Usmle Ck - Pediatrics 7

Front 1

diamond blackfan syndrome?

Back 1

- clinical picture: macrocytic anemia, low retic, CONGENITAL ANOMALIES (cleft palate, webbed neck)
- defect of erythroid progenitor cells => increases apoptosis
- therapy CS
- ddx megaloblastic anemia b/c no hypersegmented nucleus in neutrophils

Front 2

Fanconi's anemia

Back 2

- progressive pancytopenia, macrocytosis
- around 8 y.o., cafe au lait, microcephaly, short, horsehoe kidney, no thumbs

Front 3

MOA epinephrine? when to intubate?

Back 3

- alpha 1 agonist => vasoconstricts to increase BP
- beta 2 agonist = > counters bronchospasm
- intubate at cyanosis and obtundation

Front 4

pt with CNS infx

Back 4

- bulging fontonelle, neuro defects
- start abx immediately (cefotaxime) THEN CT, THEN LP

Front 5

cushing's triad

Back 5

- low pulse, htn, irregular respirations

Front 6

tet of fallot

Back 6

- baby 1st cyanotic at month+, harsh holosystolic murmur
- if cyanotic 1st day = transposition

Front 7

organisms in chronic granulomatous dz?

Back 7

s auresu, serratia marcescens, burkholderia capacia, klebsiella, aspergillus
- non suspectible to strep pyrogens, strep pneumo, h flu
- tx: daily bactrim, gamma interferon

Front 8

chediak-higashi syndrome

Back 8

- decreased degranulation, chemotaxis, granulopoiesis, occulocutaneous albinism
- dx: neutropenia, GIANT LYSOSOMES in neutrophils
- tx: bactrim, daily ascorbic acid

Front 9

leukocyte adhesion defect hallmark?

Back 9

- neutrophilia without polymorphs in infected tissue or pus

Front 10

JOb's syndrome

Back 10

- hyper IgE, chronic puruitis dermatitis, recurrent staph infx
- hyper IgE, eosinophils, course faical features

Front 11

next step once suspect minimal change dz?

Back 11

- give prednisone
- no need for biopsy (only if adult with nephrotic syndrome)

Front 12

Gram stains
- G+ diplococci
- G + cocci in clusters
- G- cocci
- G+ rods
- G- rods

Back 12

- strep pneumo
- staph
- Neisseria
- Listeria, bacillus
- pseudo, Haemophilus, Klebsiella, legionella

Front 13

severe neutropenia?

Back 13

- staph, pseudo, aspergillus

Front 14

thrombocytopenia, dry skin, otitis media?

Back 14

- WISKOTT ALDRICH SYNDROME

Front 15

breast feeding vs breast milk jaundice?

Back 15

- breast milk jaundice = 1-2 wks after birth, benign
- breastfeeding = 1st few days, because baby not getting enough milk => increases enterohepatic circulation=> see decreased poop, weight loss,

Front 16

galactosemia?

Back 16

- increased conjugated hyperibili

Front 17

when to do phototherapy vs exchange transfusion in hyperbili?

Back 17

- phototherapy: bili 17-25
- exchange transfusion: >25

Front 18

bedwetting

Back 18

normal until 5

Front 19

enuresis both day and night?

Back 19

- check for DM!

Front 20

neonatal tetanus

Back 20

- occurs with poor cord card

Front 21

rubella

Back 21

- toga virus
- transmits via placenta
- infx in 1-4 wks of pregnancy
- infection = IgM or 4x IgG x2 2 weeks apart

Front 22

scarlet fever

Back 22

- s/p group A strep skin infx, burn, sore throat => abd pain, pharyngitits, sandpaper like rash 1st in neck, axilla, groin
- can lead to desquamation
- tx: PCN V, if allergic, erythromycin, clinda

Front 23

premature adrenarche vs pubarche vs thelarche?

