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1. A 3-year-old boy who was involved in a motor vehicle crash sustained a significant head injury and had a Glasgow Coma Scale score of 8 on arrival in the emergency department.An endotracheal tube was placed shortly after his arrival, and the respiratory therapist is providing ventilation at 12 breaths/min with 100% oxygen via a bag-valve mask until a ventilator can be brought to the emergency department.Ten minutes later, the child becomes more restless and agitated. His heart rate has increased from an initial 110 beats/min to about 150 beats/min, and he appears flushed. The monitor indicates that his oxygen saturation is 96% .Of the following, a TRUE statement about this child 's ventilatory status is that

2. A 10-year-old boy (individual 111,1in the pedigree) presents for evaluation of ligamentous laxity and multiple joint dislocations.The family history is notable for a father who has scoliosis and ligamentous laxity, a paternal aunt who has had retinal detachments and mitral valve prolapse, and a paternal grandmother who had joint dislocations and now has osteoarthritis.Based on the family history, the MOST likely pattern of inheritance of this connective tissue disorder is

3. You are asked to evaluate a 1-day-old infant who has mild clitoromegaly and palpable masses in the labial folds bilaterally. Chromosome studies reveal that the infant has an XY karyotype.Of the following, additional history is MOST likely to reveal that the infant's

4. The parents of a child who was diagnosed at birth with Beckwith-Wiedemann syndrome bring in the baby for his 2-month evaluation.They ask about future health problems and his prognosis now that his omphalocele has been repaired. Of the following, the child is MOST at risk for

5. You are discussing the common indications for chromosome analysis with a group of third-year medical students. Of the following, the MOST appropriate statement to include in your discussion is that

6. You and your colleagues are discussing implementation of routine developmental screening in your office.In your research, you have found that

7. A term infant is delivered vaginally following a pregnancy complicated by diabetes mellitus.His oral and nasal airways are suctioned and found to be patent and free of meconium.He has cyanosis and respiratory distress immediately following birth that requires intubation and assisted ventilation with 100% oxygen.Because no improvement is apparent in the next 5 minutes, he is admitted to the neonatal intensive care unit.His birth weight is 4,500 g.A chest radiograph reveals fmdings consistent with decreased pulmonary blood flow. Of the following, the MOST likely cause of respiratory distress in this infant is

8. You are seeing a 14-year-old boy in your office who complains of fever, facial pain, and cough at night.His mother states that he has had problems with sinusitis for the past few months and has required repeated antimicrobial therapy during this period. Physical examination reveals a tempera ture of 101°F (38.3°C), yellow-green nasal discharge, and tenderness to palpation of the maxillary and frontal sinus regions. Of the following, the MOST likely pathogen associated with chronic sinusitis is

9. You are seeing a 2-month-old male infant who has trisomy 21for a health supervision visit. The boy's mother expresses concern that the infant has been having ''noisy breathing'' during the past 2 to 3 weeks.The infant has been exclusively formula-fed and has had no choking or difficulty feeding. According to the mother, the noise, which occurs on inspiration, is louder when the infant is supine and when crying.She has not noticed any rhinorrhea, cough, or other upper respiratory viral illness symptoms.The infant was born via an uneventful vaginal delivery that did not require forceps.Apgar scores were 8 and 9 at 1and 5 minutes, respectively. On physical examination, the infant, whose physical appearance is consistent with trisomy 21, is resting comfortably. His vital signs are appropriate for age, but you hear an audible noise during inspiration. Of the following, the MOST likely explanation for the infant's respiratory symptoms is

10. A 2-year-old boy is brought to your clinic because he has a nighttime cough. According to his mother, several times over the past few months he has awakened with a barking, nonproductive cough that improves by the next morning. She denies fever and rhinorrhea with the episodes. Evaluation of his lungs yields normal results. Of the following, the clinical feature that is MOST suggestive of spasmodic croup rather than recurrent laryngotracheobronchitis in this boy is

11. You are evaluating a 7-year-old boy who has sickle cell disease for a 5-day history of fever, rhinorrhea, headache, and worsenin g fatigue.Physical examination reveals a tired-appearing, pale boy who has a temperature of 101.5°F (38.6°C); heart rate of 150 beats/min; very pale conjunctivae and mucous membranes; and a faint diffuse, erythematous, lacy rash that is most prominent on his cheeks and trunk. Laboratory tests show a white blood cell count of 12x103/mcL (12x109/L), with 50o/o neutrophils, 45% lymphocytes, and 5o/o monocytes; hemoglobin of 4 g/dL (40 g/L); hematocrit of 16% (0.16); and reticulocyte count of less than 1% (0.01). Of the following, the MOST likely cause for this patient's present illness is infection with

