Youths These Days
Metabo-What?!
A Breath of Fresh Air
The Tiniest of Humans
Alllll The Erythemas
100

19 yr old woman w/ a history of migraines (involving neurologic symptoms) wishes to begin birth control. An injectable long-acting progestin is recommended. 

Which of the following side effects is associated with injectable progestin-only contraception?

A) Delayed return to fertility

B) Weight loss

C) Endometrial hyperplasia

D) Increased risk of pelvic inflammatory disease

E) Decrease in serum concentration of anti-convulsants

A) Delayed return to fertility

100

14 yr old boy is referred to the Geneticist because he has features suggestive of Marfan Syndrome. The evaluation, however, results in a diagnosis of a different disorder with similar clinical findings.

Which of the following disorders may share clinical findings with Marfan Syndrome?

A) Angelman Syndrome

B) DiGeorge Syndrome

C) Williams Syndrome

D) Noonan Syndrome

E) Homocystinuria

E) Homocystinuria

100

2 wk old Caucasian female was identified w/ elevated immunoreactive trypsinogen (IRT) levels on newborn screening. A mutation analysis shows 2 copies of the delta F508 mutation. 

Which of the following is the most accurate interpretation of the diagnosis & prognosis for this baby?
A) This infant has cystic fibrosis & is likely to develop distal ileal obstruction in the 1st yr of life

B) This infant does not have cystic fibrosis

C) This infant has cystic fibrosis & will not be able to have children in the future

D) This infant has cystic fibrosis & is likely to have or develop pancreatic insufficiency in the 1st yr of life

E) This infant has cystic fibrosis & is likely to develop diabetes in the 1st yr of life

D) This infant has cystic fibrosis & is likely to have or develop pancreatic insufficiency in the 1st yr of life

100

An abdominal XR of a 10 day old infant - 1 of twins born at 29 wks gestation - shows marked abdominal distention associated w/ dilated bowel loops, a small amt of free intraperitoneal air & bubbles of gas in the bowel wall.

Which of the following is most likely related to the pathogenesis of the disorder related to these XR findings?

A) Twin-to-twin transfusion syndrome

B) Meconium ileus

C) Presence of an umbilical artery catheter

D) Parvovirus infection during the 1st or 2nd trimester

E) Feeding, especially formula feeds

E) Feeding, especially formula feeds

100

A well newborn presents for her first WCV at 3 days old. Pregnancy, delivery & nursery course were all uncomplicated. She is feeding & acting well, but Mom is concerned about a rash that broke out over her face yesterday. On exam, you note a well appearing, active baby w/ diffuse, yellow papules & pustules surrounded by large erythematous rings which are present mostly on the trunk & legs (though a few are also seen on the face). 

What is the most likely diagnosis?

A) Miliaria crystallina

B) Acrodermatitis enteropathica

C) Erythema toxicum neonatorum 

D) Transient neonatal pustular melanosis

E) Seborrheic dermatitis

C) Erythema toxicum neonatorum

200

11 yr old girl complains of vaginal discharge. No associated odor, pruritis or burning, but has had "yellowish staining" in her underwear several times. First had breast development about 18 mo ago, has not yet had a period. On GU exam, there is a moderate amount of curly, pigmented pubic hair, and you note clear grayish-white vaginal discharge.

Which of the following best describes the microscopic findings you're most likely to see from a sample of the discharge?

A) Mature pinworms, often surrounded by eggs

B) Multiple motile trichomonads

C) Epithelial cells, few WBCs & lactobacilli

D) Increased number of WBCs associated w/ pseudohyphae & budding yeast

E) Numerous epithelial cells covered w/ adherent refractile bacteria

C) Epithelial cells, few WBCs & lactobacilli

200

6 day old girl, born at home w/ a midwife, presents w/ persistent vomiting. Mom states she's a poor feeder, taking ~1oz of commercial cow's milk-based formula every 3-4 hrs. She is >10% below birth weight, is jaundiced to below the umbilicus & is lethargic. Bilateral cateracts are noted.

Which organism is most likely to cause sepsis in this patient?

A) E. coli

B) Herpes simplex virus

C) Listeria monocytogenes

D) Group B streptococcus

E) Staphylococcus aureus

A) E. coli

200

A teenager presents w/ fever, cough & non-pleuritic chest pain following a month-long excursion exploring caves in northern Kentucky. His chest XR shows hilar adenopathy & focal alveolar infiltrates.

Which of the following is the most likely diagnosis?

