Peds 2

Question 1

Rx for methemeglobinemia

Answer 1

Methylene blue.

Acts as e- acceptor for NAPDH and gets reduced to leucomethylene blue and reduces methemoglobin&raquo_space; hemoglobin

Question 2

Rx for lead poisoning:

Answer 2

dimercaprol. Chelates lead&raquo_space; increased urinary/fecal excretion

Question 3

Rx for methanol/ethylene glycol poisoning:

Answer 3

Fomepizole. Inhibits alcohol dehydrogenase and prevents formation of toxic metabolites

Question 4

Rx for organophosphate poisoning:

Answer 4

pralidoxime. Antidote for cholinergic toxicity (organophosphate = AchE I). Given w/atropine to restart AChE

Question 5

Burning erythematous rash, beefy red tongue, diarrhea, poor concentration and irritability in a kid with Crohns. What’s the vit deficiency?

Answer 5

B3 (niacin) - PELLAGRA. Dermatitis, diarrhea, dementia.

Question 6

Why are obese children at higher risk of precocious puberty?

Answer 6

Adiposity triggers insulin release, which causes activation of adrenal glands to produce sex hormones.

** Isolated premature adrenarche is due to early activation of adrenal androgens. Body odor, pubic/axillary hair, oily skin, and acne are common.

Question 7

T/F Physicians are obligated to report cases of vaccine refusal to the local health department.

Answer 7

F

Question 8

MCC of bacterial meningitis in infants > 1 mo old

Answer 8

Neisseria and Strep pneumo

Question 9

Recurrent sinopulmonary infections, bronchiectasis and a PMI displaced to the R in a young kid:

Answer 9

Kartagener syndrome (primary ciliary dyskinesia). Characterized by situs inversus, bronchiectasis, and recurrent sinusitis. Autosomal recessive.

Question 10

Small face and jaw, prominent occiput, low set ears, overlapping fingers:

Answer 10

Edward syndrome (trisomy 18) Would also have limited hip abduction and rocker bottom feet

Question 11

congenital heart defect assoc with Edwards syndrome

Answer 11

VSD (and ASD + PDA to a lesser extent)

Question 12

Which is worse Becker’s or Duchenne’s?

Answer 12

Duchenne’s. This is complete absence of dystrophin whereas becker’s = reduced dystrophin. Both due to XLR del of dystrophin on Xp21. Duchennes shows up at 2-5 beckers at 5-15.

Question 13

Genetic deletion in Turner

Answer 13

45XO

Question 14

Progressive weakness in lower limbs in a teenager. High plantar arches and scoliosis

Answer 14

Friedrich ataxia. Trinucleotide GAA repeat in frataxin gene. Dysarthria, limb weakness, loss of DTR, and progressive gait and limb ataxia.

Question 15

Mucopurulent discharge, bilateral chemosis, severe eyelid swelling in infant 2 days postpartum:

Answer 15

gonococcal infection. Rx = single IM dose of 3rd gen ceph given to infant.

NOTE: All infants regardless of maternal screening results, should be given topical erythromycin within an hour of birth (effective at preventing gonococcal conjunctivitis)

Question 16

Multiple dark pigmented spots on back, abdomen, and extremities, and spots on the iris:

Answer 16

NF1 (von recklinghause). Mut of neurofibromin (NF1 tumor suppressor) on Chr 17. Café au lait, neurofibromas, and lisch nodules (iris hamartomas)

Question 17

Bilateral acoustic neuromas in a kid:

Answer 17

NF2 (central neurofibromatosis). Mut in merlin (NF2 tumor suppressor) on Chr 22.

Question 18

Rx for primary nocturnal enuresis

Answer 18

Desmopressin. Imipramine (TCA) if non-responsive to desmopressin