12. A 3-year-old boy presents to the emergency department following the abrupt onset of coughing and wheezing. You order a chest radiograph for evaluation of a suspected foreign body aspiration.Of the following, the MOST appropriate statement regarding foreign body aspiration is that

13. A 15-year-old girl who hasjuv enile rheumatoid arthritis has been treated with ibuprofen 30 mg/kg per day for 3 months. She has had epigastric abdominal pain for 1month that has been unresponsive to empiric therapy with omeprazole 20 mg/day.You are considering adding misoprostol 100 mcg four times daily to her current treatment regimen. Of the following, a TRUE statement regarding misoprostol is that the drug

14. The mother of an infant born at 34 weeks' gestation asks you whether any of the medications she was given prior to delivery will have any effect on her daughter's growth and development. Of the following, the MOST correct statement concerning drugs commonly used in labor is that

15. A 3-month-boy who has been previously healthy is brought to the emergency department with a 3-day history of rhinorrhea, mild cough, and wheezing.He has been afebrile and has had some difficulty feeding. His pulse oximetry reading is 90o/o on room air, respiratory rate is 60 breaths/min, and heart rate is 130 beats/min. Chest examination reveals mild subcostal retractions, scattered wheezes, and coarse crackles bilaterally. The rest of the physical examination fmdings are normal.Of the following, the pathogen that is MOST likely responsible for his symptoms is

16. A 17-year-old girl presents with amenorrhea of 6 months' duration.One year ago she joined the cross country team at school. At that time, her periods had been regular, about every 26 days, and remained so for the 3-month runnin g season.At the end of the season, she continued to run 5 miles a day to be more competitive for the subsequent season. She noticed that her menstrual flow was lighter for a few months preceding the amenorrhea. You suspect exercise-induced amenorrhea and recommend a decrease in exercise. Of the following, the factor MOST likely to be associated with a low bone density and stress fractures in this patient is

17. You diagnose attention-deficit/hyperactivity disorder in an 8-year-old girl and initiate therapy with a daily morning dose of long-acting methylphenidate.Her mother asks about using stimulant medication after school, on weekends, and during the sum mer. Of the following, your BEST response is that

18. A 14-year-old boy has a body mass index that is greater than the 95th percentile for age and an accelerating weight curve.His blood pressure is 135/85 mm Hg. His mother has type 2 diabetes that developed during her first pregnancy, and several paternal relatives also have type 2 diabetes.The family emigrated from the Caribbean when he was a toddler.He has dark velvety thickening of the skin on his neck and under his arms.Of the following, the findings that are MOST supportive of a diagnosis of metabolic syndrome in this young man are

19. You are present at the birth of an infant in whom bilateral hydronephrosis was diagnosed in utero.A fetal shunt was placed in each flank between the renal pelvis and the amniotic cavity. Nonetheless, the infant has bilaterally palpable flank masses, and the shunts are not apparent at birth. The infant shows no dysmorphisms and has no respiratory distress.Renal ultrasonography reveals bilateral hydronephrosis.Of the following, the MOST correct statement regarding the fetus/neonate who has obstructive uropathy is that

20. Numerous therapeutic agents are known to have teratogenic effects on the developing fetus.Of the following, the findings in the newborn that are MOST suggestive of prenatal exposure to an angiotensin-converting enzyme inhibitor are

21. The director of a community after-school program for adolescent boys is organizing an orientation session for new mentors.He wants some information about current substance abuse trends.Of the following, you are MOST likely to report that among adolescents,

22. You are evaluating a 6-year-old boy who has Duchenne muscular dystrophy. He is doing well in a regular classroom and will be attending second grade in a different school next year. On physical examination, you note a healthy-appearing boy who has pseudohypertrophy of the calf muscles and uses a Gower maneuver to rise from the floor.Ingathering information to help this child 's transition to a new school, you are MOST likely to ask about

23. An 11-day-old infant presents to the clinic with a history of a temperature of l00.8°F (38.2°C) and a 1-day history of poor feeding.Findings on physical exami nation are normal.You initiate a sepsis evaluation that includes a lumbar puncture.The cerebrospinal fluid results demonstrate a white blood cell count of 6x103/mcL (6x109/L), with 68o/o neutrophils, 2% bands, and 30% lymphocytes.The protein concentration is 200 mg/dL (2 g/L), and the glucose value is 36 mg/dL (2.0 mmol/L).The abnormal findings prompt you to order magnetic resonance imaging, which demonstrates abnormal frontal lobes bilaterally that includes some degree of infarction but also abscesses and cerebritis. Of the following, the MOST likely pathogen is