Back 23

- axilla hair before age 6 = adrneals = benign
- pubic hair before age 8 = BAD = CNS d/o
- thelarche = breast = ovaries = benign

Front 24

**teenager that starts haing unsteady gait, speech, other neuro issues?

Back 24

- Freiderich's ataxia 2/2 degen of spinal tracts (spinocerebellar, posterior columsn, pyramidal)
- death by cardiomyopathy
- some will develop DM

Front 25

tricuspid atresia

Back 25

- cyanotic heat dz, early cyanosis, EKG = left axis deviation
- no tricuspid => hypertrophied left heart
- need heart defect for survivla

Front 26

congenital diaphragmatic hernia

Back 26

- poor air entry on one side and shift of heart sounds
- do orogastric tube placement; no bag and maks b/c can distend stomach and intestines

Front 27

sickle cell now with severe anemia?

Back 27

- aplastic crisis: parvovirus => no erythropoeisis => see no reticulocytes
- splenic sequestration: pool RBC in spleen, low RBC but high retic => hypotensive shock => splenectomy

Front 28

baby born has left hand paralysis and left eye droops

Back 28

- check eye for miosis = Ipsilateral horner = KLUMPE = injury to 7, 8 cervical nerve and 1st thoracic
- MRI - nerve root avulsion
- tx depends on severity

Front 29

phrenic nerve injury

Back 29

3, 4, 5 cervical nerve => diaphragm paralysis and upper brachial palsy => irregular respitation, non bulgig abd with breathing

Front 30

MCC sepsis in sickle cell dz?

Back 30

- strep pneumo

Front 31

newborn baby doesn't pee, has midline mass?

Back 31

posterior urethral valves = MCC congenital obstructive urethral lesion in males
- do voiding cystogram

Front 32

hyposadias?

Back 32

- penile urethra opens in ventral surface 2/2 icomplete fusion of urethral folds

Front 33

cryptorchidism

Back 33

failure of testes to fall

Front 34

patent urachus

Back 34

- urachal fistula, cyst, sinus

Front 35

child with abd pain and then inbetwe, playfull, nontender belly?

Back 35

- intussusception
- do u/s then air contrast enema
- BELLY ON EXAM IS BENIGN

Front 36

x-linked agammaglobulinemia?

Back 36

- low cd-19 = low b cells
- give IVIG
- CD3 = T cells are ok
- live attenuated vaccines are c/i

Front 37

constitutional growth delay

Back 37

- individual with normal birth weight and height => then at 3 y.o. the growth velocity slows and follows 5th-10th %tile. puberty is delayed but will eventually occur. just wait
- bone radiograph = delayed compared to chronological age

Front 38

congenital hypothyroidism?

Back 38

- hypotensive baby with umbilical hernia and large tongue

Front 39

causes of floppy baby syndrome?

Back 39

- Werdnig Hoffman - degeneration of anterior horn cells and cranial nerve motor nuclei
- botulism: honey and canned foods

Front 40

myotonic congenital myopathy

Back 40

- muscle weakness, atrophy, myotonia, blad

Front 41

post exposure ppx for chickn pox

Back 41

- VZIG or acyclovir for susceptive high risk (low immune,p regnant, premature) within 96hrs

Front 42

neonate in shock?

Back 42

- ddx gastroenteritis, CAH (look for met acidosis, LOW NA), gi obstruction, CF, GBS sepsis, galactosemia
- galactosemia: galactose-1-phosphate uridyl transferase deficiency: vomit, diarrhea, hepatomegaly, catacart, MR, hypoglycemia

Front 43

chloride transport channel malfunction?

Back 43

CYSTIC FIBROSIS

Front 44

esophageal atresia?

Back 44

- MC is tracheosophageal atresia => air into stomach, reflex into lungs
- bad breathing, cant eat, aspiration PNA, rattles heard during breathing

Front 45

child with sudden onset of difficulty walking? clumsy?

Back 45

stroke, 2/2 sickle cel, infx, congeital