V

Question 1

trichophagia

Answer 1

pulling hair out and swallowing it

Question 2

treatment trichotillomania

Answer 2

CBT

Question 3

comorbid conditions with tourettes

Answer 3

OCD and ADHD

Question 4

schistocytes

Answer 4

microangiopathic hemolytic anemia

Question 5

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

Answer 5

HUS from e coli

Question 6

tetrad HSP

Answer 6

palpable purpura, athralgias, abdominal pain and renal disease

Question 7

renal manifestations of HSP

Answer 7

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

Question 8

Treatment of HSP

Answer 8

supportive with hydration and NSAIDs

Question 9

purpura fulminans

Answer 9

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

Question 10

signs of scurvy

Answer 10

petechiae, purpura, ecchymoses

Question 11

cystic hygroma

Answer 11

benign lymphangioma in neck

found in turners and trisomy 13,18,21

Question 12

dactylitis

Answer 12

vaso-occlusion in sickle cell anemia

Question 13

mutation in sickle cell

Answer 13

substitution valine for glutamic acid

Question 14

inheritance hereditary spherocytosis

Answer 14

auto dominant

Question 15

clinical presentation hereditary spherocytosis

Answer 15

hemolytic anemia, jaundice, splenomegaly

Question 16

laboratory findings of hereditary spherocytosis

Answer 16

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

Question 17

Tx for hereditary spherocytosis

Answer 17

folic acid supplementation
blood transfusions
splenectomy

Question 18

gold standard for Dx hereditary spherocytosis

Answer 18

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

Question 19

what proteins to check for in paroxysmal nocturnal hemoglobinuria

Answer 19

CD55 CD59

Question 20

exfoliative dermatitis

Answer 20

erythroderma

bright red patches coalesce and gradually peel

Question 21

scabies

Answer 21

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

Question 22

what is mutated in hereditary spherocytosis

Answer 22

ankyrin gene which is in RBC membrane

Question 23

how does hydroxyurea work

Answer 23

increased fetal Hb

Question 24

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

Answer 24

desfuroxamine

Question 25

nasal obstruction, visible nasal mass and frequent nosebleeds

Answer 25

juvenile angiofibroma | seen in young men

Question 26

what syndrome is seen with pineal gland mass

Answer 26

``` parinaud syndrome: limited upward gaze ptosis upper eyelid retraction pupillary abrnomalities ```

Question 27

what are signs of obstructive hydrocephalus

Answer 27

papilledema, HA, vomiting, ataxia

Question 28

what image to confirm pineal gland mass

Answer 28

MRI

Question 29

craniopharyngiomas

Answer 29

suprasellar masses that can compress optic chiasm

Question 30

medulloblastomas location

Answer 30

cerebellar vermis and cause ataxia and hydrocephalus

Question 31

neuroblastoms

Answer 31

sympathetic nervous system | located in adrenal glands

Question 32

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

Answer 32

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

Question 33

cause of hemophiliac arthropathy

Answer 33

iron and hemosiderin deposition leading to synovitis and fibrosis

Question 34

most common nutritional deficiency in children

Answer 34

iron

Question 35

how to prevent iron deficiency anemia

Answer 35

don't switch to cows milk before 1 year old

Question 36

howell jolly bodies

Answer 36

sickle cell

Question 37

pappenheimer bodies

Answer 37

sideroblastic anemia

Question 38

iron panel in thalassemia

Answer 38

dec MCV, dec TIBC | inc iron, increase ferritin

Question 39

iron panel in anemia of chronic disease

Answer 39

decrease in MCV, iron, TIBC and increased in ferritin

Question 40

clinical presentation immune thrombocytopenia

Answer 40

antecedent viral infection asmptomatic petechiae and ecchymosis most common mucocutanwous bleeding

Question 41

treatment children with immune thrombocytopenia

Answer 41

observe skin manifestations | for bleeding give IVIG, or glucocorticoids

Question 42

isolated thrombocytopenia

Answer 42

think about immune thrombocytopenia

Question 43

how to prevent vasoocclusive crisis in sickel cell

Answer 43

hydroxyurea

Question 44

side effect in hydroxyurea Tx

Answer 44

myelosuppresion, (anemia, neutropenia, thrombocytopenia)

Question 45

what is complication in sickle cell TRAIT

Answer 45

hematuria

Question 46

which thalassemia has more Hb A2

Answer 46

Beta

Question 47

iron therapy in thalassemia can lead to what

Answer 47

hemochromatosis

Question 48

treatment aplastic crisis

Answer 48

parvovirus B19

Question 49

acute chest syndrome in sickle cell

Answer 49

fever, chest pain, infiltrate on CXR

Question 50

sickle cell anemia, severe anemia with low or absent reticulocytes

Answer 50

aplastic crisis