24. An 11-year-old highly competitive gymnast presents to your office with complaints of increasing right wrist pain over the past 2 to 3 months. She recalls no specific trauma to the wrist or arm. On physical examination, you note no swelling of the distal forearm or wrist.She has normal range of motion at the wrist.There is moderate-to-marked tenderness of the distal radius, but no tenderness more distally over the wrist joint.A radiograph of the forearm shows slight widening of the distal radial physis.Of the following, you are MOST likely to explain to the child and family that

25. You have ju st diagnosed Ebstein anomaly in a newborn. Of the following, the MOST likely prenatal exposure to result in this abnormality is

26. A 14-year-old-boy has been followed yearly by another physician. When you see him for the first time, he brings records that you use to construct a growth curve (Item Q186A).His parents state that they are not worried about his growth because his 20-year-old brother was a slow grower and still seems to be growing a little.Physical examination reveals 6-mL testes and Sexual Maturity Rating 2 pubic hair. There are no other signs of puberty. His bone age on radiography is 12 years. Of the following, the MOST appropriate suggestion for the family is that

27. You see a 9-month-old girl for a health supervision visit.She is in her infant carrier with a propped bottle.The mother explains that the infant does not sleep through the night, which is disrupting the mother's ability to function well at work.The infant appears well, with length and head circumf erences at the 25th percentile and weight at the 95th percentile.During the examination, the infant cries. The mother hands you a bottle and asks if she may leave to check on a referral for her older child. Of the following, you are MOST likely to suggest to the mother

28. A 4-month-old boy presents to the clinic with a 2-day history of a temperature of 100.6°F (38.1°C) and vomiting.The infant was born at 26 weeks' gestation, and his birthweight was 960g. He remained in the hospital for 3 months after birth because of multiple complications, including bilateral grade 4 intraventricular hemorrhages necessitating the placement of a ventriculoperitoneal shunt 6 weeks ago.Physical examination reveals an afebrile infant who has a shunt bubble on the right temporo-occipital region and a gastrostomy button.You explain that his shunt bubble requires aspiration to evaluate the cerebrospinal fluid for infection. Of the following, the organism that is MOST likely to be isolated in the aspirate is

29. A 16-year-old previously healthy boy presents with a 2-week history of intermittent elevated temperatu res to 102°F (39°C), headache, malaise, fatigue, myalgias, and a progressively worsening nonproductive cough. During history-taking, be reports that recently he explored several caves while hiking in a forest preserve in Ohio.Physical examination reveals a tiredappearing adolescent who has a temperature of 102.4°F (39.1°C), a dry cough, and diffuse intermittent rhonchi on chest auscultation. Laboratory findings include a white blood cell count of 12x103/mcL (12x109/L), with 60% neutrophils, 2% band forms, and 38% lymphocytes. Chest radiograph (Item Q190A) shows patchy left upper and left lower lobe opacities and hilar adenopathy.Of the following, the MOST likely pathogen causing this patient's condition is

30. An African-American mother brings her previously healthy 2-year-old son to the emergency department because he looked pale when he woke up this morning.She also reports fever and decreased activity over the last few days.He was diagnosed with a urinary tract infection and given trimethoprim-sulfamethoxazole 3 days ago.On physical examination, he is markedly pale and appearsja 11ndiced. His heart rate is 170 beats/min, and his respiratory rate is 30 breaths/min.The rest of his physical examination findings are unremarkable.Laboratory evaluation reveals a hemoglobin concentration of 5 g/dL (50 g/L).Of the following, the MOST likely cause of this boy's anemia is

31. A 3-year-old girl is brought to your office for re-evaluation of a fever that began 6 days ago.Her mother tells you that her daughter 's temperature has been as high as 102.2°F (39°C).Her physical examination was unremarkable when you examined her 3 days ago, but today you note injected sclera; cracked, red lips; a strawberry appearance of her tongue; and a swollen, nontender, cervical node.You tell her mother that you believe this is Kawasaki disease. Of the following, the MOST appropriate statement to make to the mother is that

32. The mother of a 14-year-old boy arranges to meet with you privately before the boy 's annual health supervision visit.She is concerned because he is quiet, has no athletic interests, and has only a few friends.He is content to spend the weekend shopping, cooking, reading, and listening to music.Although he doesn't like school, he is an honor student.The mother also tells you her husband 's youngest brother recently disclosed his homosexuality and wants to introduce his male partner to the extended family. Of the following, while counseling the mother, you are MOST likely to include a statement that

33. You are evaluating a short 14-year-old boy who is underweight for height. His growth curve is shown in Item Q202A. He says he is feeling well. His mother worries that he does not have as much stamina as he did at age 12 years.Of the following, the laboratory study that is MOST likely to be useful in assessing the reason for poor growth is measurement of

