Neuro
Cardio/Respiratory
Nutrition
Metabolic/Genetics
Random
100
A white male infant is delivered after induction of labor at 37 weeks of gestation. The mother is a 28-year-old primipara with worsening preeclampsia and a family history of factor V Leiden mutation. Fetal ultrasonography performed just before induction revealed an abnormal area in the cerebrum along the distribution of the left middle cerebral artery thought to represent an arterial ischemic stroke. Amniotic fluid is clear and Apgar scores are 6 and 8 at 1 and 5 minutes, respectively. Initial physical examination findings are normal. Birthweight is 3.2 kg, head circumference is 34.2 cm, and length 49 cm. Because of the antenatal ultrasonography finding, you admit the infant to special care and discuss likely symptoms of stroke with the nursing staff. Of the following, the MOST common symptom to first appear in neonates with arterial ischemic cerebral infarction (stroke) is: A. abnormal tone B. feeding difficulties C. hemiparesis D. reduced level of consciousness E. seizures
What is seizures
100
A 14-day-old male infant who was born at 26 weeks’ gestational age is in the neonatal intensive care unit. The infant is breathing spontaneously without any need for respiratory support after having assisted ventilation for the first 24 hours after birth and a single dose of surfactant at 3 hours of age for respiratory distress syndrome. You review with the resident the role of surfactant in lowering surface tension at the air-alveolar interface. Of the following, in the presence of surfactant, surface tension in the alveolus: A. approximates the surface tension of pure water B. falls during inspiration C. is inversely related to alveolar radius D. is lowest at end-expiration E. remains constant throughout the respiratory cycle
What is is lowest at end-expiration
100
A newborn presents with gastroschisis. You anticipate the need for prolonged total parenteral nutrition. As you prepare orders for parenteral nutrition, you evaluate the need for addition of trace minerals. Of the following, the trace mineral that requires the EARLIEST supplementation is: A. chromium B. copper C. manganese D. selenium E. zinc
What is zinc
100
You are called to the nursery to evaluate a 1-hour-old infant who has respiratory distress and intermittent cyanosis that is relieved by crying. On physical examination, the normally grown infant has right central facial nerve palsy, and a lop-ear malformation on the right. The genitalia are hypoplastic. A good red reflex is noted on ophthalmoscopic examination, the irides are intact. Of the following, the studies that will be MOST helpful in immediate management and diagnosis for this infant are: A. chromosome analysis, ophthalmology consultation, echocardiography B. chromosome analysis, renal ultrasonography, ophthalmology evaluation C. echocardiography, head computed tomography, ophthalmology evaluation D. head ultrasonography, chromosome analysis, renal ultrasonography E. head ultrasonography, echocardiography, ophthalmology evaluation
What is echocardiography, head computed tomography, ophthalmology evaluation
100
A pregnant woman is involved in a motor vehicle accident involving blunt trauma to the abdomen. You have been called to the emergency department because of potential delivery of the fetus. As you await evaluation by the emergency physicians and obstetricians, you discuss the potential risk to the fetus with colleagues and medical students. Of the following, fetal risk after maternal blunt abdominal trauma is BEST characterized as: A. highly associated with abruptio placenta B. increasing with seat belt useage C. insignificant after 2 hours of normal fetal heart rate monitoring D. inversely related to gestational age E. not correlating with degree of vehicular damage
What is highly associated with abruptio placenta
200
You are meeting with a mother hospitalized for threatened preterm labor, with intact membranes, at 34 weeks’ gestation. She has reduced her activity at home because of a history of previous preterm labor and short cervix. She often listens to music at home. She inquires whether it would be beneficial for her fetus to listen to music in her room. You discuss fetal and newborn hearing development with her and her husband. Of the following, newly born infants born at term or late term: A. are calmed by familiar musical patterns B. are not yet able to discern exogenous auditory stimuli C. prefer lullaby music over voices D. recognize a familiar story only if spoken by mother E. respond only to low frequency (<1,000 Hz) sounds
What is are calmed by familiar musical patterns
200
