Pediatric Cases Flashcards

1
Q

when does the physiologic nadir for hemoglobin in infants occur?

A

at approximately 2 months of age

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2
Q

how does anemia effect the homoglobin-oxygen association curve?

A

the oxygen dissociation curve shifts to the right

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3
Q

how does the rate of onset of anemia affect a patient?

A

the slower the anemia develops the better the body can compensate. Rapidly developing anemia usually results in more dramatic symptoms

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4
Q

how could you tell if the bone marrow was inadequately responding to a severe anemia?

A

a low or low-normal reticulocyte count is an inadequate bone marrow response in the face of a significant anemia

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5
Q

what is diamond-blackfan anemia?

A

congenital pure red blood cell aplasia that almost always presents in infancy

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6
Q

what is occurring in diamond-blackfan anemia?

A

there is increased apoptosis in erythroid precursors

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7
Q

how does diamond-blackfan anemia look on the peripheral blood smear?

A

macrocytic with low/inadequate reticulocyte count

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8
Q

what is the most common inherited form of aplastic anemia?

A

fanconi anemia

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9
Q

when do you get diagnosed with fanconi anemia?

A

may be up to 10 years old before it presents

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10
Q

how does iron deficiency anemia look on the peripheral smear of a child?

A

microcytic, hypochromic with increased RDW; target cells on peripheral smears

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11
Q

if a question stems has a pale child and he like to drink a lot of cow’s milk, what should you think?

A

IDA

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12
Q

how does cyclic neutropenia present?

A

cyclic fever, oral ulcers, gingivitis, periodontal disease, and recurrent bacterial infections

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13
Q

what is the inheritance pattern for cyclic neutropenia?

A

autosomal dominant

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14
Q

what are the symptoms associated with Schwachman-Diamond syndrome?

A

Triad of: neutropenia, exocrine pancreas insufficiency, and skeletal abnormalities

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15
Q

what is the inheritance pattern for Schwachman-Diamond syndrome?

A

autosomal recessive

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16
Q

what is the inheritance pattern of fanconi anemia?

A

autosomal recessive

17
Q

what is a classic example of congenital pancytopenia/ bone marrow failure/ aplastic anemia?

A

fanconi anemia

18
Q

what are patients with fanconi anemia at an increased risk for?

A

AML, brain tumors, and Wilms tumor

19
Q

how does leukocyte adhesion deficiency present?

A
  1. delayed separation of umbilical cord stump, 2. recurrent and severe bacterial and fungal infection without pus, and 3. poor wound healing
20
Q

what is the inheritance pattern of leukocyte adhesion deficiency?

A

autosomal recessive

21
Q

what are the symptoms associated with hyper-immunoglobulin E syndrome (Job syndrome)?

A

Triad of symptoms: severe eczema, recurrent bacterial infections (usually staph) of skin, recurrent pulmonary infections (bacterial or fungal)

22
Q

the number 1 cause of death due to illness in the US is what?

A

brain cancer followed by leukemias

23
Q

the overall number one cause on death in children is what?

A

unintentional injuries (accidents)

24
Q

what is the most common malignancy between ages 15-19?

A

hodgkin lymphoma (HL)

25
Q

what is the pathognomonic feature of HL?

A

reed-sternberg cells

26
Q

what is the inheritance pattern of wiscott-aldrich syndrome?

A

x-linked recessive

27
Q

what are the symptoms of wiscott-aldrich syndrome?

A

classic triad of: recurrent sino-pulmonary and ear infections, severe atopic dermatitis, and bleeding secondary to significant thrombocytopenia

28
Q

name the condition responsible for the greatest percentage of childhood malignancies?

A

leukemias (#1 cause of death is brain malignancies, but #1 cause of malignancies is leukemias)

29
Q

when does ITP usually present in kids?

A

50% follows a viral infection by 1-3 weeks

30
Q

what do the labs show in a kid with ITP?

A

platelets will be less than 20k with all other cell lines being normal; PT/PTT is normal

31
Q

how do you treat ITP in kids?

A

it will usually self resolve with supportive care, but prednisone is used when severe

32
Q

how does kasabach-merritt phenomenon present?

A

thrombocytopenia and hypo-fibrinogenemia associated with giant hemangioma