Random U-World 1

Question 1

signs of severe combined immune deficiency

Answer 1

• recurrent sinopulmon infxn, oral candidiasis, persistent diarrhea, opportunistic / viral infxns
• absent lymph nodes/tonsils
• absent thymic shadow
• abnL T, B, NK cell enumeration by flow cytometry

Question 2

common variable immunodeficiency

Answer 2

• less severe symptoms than Bruton’s agammaglobulinemia
• IgG, A, M, E dec.
• no absence in circulating B cells

Question 3

X-linked agammaglobulinemia

Answer 3

• after 6-9 mo of age, recurrent pygoenic (S. pneumoniae / H. influenzae) infxn
• dec. serum IgG, IgA, IgM, IgE
• absent / dec. B cells

Question 4

wiskott-aldrich syndrome

Answer 4

• x-linked recessive
• eczema, thrombocytopenia, recurrent infxns to encapsulated
• petechaie, bleeding from circumcision / blood stools
• low IgM; high IgA/IgE
• reduced T-cells / platelets

Question 5

Chronic granulomatous disease

Answer 5

• defective phagocytic cells
• dysfunctional NADPH oxidase enzyme complex –> recurrent / uncontrolled infxns w/ catalase-positive
• lymphadenitis / skin/visceral abscesses
• diagnose w/ nitro-blue tetrazolium test

Question 6

beck’s triad

Answer 6

• progression of pericardial effusion to tamponade
• distant heart sounds, distended jugular/scalp veins
• hypotension

Question 7

definitive treatment of pericardial effusion

Answer 7

-pericardiocentesis / pericardiectomy

Question 8

what is the only recommended IV fluid resuscitation in children?

Answer 8

isotonic crystalloid

Question 9

what are risk factors for ECG/echocardiography testing for sudden cardiac death?

Answer 9

-hx ofMarfan, chest pain, dyspnea on exertion, FHx of cardiomyopathy / long-QT, premature cardiac death / disability in a close relative age < 50

Question 10

when is universal lipid screening recommended?

Answer 10

9-11 / 17-21

lipid levels relativelys table prior to / after puberty