3. You are making rounds in the normal newborn nursery on a weekend. A 36-hour-old newborn infant has a pulse oximetry reading of 96% in the right leg. You are discussing with the pediatric residents the interpretation of pulse oximetry readings. One resident questions the sensitivity of the screening process for detecting severe heart defects. Of the following, the critical congenital heart defect MOST likely to be missed on pulse oximetry screening is: A. coarctation of the aorta B. pulmonary atresia C. total anomalous pulmonary venous return D. transposition of great arteries E. truncus arteriosus
What is coarctation of the aorta
200
A neonatal intensive care unit (NICU) located in the Midwest established a repository for donor milk in the 1990s to provide nutrition for premature infants whose mothers, for one reason or another, could not supply expressed milk. Several reports suggested that human milk might decrease the incidence of necrotizing enterocolitis in premature infants. They measured the protein and fat content of donor milk routinely. In 2010, two sets of recommendations were published for the management of donor milk banks, one from the United Kingdom and one from Italy. Both sources called for pasteurization of donor milk before use. The NICU then began to follow these recommendations. After 3 years of experience with routine pasteurization, members of the NICU team reviewed their generic data for infants fed with donor milk before and after the change in protocol. The mean fat and protein contents of fortified donor milk had not changed over time. To their surprise, the data indicated that premature infants fed fortified pasteurized donor milk grew with significantly slower velocity than those fed essentially identical but unpasteurized milk. Of the following, the MOST likely cause of the decrease in growth velocity observed is: A. fat malabsorption B. increase in acquired infections C. other care changes over time D. protein malabsorption E. vitamin A deficiency
What is fat malabsorption
200
You are consulted by an obstetrician about a fetus found to have dolichocephaly on ultrasonography. The family is inquiring about the possibility of other abnormalities in the infant and the obstetrician asks you to meet with them. Of the following, this child is MOST likely to fit the diagnostic criteria for: A. Apert syndrome B. Crouzon syndrome C. nonsyndromic craniosynostosis D. Pfeiffer syndrome E. Saethre-Chotzen syndrome
What is nonsyndromic craniosynostosis
200
You are preparing to participate in an international health program in an area with high maternal and perinatal mortality and morbidity rates. Birth spacing, the interval between births, has been shown to influence perinatal outcomes. You consider for whom and in what ways a very short interpregnancy interval (IPI)—the time from delivery of one infant to the onset of the subsequent pregnancy—influences maternal and perinatal outcomes. Of the following, a short IPI (<6 months) is MOST likely to increase the mother’s perinatal risk for: A. gestational diabetes B. mortality C. preeclampsia D. postpartum hemorrhage E. post-term delivery
What is mortality
300
A 39-week-gestation infant with severe perinatal hypoxic-ischemic brain injury continues to have encephalopathy 48 hours after completion of a total body cooling regimen. He was not given any anticonvulsants. He is comatose and without muscle tone, spontaneous movements, respiratory effort, response to stimuli, or pupillary, corneal or gag reflexes. He continues to have apnea after discontinuation of mechanical ventilation, despite an increase in partial pressure of carbon dioxide from 40 mm Hg (5.3 kPa) to 70 mm Hg (9.3 kPa). The parents ask about the possibility of recovery, and whether the infant is “brain dead.” Of the following, the additional test MOST preferred to establish a diagnosis of brain death is: A. brainstem auditory evoked response B. electroencephalography C. magnetic resonance imaging with spectroscopy D. oculovestibular reflex assessment by caloric testing E. radionuclide cerebral blood flow study
What is oculovestibular reflex assessment by caloric testing
300
A 30-week-gestation infant with a birthweight of 1,260 g undergoes intubation at 8 hours of age with a 3.0-mm endotracheal tube for management of respiratory distress. Before intubation, she is noted to have grunting, retractions, and a normal cry, without stridor. Her course is complicated by a pneumothorax, which is treated with tube thoracostomy. Shortly after extubation at 8 days of age, she requires reintubation for severe respiratory distress with stridor, which was unresponsive to racemic epinephrine treatment. She receives minimal maintenance ventilator support, and undergoes extubation again at 10 days of age, with corticosteroid pretreatment. She initially does well, but develops biphasic stridor with suprasternal and subcostal retractions at 11 days of age. Of the following, the factor MOST likely involved in the pathogenesis of this infant’s respiratory distress is: A. edema of the vocal folds B. funnel-shaped lumen at the level of the cricoid cartilage C. generous submucosal tissue layer at the level of the cricoid cartilage D. presence of an incomplete cricoid ring E. use of an uncuffed endotracheal tube