A) Histoplasmosis

B) Blastomycosis

C) Aspergillosis

D) Cryptococcus gattii

E) Coccidioidomycosis 

A) Histoplasmosis

200

At 17 wks gestation, an 18 yr old female is noted to have elevated levels of serum alpha-fetoprotein. 

Which of the following findings is most likely to be identified on ultrasound examination?

A) Fetal hydrops & coarctation

B) Isolated myelomeningocele

C) Severe midline facial abnormalities & polydactyly

D) Clenched hands, cystic hygroma & horseshoe kidney

E) Duodenal atresia & endocardial cushion defect

B) Isolated myelomeningocele

200

9 yr old girl is hospitalized w/ intermittent daily fever up to 103.3*F for the last 11 days. The day before admission, she developed a swollen, warm & tender right ankle & left knee, along w/ non-pruritic, erythematous, macular rash associated w/ pale centers on the posterior trunk & extremities. A murmur is noted & EKG shows a prolonged PR.

Which of the following is most likely to be identified with additional evaluation?

A) Elevated antibody titers to Rickettsia rickettsii

B) Elevated antibody titers to EBV early antigen

C) Positive screen for HepB surface antigen

D) Elevated antistreptolysin O (ASO) antibodies

E) Positive blood culture for salmonella typhi


D) Elevated antistreptolysin O (ASO) antibodies [Erythema marginatum]

300

What is the most common cause of death in US adolescents?

A) Heart disease

B) Suicide

C) Homicide

D) Unintentional injury

E) Malignant neoplasms

D) Unintentional injury

300

10 mo old boy comes to establish care. He has developmental delays, poor growth & episodic seizures. Seizures were first noted at 4 mo & his development diminished from there. On exam, he has sparse hair that is very lightly colored & easily broken.

Which of the following tests is most likely to be abnormal?

A) Decreased serum zinc levels

B) Elevated serum ceruloplasmin

C) Decreased serum copper levels

D) Elevated urine metanephrines

E) Increased urinary dermatan sulfate

C) Decreased serum copper levels

300

20 mo old boy is sent to the ED w/ a high pitched, barky cough & in mild respiratory distress, following 2-3 days of URI symptoms w/ a low-grade fever.

What radiographic findings would you expect for this infant?

A) Thickening of aryepiglottic folds & epiglottis

B) Retropharyngeal soft tissue swelling

C) Multiple luminal soft tissue irregularities in the trachea

D) A small radio-opaque intratracheal mass

E) Loss of normal shoulders of the subglottic airway

E) Loss of normal shoulders of the subglottic airway

300

2 mo old baby presents w/ foster parents for evaluation. Little is known about bio mom's history, except that she received no prenatal care & arrived to the ED in active labor. The baby is noted to have several dysmorphic features, including arched eyebrows, hypertelorism, a short & upturned nose, and shortened, stubby digits w/ hypoplastic nails.

Which of the following was the infant most likely exposed to in utero?

A) Phenytoin

B) Methotrexate

C) Retinoic acid

D) Lithium

E) Alcohol

A) Phenytoin

300

12 yr old girl presents w/ complaints of persistent tender bruises on her lower legs. She denies associated trauma or systemic symptoms. On exam, there are several tender, firm, nonfluctuant, dusky-red pre-tibial nodules noted bilaterally.

Which of the following is most likely to be associated w/ this patient's findings?

A) Nystagmus

B) Splenomegaly

C) Oral aphthous ulcers

D) Neurofibromas

E) Yellowish, malodorous vaginal discharge 

C) Oral aphthous ulcers [Erythema nodosum]

400

17 yr old girl comes to the ED w/ 2 days of nausea, vomiting, dizziness & fatigue. Two wks ago, she underwent a primary C/S due to failure to progress. She had considerable bleeding, went back to the OR & required a hysterectomy. She continued to hemorrhage, but stabilized after receiving multiple units of pRBCs, platelets & FFP.

This patient is at risk for which of the following complications?

A) Functional asplenia

B) Myocarditis

C) Hypopituitarism

D) Type 2 Diabetes

E) Autoimmune Hepatitis

C) Hypopituitarism

400

2 day old Amish girl has detectable L-alloisoleucine, which was discovered during a routine newborn screening lab. 

Which of the following is the most appropriate dietary restriction for this patient?

A) Fructose, sucrose, sorbitol

B) Galactose, lactose

C) Phenylalanine

D) Branched-chained amino acids

E) Phenylalanine, tyrosine

D) Branched-chained amino acids

400

2 wk old infant is noted to have continued difficulty feeding. He latches well, but soon after starting to feed, he coughs & begins choking. In addition, his cry is weak & at times indiscernible. He's taken to the OR, where direct laryngoscopy reveals unilateral vocal fold paralysis.