34. You are asked to review a case for morbidity and mortality conference.The infant was born at term to a 19-year-old gravida 1, para 1woman by normal spontaneous vaginal delivery. The mother was known to be group B Streptococcus-negative, but she did have genital warts.The Apgar scores were 9 at 1minute and 10 at 5 minutes. On the seventh postnatal day, the infant developed a temperature of 103°F (39.4°C) and was brought to the emergency department.At this time, the infant was in shock and required mechanical ventilation. Physical examination revealed scleral icterus and hepatosplenomegaly but no skin lesions.A lumbar puncture could not be performed.Laboratory results include: White blood cell count of 2.34x103/mcL (2.34x109/L), with 32% lymphocytes, 41% neutrophils, 8% bands, 15% monocytes, 3% eosinophils, and 1% basophils, Hemoglobin of 7.1g/dL (71g/L), Hematocrit of 21% (0.21), Platelet count of 40x103/mcL (40x109/L), Prothrombin time of 41.2 seconds, Activated partial thromboplastin time of >106 seconds, Aspartate aminotransferase concentration of 3,086 U/L, Alanine aminotransferase concentration of 456 U/L, Total bilirubin of 4.4 mg/dL (75.2 mcmol/L). The chest radiograph demonstrated diffuse interstitial infiltrates bilaterally (Item Q204A).The patient did poorly over the next 3 days and died despite aggressive management in a pediatric intensive care unit. Of the following, the MOST likely cause of this patient's death is

35. A 5-month-old female infant presents with a 1-day history of fever to 102°F (38.9°C), emesis, and multiple episodes of greenish diarrhea with streaks of blood. Her mother states that the infant is less active, sleepier, and has no interest in feeding. Physical examination reveals a listless inf ant who has a sunken anterior fontanelle, dry mucous membranes with decreased skin turgor, and skin irritation in the diaper area. Of the following, the MOST likely cause of this patient's gastroenteritis is

36. A mother brings in her 2-year-old boy for food avoidance education after he was seen in the emergency department last week for anaphylaxis following peanut ingestion. This is the third anaphylactic episode after peanut ingestion since he was diagnosed with a peanut food allergy at age 1year.At that time, he was evaluated by a pediatric allergist and determined to have positive skin tests and an elevated serum immunoglobulin (lg) E to peanut antigen, consistent with an lgE-mediated allergy. The mother is frustrated and would like advice regarding peanut food allergy. Of the following, the BEST advice at this time is to

37. The family of a diabetic patient in your practice requests evaluation for celiac disease.They have heard from other families of children who have diabetes that patients who have type 1diabetes are at increased risk for this condition. Of the following, a TRUE statement regarding celiac disease screening is that

38. A newborn in your neonatal intensive care unit has had intermittent seizures for 72 hours.You have been unable to control the seizures with phenobarbital, hydantoin, an.d lorazepam. Electrolyte, calcium, magnesium, and glucose concentrations are normal. The infant subsequently becomes apneic, comatose, and unresponsive.Of the following, the BEST laboratory test to determine the cause of coma in this infant is

39. During clinic rounds with medical students, you are seeing a 2-year-old girl who has otitis media.One of the students asks about the potential complications of otitis media. Of the following, the statement you are MOST likely to make is that

40. An 18-year-old girl presents for a health supervision visit prior to leaving for college.She has a letter from the college she plans to attend instructing entering freshman who choose to live in the dormitories to obtain a meningococcal vaccination. You review vaccine information and obtain consent for the immunization.Of the following, an ACCURATE statement about recommendations for the use of the meningococcal MCV4 vaccine is that it

41. You are seeing a 6-year-old girl whose mother is concerned about the girl masturbating. For the past several months, the girl has touched her genitals while watching television with her mother. The parents have been divorced for 2 years, and the girl spends weekends with her father.You question her mother further.Of the following, the response that MOST increases your suspicion for behavior that is out of the norm is

42. The mother of a 4-month-old infant is planni ng a winter trip to the tropics with her infant and asks about the use of sunscreens and the safe amount of sun exposure for the infant.Of the following, the MOST appropriate advice for the infant is to

43. A 5-month-old inf ant presents with a history of vomiting between 10 and 20 times a day. She is growing and developing normally. There is no blood in the vomitus, no respiratory symptoms, and no history of apnea.The parents are frustrated from cleaning up after the baby vomits and want something done.Physical examination and upper gastrointestinal radiograph evaluation results are normal. Of the following, the MOST accurate statement about this patient is that she

44. A 4-year-old girl remains intubated, mechanically ventilated, and completely unresponsive in the intensive care unit following a massive subarachnoid hemorrhage of unknown cause. She is hemodynamically stable and not receiving any sedating medications. She has a temperature of 98.1°F (36.7°C) and has no evidence of infection. The girl has not exhibited any brainstem or cerebral function for more than 24 hours. Of the following, the statement you are MOST likely to make while on rounds with the resident team is that