What is funnel-shaped lumen at the level of the cricoid cartilage
300
A family-practice resident calls you about a newborn infant of a diabetic mother. The child is irritable and has hyper-reflexia. The blood glucose concentration is normal. The calcium concentration is pending. Of the following, the elemental deficiency MOST likely to be involved is: A. copper B. chromium C. magnesium D. manganese E. selenium
What is magnesium
300
A 37-year-old woman with 3 normal children opts for chorionic villus sampling at 9 weeks’ gestation because of advanced maternal age. An abnormal chromosome pattern is detected. Of the following, the abnormal chromosome pattern MOST likely to be associated with early fetal loss is: A. monosomy X (45,X) B. trisomy 13 (47, +13) C. trisomy 16 (47, +16) D. trisomy 18 (47, +18) E. trisomy 21 (47, +21)
What is trisomy 16 (47, +16)
300
A woman who recently received a diagnosis of factor V Leiden mutation has no history of bleeding problems or coagulopathy. Recently married, she and her husband are concerned about the effect of this diagnosis on the pregnancy or fetus. Of the following, compared with nonaffected women, women with factor V Leiden mutation during pregnancy: A. are more likely to be of Asian descent B. demonstrate no added hypercoagulability related to pregnancy C. are more likely to experience very early pregnancy loss D. have higher risk for second-trimester losses E. usually have growth-restricted fetuses
What is have higher risk for second-trimester losses
400
A 7-day-old full-term female infant remains in the neonatal intensive care unit because of episodes of hyperpnea alternating with spells of apnea of up to 25 seconds. Physical examination reveals a healthy-appearing child who has nystagmus of both eyes that is unassociated with the respiratory variations. Of the following, the test that is MOST likely to establish the correct diagnosis is: A. electroencephalography B. magnetic resonance imaging of the brain C. MECP2 gene testing D. molecular genetic testing for spinal muscular atrophy E. urine toxicology screen
What is magnetic resonance imaging of the brain Bonus for 1000: What is the diagnosis?
400
A 30-week-gestation fetus with intrauterine growth restriction is noted to have oligohydramnios, tricuspid valve regurgitation, and pulsatile flow in the umbilical vein, indicating myocardial dysfunction. Of the following, the capacity of a fetus to increase cardiac output is limited compared with the older child or adult, PRIMARILY by a (an): A. enhanced response to changes in preload B. higher percentage of noncontractile proteins in the myocardium C. inability to increase heart rate D. increased amounts of stored calcium in the sarcoplasmic reticulum E. limited contribution from atrial systole
What is higher percentage of noncontractile proteins in the myocardium
400
You are discussing the course of a 3-week-old male infant, 1,100 g and 30 weeks’ gestation at birth, who underwent exploratory laparotomy and extensive bowel resection on the 12th day of age. He has received no enteral feedings so far and is supported with standard intravenous alimentation including 2.5 g/kg per day of lipid using a 20% lipid emulsion. The only intravenous lipid available in the United States is made primarily of soybean oil and contains adequate amounts of ω-6 polyunsaturated (essential) fatty acids but little ω-3 polyunsaturated fatty acids. A member of the care team asks if we should anticipate any problems because of ω-3 fatty acid deficiency in this infant. Of the following, the function MOST likely to be impaired in premature infants whose intake is deficient in ω-3 essential fatty acids is: A. leukocyte response B. platelet aggregation C. skin integrity D. visual acuity E. weight gain
What is visual acuity
400
On rounds with your team in the neonatal intensive care unit, you examine a newborn admitted yesterday. The child's initial hypoglycemia has been corrected with the aid of frequent feedings at the breast and a low rate of intravenous glucose infusion. You ask your intern, a PhD-MD, to discuss sources of glucose in the newborn, including glycogenolysis, gluconeogenesis, and lipolysis. Of the following, the amino acid MOST likely to be used in gluconeogenesis is: A. alanine B. citrulline C. glycine D. hydroxyproline E. tyrosine
What is alanine
400
A family medicine colleague calls you about a perplexing report on a patient. She has just received a prenatal ultrasound report from a radiologist describing the placenta as abnormally thickened at 5 cm, at 20 weeks’ gestation. Cystic spaces are seen in the placenta. She inquires as to what, if anything, this finding means to the pregnancy or fetus. Of the following, the condition MOST associated with cystic placentomegaly is: A. congenital nephrosis B. cytomegalovirus infection C. maternal diabetes D. Rh disease E. triploidy