Which of the following is associated w/ an increased risk of vocal cord paralysis?

A) Thyroglossal duct cyst

B) Cystic hygroma

C) Difficult breech delivery

D) Cleft lip/cleft palate

E) Tracheoesophageal fistula

C) Difficult breech delivery

400

During exam on arrival to the NICU, a female infant is noted to be covered in a thick layer of vernix. The areola is slightly raised, but no breast tissue is palpated. Incurving of the ear is noted only in the superior portion. Ear cartilage is scant, and the pinna returns only slowly from folding. Two anterior sole creases are noted. Languo covers most of the body but is absent from the face.

What is the most likely gestational age of this baby?

A) 28 weeks

B) 30 weeks

C) 32 weeks

D) 34 weeks

E) 36 weeks

D) 34 weeks

400

4 yr old girl presents w/ 24 hr history of a pruritic rash. On exam, there are pink patches & edematous plaques w/ dusky centers that appear "targetoid". They are distributed symmetrically on the distal extremities, including the palms & soles. 

What clinical scenario most likely preceded this patient's rash?

A) Purpuric papules & plaques localized to the buttocks & lower extremities

B) Generalized multiple papules & vesicles on an erythematous base, some of which are crusted over

C) Diffusely distributed, erythematous, small, punctate papules, accentuated in the flexural regions

D) Relatively nondescript, erythematous, blanchable macules & papules

E) Grouped vesicles & "punched out" erosions on an erythematous base of the orolabial mucosa

E) Grouped vesicles & "punched out" erosions on an erythematous base of the orolabial mucosa [Erythema multiforme]

500

16 yr old girl w/ anorexia nervosa has been "forcing herself to eat all she can" over the last 2 wks in order to avoid hospitalization. On presentation, she is irritable & appears confused. Vitals show a HR of 130 and RR of 36. A gallop rhythm is noted on CV exam, and she has bilateral lower extremity edema.

Which of the following lab findings is most likely to also be noted in this patient?

A) Hypochloremia

B) Hypophosphatemia

C) Hypermagnesemia

D) Hyperkalemia

E) Hypernatremia

B) Hypophosphatemia

500

3 day old male is being sent home when the RN realizes he doesn't awaken for his heelstick newborn screen. A sepsis evaluation is started. Labs are obtained, including: pH 7.5, HCO3 23, glucose 60, urine negative ketones & negative reducing substances, ammonia 1,500 & urine orotic acid low. 

Which of the following is the most likely diagnosis? 

A) Galactosemia

B) Carbamoyl phosphate synthetase deficiency

C) Proprionic acidemia

D) Ornithine transcarbamylase deficiency

E) Medium-chain acyl-CoA dehydrogenase deficiency

B) Carbamoyl phosphate synthetase deficiency

500

15 yr old boy w/ a hx of recurrent epistaxis is noted to have multiple telangiectasias on his lips, tongue & palate. His mom reports that multiple other family members also have mucocutaneous telangiectasias similar to those of the patient.

For which of the following complications is the patient most at risk of developing?

A) Sudden vision loss due to spontaneous hyphema

B) Pulmonary hemorrhage

C) Recurrent gross hematuria

D) Recurrent synovial & intraarticular hemorrhage

E) Pericardial tamponade

B) Pulmonary hemorrhage

500

A 37 wks gestation male is born & immediately begins to have respiratory distress. Physical exam shows a scaphoid abdomen & decreased breath sounds over one side of the chest.

Which of the following statements is true about this diagnosis?

A) The most common site of herniation is an anterior retrosternal defect on the right

B) The most common site of herniation is a posterolateral defect on the left

C) Other birth anomalies are rare

D) The infant will develop pulmonary hyperplasia on the same side as the defect

E) The most common site of herniation is located just behind the sternum

B) The most common site of herniation is a posterolateral defect on the left

500

7 yr old girl presents w/ a non-pruritic rash and "sleeping with her left eye partly open". She's been well until 4-6 wks ago, when she complained of fatigue, headache & arthralgia - at which time she was dx'd w/ a viral illness & treated supportively. Now on exam, she has multiple annular, erythematous lesions on the trunk & upper extremities. She can't close her left eye & the left corner of her mouth is drooping. 

Which of the following is the most likely cause of these symptoms?

A) Borrelia burgdorferi

B) Herpes simplex virus

C) Bartonella henslae

D) Rickettsia rickettsii

E) Ehrlichia chaffeensis

A) Borrelia burgdorferi [Erythema migrans]