V Flashcards

1
Q

trichophagia

A

pulling hair out and swallowing it

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2
Q

treatment trichotillomania

A

CBT

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3
Q

comorbid conditions with tourettes

A

OCD and ADHD

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4
Q

schistocytes

A

microangiopathic hemolytic anemia

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5
Q

child recovered from diarrheal illness now with anemia, thrombocytopenia and schistocyes

A

HUS from e coli

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6
Q

tetrad HSP

A

palpable purpura, athralgias, abdominal pain and renal disease

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7
Q

renal manifestations of HSP

A

hematuria, non-nephrotic range proteinuria and mildly elevated serum Cr

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8
Q

Treatment of HSP

A

supportive with hydration and NSAIDs

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9
Q

purpura fulminans

A

life threatening condition in bacterial infections- N men, Strep pneumo
black hemorrhagic purpuric lesions, fever, hypotension, DIC

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10
Q

signs of scurvy

A

petechiae, purpura, ecchymoses

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11
Q

cystic hygroma

A

benign lymphangioma in neck

found in turners and trisomy 13,18,21

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12
Q

dactylitis

A

vaso-occlusion in sickle cell anemia

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13
Q

mutation in sickle cell

A

substitution valine for glutamic acid

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14
Q

inheritance hereditary spherocytosis

A

auto dominant

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15
Q

clinical presentation hereditary spherocytosis

A

hemolytic anemia, jaundice, splenomegaly

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16
Q

laboratory findings of hereditary spherocytosis

A

inc MCV, spherocytes in peripheral smear, negative coombs test, increased fragility on acidified glycerol lysis test
abnormal eosin-5 maleimide binding test

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17
Q

Tx for hereditary spherocytosis

A

folic acid supplementation
blood transfusions
splenectomy

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18
Q

gold standard for Dx hereditary spherocytosis

A

eosin-5-maleimide binding test (flow cytometry) with acidified glycerol lysis test

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19
Q

what proteins to check for in paroxysmal nocturnal hemoglobinuria

A

CD55 CD59

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20
Q

exfoliative dermatitis

A

erythroderma

bright red patches coalesce and gradually peel

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21
Q

scabies

A

small pruritic papules in linear arrangement (burrow) in webs spaces, wrists and ankles, genitals, nipples and waistline

22
Q

what is mutated in hereditary spherocytosis

A

ankyrin gene which is in RBC membrane

23
Q

how does hydroxyurea work

A

increased fetal Hb

24
Q

give what to prevent side effects of transfusions in sickle cel patient

A

desfuroxamine

25
Q

nasal obstruction, visible nasal mass and frequent nosebleeds

A

juvenile angiofibroma

seen in young men

26
Q

what syndrome is seen with pineal gland mass

A
parinaud syndrome:
limited upward gaze
ptosis
upper eyelid retraction
pupillary abrnomalities
27
Q

what are signs of obstructive hydrocephalus

A

papilledema, HA, vomiting, ataxia

28
Q

what image to confirm pineal gland mass

A

MRI

29
Q

craniopharyngiomas

A

suprasellar masses that can compress optic chiasm

30
Q

medulloblastomas location

A

cerebellar vermis and cause ataxia and hydrocephalus

31
Q

neuroblastoms

A

sympathetic nervous system

located in adrenal glands

32
Q

what could be reason for PT to be elevated in someone with CF?

A

non absorbable fat soluble vitamins, so vitamin K now is not readily available for platelets

33
Q

cause of hemophiliac arthropathy

A

iron and hemosiderin deposition leading to synovitis and fibrosis

34
Q

most common nutritional deficiency in children

A

iron

35
Q

how to prevent iron deficiency anemia

A

don’t switch to cows milk before 1 year old

36
Q

howell jolly bodies

A

sickle cell

37
Q

pappenheimer bodies

A

sideroblastic anemia

38
Q

iron panel in thalassemia

A

dec MCV, dec TIBC

inc iron, increase ferritin

39
Q

iron panel in anemia of chronic disease

A

decrease in MCV, iron, TIBC and increased in ferritin

40
Q

clinical presentation immune thrombocytopenia

A

antecedent viral infection
asmptomatic petechiae and ecchymosis most common
mucocutanwous bleeding

41
Q

treatment children with immune thrombocytopenia

A

observe skin manifestations

for bleeding give IVIG, or glucocorticoids

42
Q

isolated thrombocytopenia

A

think about immune thrombocytopenia

43
Q

how to prevent vasoocclusive crisis in sickel cell

A

hydroxyurea

44
Q

side effect in hydroxyurea Tx

A

myelosuppresion, (anemia, neutropenia, thrombocytopenia)

45
Q

what is complication in sickle cell TRAIT

A

hematuria

46
Q

which thalassemia has more Hb A2

A

Beta

47
Q

iron therapy in thalassemia can lead to what

A

hemochromatosis

48
Q

treatment aplastic crisis

A

parvovirus B19

49
Q

acute chest syndrome in sickle cell

A

fever, chest pain, infiltrate on CXR

50
Q

sickle cell anemia, severe anemia with low or absent reticulocytes

A

aplastic crisis