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SBL > Pediatric Disorders - cont. > Flashcards

Pediatric Disorders - cont. Flashcards

1
Q

Acute and Chronic ITP - age of onset?

A

Acute - 2-6 years old

Chronic - 20-40 year old women usually

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2
Q

Acute and Chronic ITP - prior infection?

A

Acute - prior viral infection 1-3 weeks prior

Chronic - NONE

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3
Q

Acute and Chronic ITP - onset of bleeding?

A

Acute - rapid

Chronic - insidious

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4
Q

Acute and Chronic ITP - platelet levels?

A

Acute - < 20K

Chronic - 30K-80K

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5
Q

Acute and Chronic ITP - duration?

A

Acute - 2-6 weeks

Chronic - months/years

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6
Q

Acute and Chronic ITP - eosinophilia and lymphocytosis present?

A

Acute - Yes

Chronic - No

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7
Q

Acute and Chronic ITP - remission?

A

Acute - common spontaneous remission

Chronic - rare

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8
Q

How is Wiscott-Aldrich syndrome inherited and what does it predispose to development of?

A

X-linked recessive

- Increased risk for NON-Hodgkin lymphoma

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9
Q

Symptoms of Wiscott-Aldrich syndrome?

A
  • Thrombocytopenia
  • Atopic dermatitis
  • Recurrent sino-pulmonary and ear infections
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10
Q

Thrombotypenia, atopic dermatitis, and recurrent sino-pulmonary and ear infections suggests?

A

Wiscott-Aldrich syndrome

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11
Q

Wiscott-Aldrich syndrome predisposes to development of?

A

NON-Hodgkin Lymphomas

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12
Q

Kostmann Syndrome ANC level?

A

ANC < 200

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13
Q

Kostmann Syndrome has an increased risk for?

A

AML due to maturation of neutrophil precursors

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14
Q

Cyclic Neutropenia ANC level?

A

ANC < 200 for 3-7 days every 15-35 days

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15
Q

ANC < 200 for 3-7 days every 15-35 days suggests?

A

Cyclic Neutropenia

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16
Q

Symptoms of Cyclic Neutropenia?

A

Cyclic fever, recurrent bacterial infections

Oral ulcers and gingivitis

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17
Q

How can Cyclic Neutropenia be inherited?

A

Autosomal Dominant or sporadic

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18
Q

3 symptoms for Schwachman-Diamond Syndrome?

A

Neutropenia
Exocrine pancreas insufficiency
Skeletal abnormalities

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19
Q

Schwachman-Diamond Syndrome has defects in?

A

Neutrophil mobility

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20
Q

How does cyclic neutropenia occur?

A

Stem cell regulatory defect that results in defective maturation

21
Q

How is Leukocyte Adhesion Deficiency inherited?

A

Autosomal recessive

22
Q

3 signs of Leukocyte Adhesion Deficiency?

A

Delayed separation of umbilical cord stump (>3 weeks)
Poor wound healing
Recurrent bacterial/fungal infection with pus

23
Q

Delayed separation of umbilical cord stump, poor wound healing and recurrent bacterial/fungal infections with pus suggest?

A

Leukocyte Adhesion Deficiency

24
Q

How is Hyper-Immunoglobin E syndrome (job syndrome) inherited?

A

Autosomal Dominat or sporadic

25
Q

Symptoms of Hyper-immunoglobin E syndrome (job syndrome)?

A

Severe Eczema

Recurrent skin and pulmonary infections

26
Q

Levels of IgE, eosinophils and neutrophil chemotaxis with Hyper-immunoglobin E syndrome (job syndrome)?

A

IgE = HIGH
Eosinophils = HIGH
Neutrophil Chemotaxis = LOW

27
Q

Those with Hyper-immunoglobin E syndrome (job syndrome) are at an increased risk for?

A

Hodgkin lymphoma

28
Q

How is Chronic Granulomatous Disease inherited?

A

X-linked recessive

29
Q

Chronic Granulomatous Disease has a defect in?

A

Oxidative metabolism (no superoxide formation)

30
Q

When does Chronic Granulomatous Disease start?

31
Q

Symptoms of Chronic Granulomatous Disease?

A

Recurrent purulent infections with fungal/catalase (+) bacteria
- chronic inflammatory granulomas

32
Q

Recurrent purulent infections with fungal/catalase (+) bacteria is seen with?

A

Chronic granulomatous disease

33
Q

What is Fanconi Anemia?

A

Inherited form of Aplastic Anemia

- autosomal recessive

34
Q

What is an autosomal recessively inherited form of Aplastic Anemia?

A

Fanconi Anemia

35
Q

Fanconi Anemia involves ______ in bone marrow

A

Increased apoptosis

36
Q

GU and skeletal anomalies are seen with?

A

Fanconi Anemia

37
Q

Symptoms of Fanconi Anemia?

A

Pancytopenia, Macrocytic anemia
GU and skeletal anomalies
Chromosome fragility

38
Q

Symptoms of Fanconi Anemia?

A

Pancytopenia, Macrocytic Anemia
GU and skeletal anomalies
Chromosome fragility

39
Q

When will Fanconi Anemia present?

A

May be up to 10 YEARS of age before it presents

40
Q

When will Fanconi Anemia present?

A

May be up to 10 YEARS of age before it presents

41
Q

Those with Fanconi Anemia are at an increased risk for?

A

AML, brain and wilms tumors

42
Q

What is Diamond-Blackfan syndrome/anemia?

A

Congenital pure red blood cell aplasia that presents in infancy

43
Q

What is a congenital pure red blood cell aplasia?

A

Diamond - Blackfan Syndrome/Anemia

44
Q

When does Diamond-Blackfan syndrome/anemia present?

45
Q

What occurs with Diamond-Blackfan syndrome/anemia?

A

Increased apoptosis in erythroid precursors

46
Q

What occurs with Diamond-Blackfan syndrome/anemia?

A

Increased apoptosis in erythroid precursors

47
Q

What type of anemia and reticulocyte count are present with Diamond-Blackfan syndrome/anemia?

A

Macrocytic anemia

LOW reticulocytes

48
Q

What type of anemia and reticulocyte count are present with Diamond-Blackfan Syndrome/anemia?

A

Macrocytic anemia

LOW reticulocytes

49
Q

Inherited forms of macrocytic anemia?

A

Diamond-Blackfan syndrome/anemia

Fanconi Anemia

SBL (48 decks)

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