Usmle Step 2 First Aid Pediatrics

Front 1

What is the most common cause of bowel obstruction in the first two years of life?

Back 1

Intussusception

Front 2

What is the classic triad for intussusception

Back 2

Abdominal pain, vomiting, blood per rectum

Front 3

Red currant-jelly stools are indicative of what?

Back 3

Intussusception

Front 4

What is diagnostic for intussusception

Back 4

Air-contrast barium enema

Front 5

The string sign on a barium study indicates what?

Back 5

Pyloric stenosis

Front 6

What type of acid/base abnormality can occur with persistent emesis, such as that seen with pyloric stenosis?

Back 6

Hypochloremic, hypokalemic metabolic alkalosis

Front 7

What type of immunodeficiency presents after six months of age with recurrent sinopulmonary, GI, and urinary tract infections with encapsulated organisms (H. influenzae, Streptococcus pneumoniae, Neisseria meningitidis)?

Back 7

B-cell deficiencies

Front 8

What immunodeficiency is seen in boys only and presents with frequent pseudomonas infections?

Back 8

X-linked agammaglobulinemia (Bruton's)

Front 9

With what immunodeficiency will there be a drop in immunoglobulin levels to the 20s and 30s, increased pyogenic upper and lower respiratory diseases, and increased risk of lymphoma and autoimmune disease?

Back 9

Common variable immunodeficiency

Front 10

What is the most common immunodeficiency

Back 10

IgA deficiency

Front 11

What does CATCH-22 stand for in DiGeorge syndrome?

Back 11

Congenital heart disease, Abnormal facies, Thymic aplasia, Cleft palate, Hypocalcemia, 22q deletion

Front 12

With what immunodeficiency syndrome will patients have increased infections with fungi and PCP?

Back 12

Thymic aplasia (DiGeorge syndrome)

Front 13

With what immunodeficiency will patients present with cerebellar ataxia and increased incidence of non-Hodgkin's lymphoma and gastric carcinoma?

Back 13

Ataxia-telangiectasia

Front 14

With what immunodeficiency will patients have severe, frequent bacterial infections; chronic candidiasis, and opportunistic organisms?

Back 14

Severe combined immunodeficiency

Front 15

What is the treatment for SCID?

Back 15

PCP prophylaxis, bone marrow transplant or stem cell transplant, IVIG for antibody deficiency

Front 16

With what immunodeficiency will patients present with eczema, increased IgE/IgA, decreased IgM, and thrombocytopenia?

Back 16

Wiskott-Aldrich syndrome

Front 17

What is the classis presentation of Wiskott-Aldrich syndrome (3)

Back 17

Bleeding, eczema, recurrent otitis media

Front 18

With what immunodeficiency will patients present with anemia, lymphadenopathy, hypergammaglobulinemia, and infections with catalase-positive organisms?

Back 18

Chronic granulomatous disease

Front 19

Pts with chronic granulomatous disease require daily treatment with what antibiotic?

Back 19

TMP-SMX

Front 20

With what immunodeficiency will patients present with oculocutaneous albinism, neuropathy, neutropenia, and increased incidence of overwhelming infections with S. pyogenes, S. aureus, and Pseudomonas?

Back 20

Chediak-Higashi syndrome

Front 21

What is the defect in Chediak-Higashi syndrome?

Back 21

Neutrophil chemotaxis (Autosomal recessive)

Front 22

What is the diagnostic test for chronic granulomatous disease?

Back 22

Nitroblue tetrazolium test

Front 23

What immunodeficiency presents with recurrent episodes of angioedema lasting 2-72 hours and provoked by stress or trauma

Back 23

C1 esterase deficiency (autosomal dominant)

Front 24

What type of immunodeficiencies tend to present at 1-3 months?

Back 24

T-cell deficiencies

Front 25

With what immunodeficiency are patients unable to form membrane attack complexes

Back 25

Terminal compliment deficiency (C5-C9)

Front 26

With what immunodeficiency will patients get recurrent miningococcal or gonococcal infections?

Back 26

Terminal compliment deficiency (C5-C9)

Front 27

What type of immunodeficiencies are characterized by mucous membrane infections, gram-negative enteric organisms, and delayed umbilical cord separations?

Back 27

Phagocyte deficiencies

Front 28

What type of immunodeficiencies are characterized by recurrent bacterial infections with encapsulated organisms

Back 28

Complement deficiencies

Front 29

What are the subacute-phase manifestations of Kawasaki disease? (2)

Back 29

Thrombocytosis and increased ESR

Front 30

What are the acute-phase manifestations of Kawasaki disease? Hint: Crash and Burn

Back 30

Conjunctivitis, Rash, Adenopathy, Strawberry tongue, Hands and feet (red, swollen, flacky skin), AND Burn=fever >40 for 5 days

Front 31

What is the treatment for Kawasaki disease?

Back 31

High-dose aspirin and IVIG

Front 32

What treatment is contraindicated with Kawasaki disease?

Back 32

Corticosteroids (may increase aneurysm formation)

Front 33

Untreated patients with Kawasaki disease are at risk for what complications?

Back 33

Coronary artery aneurysms and MI

Front 34

What disease is defined as an acute inflammatory illness of the small airways that primarily affects infants and children under 2?

Back 34

Bronchiolitis (RSV most common)

Front 35

What disease is characterized by the classic barking cough (usually at night)?

Back 35

Croup (Laryngotracheobronchitis)

Front 36

What is the steeple sign?

Back 36

Subglottic narrowing of the airway, commonly seen with Croup

Front 37

With what disease will patients age 3-7 present with muffled voice and drooling?

Back 37

Epiglottitis

Front 38

The thumbprint sign on lateral film is characteristic of what?

Back 38

Epiglottitis

Front 39

What three organisms commonly cause otitis media?

Back 39

S.pneumoniae, H.influenzae, Moraxella catarrhalis

Front 40

What disease is characterized by a slapped-cheek rash that is worse with fever and sun exposure?

Back 40

Erythema infectiosum, caused by Parvovirus B19

Front 41

What disease has an acute onset of high fever followed by a maculopapular rash that appears as the fever breaks?

Back 41

Roseola infantum (HSV-6)

Front 42

An abominal radiograph showing the "double-bubble" sign indicates what?

Back 42

Duodenal atresia

Front 43

What is the most common cause of respiratory distress in infants?

Back 43

Respiratory distress syndrome

Front 44

Ground-glass appearance and air bronchograms on CXR in an infant indicate what disease?

Back 44

Respiratory distress syndrome

Front 45

What are the five Ts and 1P of cyanotic heart disease?

Back 45

1. Truncus arteriosus 2. Transposition of the great arteries 3. Tricuspid atresia 4. Tetralogy of Fallot 5. Total anomalous pulmonary venous return 6. Pulmonary atresia

Front 46

What congenital heart defect presents with a harsh holosystolic murmur heard best at the lower left sternal border?

Back 46

VSD

Front 47

What congentital heart defect presents with a wide and fixed split S2 and a systolic ejection murmur heard best at the upper left sternal border?

Back 47

ASD

Front 48

What drug is given to close a patent ductus arteriosus?

Back 48

Indomethacin

Front 49

What congenital defect often presents in childhood with asymptomatic hypertension?

Back 49

Coarctation of the aorta

Front 50

What is the most common childhood malignancy?

Back 50

ALL