45. A 4-month-old child is admitted to the hospital for evaluation of failure to thrive and generalized seizures.On physical examination, the child appears wasted and has a protuberant abdomen and marked hepatomegaly.Laboratory evaluation reveals fasting hypoglycemia, lactic acidosis, hyperuricemia, and hyperlipidemia.The boy 's parents are first cousins. Of the following, the BEST long-term management of this disorder is

46. A 17-year-old boy presents for a sports physical. He has a learning disability and is shy. His height is at the 75th percentile, and his body mass index is at the 85th percentile.Physical examination findings include minimal facial hair, bilateral gynecomastia (breast >4 cm in diameter), and small testes (testicular volume of 6 ml,). Of the following, the MOST likely cause of this patient's gynecomastia is

47. You are evaluating a 5-year-old boy who has cerebral palsy and mental retardation, is fed through a gastrostomy tube, and is dependent for all his care.He will be attending a full-day program at the school in which he previously was enrolled. His parents are divorced, and his mother is his primary caretaker.She will begin working while he is in school.He has a 10- yearold brother with whom he shares a room and who alerts his mother when his brother needs help at night. Of the following, the concern you are MOST likely to address is

48. You are discussing diarrheal diseases with a group of medical students interested in international health.You advise them that there are more than 2,000 serovars of Salmonella. Of the followin g, the serovar that has the MOST public health implications is

49. The father of three children in your practice recently was diagnosed with Crohn disease.His wife does not have Crohn disease.He asks you if his children, ages 10, 12, and 16 years, are at increased risk for developing the same illness.Of the following, you are MOST likely to advise the father that

50. During a health supervision visit of a 6-year-old child, you ask the mother if there are any guns in the home.She states that her husband is a hunter, but he keeps his shotgun in his pickup truck. Of the following, the BEST anticipatory guidance with regard to firearm safety is to tell the mother to

51. You are seeing a new patient who is 11years old and has Duchenne muscular dystrophy.His uncle died of the disorder 2 years ago.His mother asks to see you privately and tells you that her son does not know his diagnosis; she asks that you refer to his ''walking problem.'' On physical examination, you note that the boy is in a wheelchair and is unable stand without support.You ask to speak with the mother after evaluating the boy. Of the following, you are MOST likely to tell her that

52. A 5-year-old girl presents after having a brief generalized seizure.Her mother reports that the child has had a 3-day history of fever, tenesmus, and bloody diarrhea.On physical examination, you find a mildly toxic-appearing child who has a temperature of 104°F (40°C) and diffuse abdominal tenderness.The rectal examina tion produces significant pain. Stool from her rectum is guaiac-positive.You tell her mother that you believe the diarrhea has an infectious cause. Of the following, the MOST likely pathogen is

53. A 3-month-old boy is admitted to the hospital for evaluation of failure to thrive. His birthweight was at the 50th percentile and length at the 75th percentile. Currently, his weight is below the 5th percentile and length is at the 25th percentile.His vital signs and physical examination results are otherwise normal. He appears well hydrated.Measurement of serum electrolytes reveals:sodium, 139 mEq/L (139 mmol/L); potassium, 4.7 mEq/L (4.7 mmol/L); chloride, 114 mEq/L (114 m mol/L); bicarbonate 12 mEq/L (12 mmol/L); blood urea nitrogen, 8 mg/dL (2.9 mmol/L); and creatinine, 0.3 mg/dL (26.5 mcmol/L).A consulting nephrologist recommends measurement of urine pH (which is 7.5) and urine ammonium (which is 12,000 mcM/L) (normal, >60,000 mcM/L). Of the following, the MOST likely cause of this child's acidosis is

54. You are asked to evaluate two children, ages 3 and 9 years, on the pediatric hematologyoncology inpatient unit who have developed fever, cough, increased work of breathing, and nodular lesions on their chest radiographs.The children are isolated in private rooms and have different nurses and doctors caring for them.The children 's rooms are located adjacent to an area where a new playroom is being constructed. Of the following, the MOST likely pathogen causing these patients' pneumonia is

55. A 15-year-old boy presents with melena and anemia.Endoscopy demonstrates a nodular gastritis of the antrum and an ulcer.Biopsies of the antrum demonstrate spiralshaped organisms consistent with Helicobacter pylori.You prescribe amoxicillin, clarithromycin, and lansoprazole for 2 weeks.At a follow-up visit, the family asks whether the treatment has been successful in eradicating the organism. Of the following, the PREFERRED noninvasive test to evaluate whether the pathogen has been eradicated is