What is triploidy
500
A 34-year-old primigravida visits her obstetrician during the 37th week of gestation because of a perceived reduction in fetal movement. The fetal heart rate was 96 beats per minute and tests of fetal well-being were not reassuring. Emergent cesarean section was performed. The infant weighed 2.6 kg and had Apgar scores of 3 and 7 at 1 and 5 minutes, respectively. Blood gases measured 15 minutes after birth showed a mild metabolic acidosis. At 4 hours of age, he exhibited tonic/clonic movements of both arms and left leg that recurred 3 times during the ensuing hour. Laboratory findings including blood sugar, sodium, calcium, and magnesium concentrations were normal. Seizures were confirmed with standard electroencephalography (EEG). An infusion of pyridoxine during the study did not change the seizure pattern on the EEG. You meet with the parents and inform them of the seizures and your plans for further investigations. You propose administering phenobarbital along with drug monitoring to treat the seizures. You discuss with them the evidence-based information about phenobarbital treatment along with alternatives. Of the following, the BEST information about the use of phenobarbital for seizures in newborn infants is that: A. long-term treatment yields better outcomes than short-term B. phenobarbital is more effective than phenytoin C. side effects (eg, cardiorespiratory depression) are rare D. treatment is more effective for electrical than for clinical seizures E. treatment stops seizures in more than 90% of cases
What is side effects (eg, cardiorespiratory depression) are rare.
500
A 7-week-old male infant who was born at 27 weeks’ gestation is receiving mechanical ventilation with a mean airway pressure of 11 cm H2O and a fraction of inspired oxygen of 0.48. He was born at a referral hospital after a precipitous labor; antenatal corticosteroids were not given. Exogenous surfactant was given, with little improvement in ventilator or oxygen requirements. Recent chest radiographs show normally expanded lung fields with prominent interstitial markings. The level of respiratory support has essentially remained unchanged for the last 6 days. He is tolerating enteral feedings of 135 mL/kg per day of 27 kcal/oz human milk and growing at a rate of 15 g/kg per day. You consider treatment with hydrochlorothiazide and spironolactone. Of the following, the outcome anticipated to be MOST significantly improved with hydrochlorothiazide and spironolactone is: A. lung compliance at 10 weeks B. mortality C. oxygen requirement D. rehospitalization E. sodium supplementation
What is mortality
500
A family physician pleads with you to see the parents of a term newborn. They are dairy farmers and wish to understand the nutritional impact of substituting or supplementing cow milk for human milk. You calmly and respectfully hear them out. Of the following, the substance with a HIGHER concentration in human milk than in cow milk is: A. alpha-lactalbumin B. beta-lactoglobulin C. casein D. fat E. phosphate
What is alpha-lactalbumin
500
You have just attended an urgent cesarean section delivery of a 36-week gestation infant. The mother is a 32-year-old gravida 1 in good previous health. The pregnancy was unremarkable until she presented that day with nausea, vomiting, abdominal pain, and fever. Her examination findings showed hypertension, jaundice, and edema. Maternal laboratory findings included elevated transaminases, hyperbilirubinemia, hypoglycemia, hyperammonemia, prolonged coagulation studies, and thrombocytopenia. Of the following, the MOST likely diagnosis in the infant in the vignette is: A. biliary atresia B. long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency C. medium-chain acyl-CoA dehydrogenase deficiency D. neonatal hemochromatosis E. neonatal hepatitis
What is long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency
500
Chromosome studies were performed on an amniotic fluid specimen because of advanced maternal age. The male fetus’ karyotype was preliminarily reported to the mother as normal, 46,XY. The male child was delivered at term appearing normal. As you review the maternal chart after delivery, you find the final chromosome report to be 46,XY (fragile Xq27.3). The mother has an identical twin sister who is engaged to be married. On being informed of the genetic diagnosis, the mother says she wants no one to know about this diagnosis. You explain to the mother that her sister must be informed, and in your explanation, you invoke your ethical “duty to warn” the sister if she, the patient, is unwilling to do so. Of the following, the use of the “duty to warn” concept in such a situation is supported by: A. American Medical Association Counsel on Ethical Affairs B. American Society of Clinical Oncologists policy C. case law D. federal regulations E. state legislation
What is case law
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