

What are the clinical manifestations of cyclic neutropenia?

A

Cyclic fever, oral ulcers, gingivitis, periodontal disease, recurrent bacterial infections

33
Q

What disease is described by a stem cell regulatory defect resulting in defective maturation

A

Cyclic neutropenia

34
Q

What is the inheritance pattern of cyclic neutropenia?

A

Sporadic or AD

35
Q

What is the triad in Shwachman diamond syndrome?

A

Neutropenia
Skeletal abnormalities
Exocrine pancreas insufficiency

36
Q

Which disease is caused by a defect in neutrophil mobility, migration and chemotaxis in addition to neutropenia?

A

Shwachman diamond syndrome

37
Q

What is the inheritance pattern shwachman diamond syndrome?

A

AR

38
Q

Scwachman diamond syndrome increases the risk of which malignancy?

A

Myelodysplastic syndrome or leukemia

39
Q

What is fanconi anemia?

A

Autosomal recessive disorder in which there is a bone marrow failure that results in pancytopenia

40
Q

What is leukocyte adhesion deficiency?

A

Delayed separation of umbilical cord >3 weeks, recurrent and severe bacterial and fungal injections without pus accumulation

41
Q

What’s the triad of hyper IgE syndrome?

A

Eczema
Recurrent bacterial infections of the skin
Recurrent pulmonary infections

42
Q

What is the inheritance of hyper IgE syndrome?

A

Autosomal dominant

43
Q

Which disease results from defects in granule morphogenesis, chemotaxis and degranulation?

A

Chediak higashi syndrome

44
Q

A defect in oxidative metabolism, absent generation of superoxide describes what disorder?

A

Chronic granulomatous disease

45
Q

What is a virus that commonly causes neutropenia in chillens?

A

Parvovirus B19

SBL Clin (9 decks)

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