56. A term newborn is delivered by emergent cesarean section because of intrauterine growth restriction, oligohydramnios, and nonreassuring fetal heart rate monitoring in labor.Delivery room resuscitation includes endotracheal intubation and assisted ventilation with lOOo/o oxygen, chest compressions, intravenous epineph rine, and volume expansion.Apgar scores are 1, 2, and 3 at 1, 5, and 10 minutes, respectively.An umbilical cord arterial blood gas measurement documents a pH of 6.9 and a base deficit of 20 mmoVL.At 12 hours of age, the infant demonstrates tonic-clonic convulsive activity of the arms and legs with a concomitant decrease in heart rate and bedside pulse oximetry saturation. Of the following, the MOST likely cause for this infant's seizure is:

57. You are precepting a resident who has just evaluated a 4-year-old incompletely immunized immigrant boy who has classic varicella lesions and a history that is consistent with this diagnosis.Of the following, the MOST accurate statement is that

58. The parents of a child who has Down syndrome and a 47,XX+21karyotype come to you for counseling about future pregnancies. Of the following, their risk for giving birth to another child who has trisomy is CLOSEST to:

59. A 15-month-old infant has been breastfed since birth.He eats finger foods (eg, peas, carrots) and occasionally some cereal.His mother adheres to a vegan diet and plans the same for her child.A complete blood count documents anemia. Of the following, the MOST likely cause of this infant's anemia is a deficiency of:

60. A 15-year-old boy comes to your office for a health supervision visit.He expresses concern that he is only 5 ft, 2 in tall and is not competitive in track. On physical examination, he appears healthy, has a height of 62 in, and weighs 96 lb. His testes are 8 mL in volume bilaterally, there is slight pubertal phallic enlargement, and he has Sexual Maturity Rating 3 pubic hair. He has a small amount of subareolar breast tissue. His last health supervision visit was 2 years ago.He did not have pubic hair at the last visit, and his testes were described as ''prepubertal'' in size. Of the following, the MOST likely cause of his short stature is

61. You are examining a 2-year-old girl who has a 6-month history of developmental regression. During her first postnatal year, she met all motor, language, and social milestones.Her head circumference, which currently is at the 3rd percentile, was at the 75th percentile at birth. On physical examination, she makes poor eye contact and repetitively wrings her hands. Of the following, the MOST appropriate diagnostic test is:

62. You are conducting rounds in the newborn nursery with a group of residents.You describe the choices for infant nutrition that might optimize growth and development.Of the following, you are MOST likely to state that:

63. A 3-year-old child who has a history of recurrent otitis media with effusion (OME) in infancy is brought to the clinic.His mother is afraid that he has a hearing loss because he does not talk as much as his brother did at the same age.He speaks in three-word sentences, and you can understand fewer than 50% of his words.Results of his physical examination, including the ears, are normal.Of the following, the MOST appropriate statement regarding this child's condition is that:

64. You are asked to see a term infant in the newborn nursery at 8 hours of age and consider her transfer to the neonatal intensive care unit. The problems and fmdings reported to you include: poor feeding, emesis, temperature instability (core temperature of 96°F [35.5°C]), hypoglycemia (whole blood glucose concentration of 25 mg/dL [1.4 mmol/L]), and polycythemia (hema tocrit of 70o/o [0.70]).Of the following, the physical finding that is MOST likely to accompany these problems is

65. A normal-appearing 9-month-old boy is discovered on routine herniorrhaphy to have bilateral Fallopian tubes and a rudimentary uterus.Biopsy of the gonads performed during this procedure revealed normal testicular tissue. On examination today, his phallus is normal in size and appearance, and his descended testes are both 2 ml, in volume. Of the following, the MOST likely sex chromosome complement for this child is

66. An 8-year-old boy has difficulty with reading; he reads slowly and makes many mistakes.He has a history of a febrile seizure at 1year of age and a fall at age 2 years that resulted in a brief loss of consciousness.His mother read an article about interventions for improving vision function in children who have reading difficulties and would like your opinion.Findings on his physical examination are normal, except for vision of 20/40 bilaterally. Of the following, your BEST response is that you would like to

67. The mother of a child who is infected with human immunodeficiency virus (HIV) would like to enroll her child in a local child care center. Of the following, the circumstance that is MOST likely to exclude the child who has HIV infection from attending a child care center is

68. You see an 8-year-old boy in accident and emergency who fell off his bike 3 days ago and scraped his left calf. The cuts are now angry, red and painful.You note he is a big boy and plot his growth: his weight is on the 99th centile and height is on the 15th centile.You note mild gynaecomastia and stretch marks on his abdomen which are normal skin colour.His past medical history is unremarkable except for mild asthma.What is the most likely cause of his large size?

