UWORLD

Question 1

Cardiac rhabdomyomas and renal angiomyolipomas associated with

Answer 1

Tuberous sclerosis

Question 2

Genetic neurocutaneous disease. Almost all pts eventually diagnosed with epilepsy. Most have MR or behavioral probs.

Answer 2

Tuberous sclerosis

Question 3

Intermittent muscle weakness triggered by strong emotions. Specific for narcolepsy

Answer 3

Cataplexy

Question 4

Optic gliomas are associated with

Answer 4

Neurofibromatosis type 1

Question 5

Multiple cafe-au-lait macules and neurofibromas

Answer 5

Neurofibromatosis type 1

Question 6

Joints (migratory arthritis), carditis, subcutaneous nodules, erythema marginatum, Sydenham chorea

Answer 6

Rhematic fever

Question 7

Antibiotic for strep throat (group A strep pharyngitis)

Answer 7

Pencillin

Question 8

Positive strept antigen test or elevated anti streptolysin O titer

Answer 8

Rheumatic fever

Question 9

Acute lymphoblastic leukemia is a malignancy of

Answer 9

WBCs

Question 10

Can have either lymphocytosis or lymphopenia, along with anemia or thrombocytopenia

Answer 10

Acute lymphoblastic leukemia

Question 11

Diagnosed when arthritis present for >6 wks. Arthritis is not migratory. See systemic symptoms with a rash

Answer 11

Juvenile idiopathic arthritis

Question 12

Vasculitis. Can cause transient migratory arthritis and a rash. Rash is purpurin. Arthritis usually LEs

Answer 12

HSP

Question 13

Coxsackie B and adenovirus can cause

Answer 13

Viral myocarditis with SOB, syncope, tachycardia, nausea, vomiting, and hepatomegaly

Question 14

Cardiomegaly and pulmonary edema with sinus tachycardia.

Answer 14

Viral myocarditis

Question 15

Gold standard for diagnosis of viral myocarditis

Answer 15

Endomyocardial biopsy

Question 16

See inflammatory infiltrate of myocardium with myocyte necrosis

Answer 16

Viral myocarditis on biopsy

Question 17

Can cause myocarditis. Occurs 2-4 wks after untreated or incompletely treated GAS pharyngitis. Arthritis first manifestation

Answer 17

Acute rheumatic fever

Question 18

Fever for 5 days. Cervical LNs. BL nonexudative conjunctivitis. Rash. Mucosa tis. Swelling/erythema of palms and soles

Answer 18

Kawasaki disease

Question 19

Able to tie shoelaces

Answer 19

Age 5

Question 20

Chantal atresia

Answer 20

Congenital nasal malformation

Question 21

Suspected in newborn with cyanosis aggravated by feeding and relieved by crying. Infants must be able to breathe through mouth

Answer 21

Choanal atresia (congenital nasal malformation)

Question 22

Failure to pass catheter through nares into oropharynx is suggestive of

Answer 22

Choanal atresia

Question 23

Inspiratory stridor exacerbated by exertion or distress. Appears within first few weeks of life

Answer 23

Laryngomalacia

Question 24

Mild obstruction of pulmonary blood flow. Asymptomatic at rest and become cyanotic when stressed

Answer 24

TOF

Question 25

Systolic ejection murmur of pulmonary stenosis and holosystolic VSD murmur

Answer 25

TOF

Question 26

Tachypnea immediately after birth unrelated to crying. Nasal flaring, retractions, and expiratory grunting

Answer 26

TTN