Peds 4

Question 1

Rx for Wilson’s Dz:

Answer 1

Chelators (d-penicillamine, trientine) and zinc (interferes with copper absorption)

Question 2

How can you distinguish between central vs peripheral precocious puberty?

Answer 2

Central has high LH at baseline due to hypothalamic secretion of GnRH or following GnRH stimulation.

• Once a CNS tumor is excluded, treatment for central PP is GnRH agonist therapy to prevent premature closure of the growth plates.

Question 3

Kid with aplastic anemia, bent thumbs, small for age, chronic ear infections, and hypopigmented spots on body:

Answer 3

Fanconi Anemia

Question 4

Kid with nonblanching palpable purpura of lower extremities, abdominal pain, arthralgia/arthritis and hematuria:

Answer 4

HSP. IgA leukocytoclastic vasculitis. ** High assoc with intussusception

Rx = supportive care (hydration & NSAIDs, hospitalization and glucocorticoids for severe Dz)

Question 5

Most common side effect of hydroxyurea:

Answer 5

Myelosuppression (neutropenia, anemia, thrombocytopenia)

Question 6

MOA of measles transmission

Answer 6

aerosolized droplets.

Question 7

Mutation assoc with fragile X syndrome:

Answer 7

CGG trinucleotide repeat in fragile X retardation gene of X chromosome.

Question 8

Rx for tinea capitis:

Answer 8

PO griseofulvin or terbinafine

Question 9

Congenital aganglionic megacolon has a strong assoc with what developmental disease?

Answer 9

Down Syndrome (Tri 21)

Question 10

Mutation in Marfan syndrome:

Answer 10

Mut in fibrillin 1

Question 11

Mutation in Ehler’s Danlos

Answer 11

Defective collagen synthesis. Characterized by joint laxity, aortic dilation, scoliosis

Question 12

Defect in fibrillin 2 is found in:

Answer 12

Congenital contractural arachnodactyly. AD mut. Pt has tall stature, arachnodactyly, and multiple contractures in large joints.

Question 13

High fever, sudden onset respiratory distress, dysphagia, and drooling in a child:

Answer 13

Epiglottitis (due to Haemophilus influenza b). Can be life threatening airway obstruction. First line management is intubation in a controlled setting.

Question 14

Fever, urticarial rash, arthralgia, and lymphadenopathy 1-2 weeks after b-lactam and sulfa drug use.

Answer 14

serum sickness like reaction

Question 15

Bilious emesis in a neonate suggesting intestinal obstruction. What tests should you order?

Answer 15

Imaging should be done in stable patients. Abdominal x-ray is done first to check for pneumoperitoneum or perf bowel. Water soluble contrast enema is next step. NO CT. Then can do rectal suction biopsy if suspect hirschsprung

Question 16

Rx for acute bacterial sinusitis:

Answer 16

amoxicillin +/- clavulanate. For strep pneumo or nontypeable h flu

Question 17

Most common complication of sickle cell trait:

Answer 17

gross hematuria (sickling in renal medulla due to low partial pressure of O2)