69. A 16-year-old boy is brought to the GP by his parents.They are concerned he is the shortest boy in his class.He is otherwise well.His height and weight are on the 9th centile.His father plots on the 75th centile and his mother on the 50th centile for adult height.On exami nation, his testicular volume is 8 mL, he has some fine pubic and axillary hair.The rest of the physical examination is normal. On further questioning you elicit from his father that he was a late bloomer and did not reach his full height until he was at university. What is the most likely cause of the boy 's short stature?

70. An 8 year old known asthmatic is brought into accident and emergency by ambulan ce as a 'blue call'.He has been unwell with an upper respiratory tract infection for the past 2 days.For the past 24 hours his parents have given him 10 puffs of salbutamol every 4 hours, his last dose being 90 minutes ago.The ambulance staff have given him a nebulizer but he remains agitated with a heart rate of 155, respiratory rate of 44 and sub/intercostal recessions and on auscultation there is little air movement heard bilaterally.Saturations in air are 85 per cent.He is started on 'back to back' nebulizers with high flow oxygen. How severe is his asthma exacerbation and what other bedside test would support this?

71. A 6-year-old boy with a history of anaphylaxis to peanuts is brought in by ambulance unconscious. He was attendin g a children 's birthday party. His mother says there was a bowl full of candy and he may have eaten a Snickers bar but she is not sure and she did not have bis EpiPen with her.His face and lips are swollen and erythematous, he is still breathing but weakly and there is wheeze.His pulse is tachycardic and thready.Which type of shock is this?

72. A newborn baby is born to non-consanguineous parents.She is noted to have puffy feet on her 1st day check. She weighs 2.0 kg with widely spaced nipples and absent femoral pulses.You have asked your registrar to review her as you think she may have Turner 's syndrome. She agrees and asks you to send blood tests for karyotyping.Which is the chromosomal diagnosis of Turner's syndrome?

73. A baby is born and you are asked to do the baby check at 6 hours post-natal age.You go to see the baby and mum states that he has not yet had a feed. You advise they stay in hospital until the feeding is established.This is the first child of non -consanguineous parents.On day 4 when you review the baby he has still not had an adequate intake, has lost over 10 per cent in birth weight and is markedly hypotonic.Your consultant asks you to request genetic testing for Prader-Will syndrome.What is the inheritance of Prader-Willi syndrome?

74. A 5-day-old baby who is formula fed is on the neonatal unit being treated for sepsis secondary to an Escherichia coli urinary tract inf ection.He has been on antibiotics for 5 days.He is still unwell and vomiting. The parents are consanguineous and this is their first child. He has had repeat blood and urine cultures taken. Urine reducing substances are positive.What is the most likely 11nderlying diagnosis?

75. A 10-year-old boy is brought to the GP with tall stature.He is taller than his peers at school.His arm span is greater than his height, he has long, thin fmgers, scoliosis and pectus excavatum. He is also concerned that he gets short of breath at school during PE lessons.You refer him for an echocardiogram and chest x-ray.You make a clinical diagnosis of Marfan 's syndrome.What is the inheritance of Marfan 's syndrome?

76. A 1-day-old baby is on the post-natal ward. You are asked to review her as she is febrile and lethargic.On examination she is tachycardic, has a capillary refill time of 3 seconds centrally and reduced urine output.Her blood culture 24 hours later grows Gram-positive cocci.Which is the most likely causative organism?

77. A preterm baby is born at 25 + 6 weeks gestation. He is delivered by caesarean section due to maternal pre-eclampsia. He is intubated at birth and given surfactant via the endotracheal tube. He is ventilated and commenced on IV dextrose.After 4 hours of age he has increased work of breathing, with intercostal and subcostal recession and a respiratory rate of 60/min. A chest xray shows a ground glass pattern in both lung fields.He has no audible murmur.He is afebrile. You diagnose respiratory distress syndrome.What is the aetiological factor responsible for respiratory distress syndrome?

78. A 5-year-old girl was admitted to the ward after she presented to her local accident and emergency with diarrhoea. She was passing 7-8 loose, watery stools per day for the last 4 days and had been vomiting for 1day prior to this.There was blood in the stools and this had worried her mother.You ask about foreign travel and her mother reveals they had been in India until 2 weeks ago, staying with family and drinking tap water.She had no vaccines prior to travelling. On examination, she now has abdominal pain, swinging pyrexias, right upper quadrant tenderness but no rebound or guarding.You notice a pale pink (rose) spot on her trunk. What is the most likely infecting organism?

79. A 3-month-old baby is brought to accident and emergency because he has been vomiting and having diarrhoea for the past month.His mother breastfed him until he was 8 weeks old and he is now taking formula milk, 4-5 oz every 4 hours.On exami nation he is alert but fussy and looks thin. He has eczema on his face, neck and torso and the mother says this is new.The abdomen is soft, the genitalia are normal with a significant nappy rash and the anal margin is erythematous. You plot his growth in his red book and find that he was born on the SOth centile and was following that but now he is on the 25th centile for weight.What is the most likely diagnosis?

80. A 15-year-old Asian girl with Down 's syndrome came to accident and emergency with a prolonged fever.She has severe learning difficulties and was difficult to assess.Her parents think she is more unsettled than usual and not eating and drinking properly for the last 3 weeks. She is admitted as you cannot confidently find the source of the infection, but she has no cough, rash, vomiting, diarrhoea or meningism.The next day she complains of a headache and starts to vomit.She has a CT scan which is normal and then a lumbar puncture (LP). White cell count (WCC) 150 x109/L (20 per cent neutrophils), red blood count 0, protein 2 g/L, glucose 1.2 mmol/L (serum glucose 6.0 mmol/L). What is the most likely cause of this meningitis?

81. A couple are referred to a geneticist as they are planning on having their first child. There is a history of Wiskott-Ald rich syndrome on the woman 's side.The woman 's father and great grandfather have the condition (eczema, thrombocytopenia, recurrent infection) but she is unaffected. There is no history of the condition in the man's family. What is the risk of having the condition if the child is a boy or a girl respectively?

82. A 4-year-old boy with severe ezcema is brought to accident and emergency by his mother.His skin has been worse recently since the weather ha.s become colder.He is scratching a lot more and now is very miserable and has a temperature of 38.6°C today.On examination of his skin he has multiple areas of erythematous, excoriated lesions on his elbow and knee flexures as well as his trunk and back. In addition they are hot, tender and slightly swollen with areas of broken skin. There are also some yellow fluid-filled vesicles on some of these lesions.You send some blood tests and commence him on IV flucloxacillin and aciclovir.Which are the two most likely organisms that can complicate eczema?

83. A 3-year-old boy has been admitted to hospital with a right-sided pneumonia and pleural effusion.The pleural fluid grew Gram-positive cocci.He is on IV ceftriaxone, oral azithromycin and has a chest drain in situ. On further questioning of Richard 's mother, you establish that he has had multiple chest infections since he was born (in the UK).He has been admitted three times before and also had a sinus wash out following an episode of sinusitis.He has no cardiac anomalies or dysmorphism.His mother also tells you about his older brother, who sadly died of menin gitis aged 6 years old.He too had 'more than his fair share of infections'. The two brothers had different fathers but his mother is IDV negative.What is the most likely underlying immunodeficiency in this family?

84. You are in immunology clinic and the first patient is a 2-year-old boy who has a complement deficiency. You know this involves a cascade of proteins involved with innate immunity but are unsure about the manifestations in children.The professor of immunology asks you which organism is this child at risk of being infected with.He gives you a clue by telling you the child has a late complement deficiency, meaning C5-C9.What is the most likely causative organism that infects these children?

85. A 3-year-old boy is admitted to the children 's ward. He has been isolated in a cubicle as he is at risk of infections.He is awaiting a bone marrow transplant and has a brother with the same condition. His mother tells you they both have SCID.What are the likely immune function test results in SCID?

86. A 3-week-old baby attends accident and emergency with bloody diarrhoea.Mum says he has been having diarrhoea for the past 2 days since she started using formula milk.He was previously breastfed and mum was not having any dairy products due to lactose intolerance.He also has eczema on his cheeks and a strong family history or asthma and eczema. Mum is concerned that he may be allergic to milk too.What is the most likely diagnosis?

87. A 2-year-old child is brought to cardiology clinic due to a heart murmur heard by the GP after an examination when she was recently unwell.She was born at 40 weeks by normal vaginal delivery but was noted to have a cleft palate at birth. She was kept in hospital for establishment of feeding but during this time she had a seizure, noted later to be because her calcium was low. You bear a harsh, grade 3/6 pansystolic murmur, loudest at the left lower sternal edge, consistent with a ventral septal defect (VSD) as seen on echocardiogram.With this history and current examination fmding, you wish to exclu de DiGeorge's syndrome. What is the best diagnostic test?

88. A 15-year-old boy attends his GP with a week of cough productive of yellow sputum, fever to 39°C and chest pain on the right side of the chest on coughing.There is no history of foreign travel or unwell contacts. On examination there is reduced air entry in the right lower zone with crepitations and bronchial breathing.You diagnose a right-sided chest infection. What is the most likely causative organism?

89. A 2-year-old boy is admitted to the paediatric ward with a swollen, painful left knee.He has been afebrile and has a history of minor trauma to his knee earlier today. His mother is a haemophilia carrier and his father is not affected.You are keen to rule out haemophilia in this child. Which two clotting factors should you test for?