DES 2016. Final (Part 53)

211) A 3-week-old male infant is brought to the office for the evaluation of red eyes. His temperature is 37.2 C (99F), pulse is 100/min, and respirations are 34/min. On examination, the infant has conjunctival congestion and scant mucoid discharge. The rest of the physical examination is normal. What is the most appropriate next step in the management of this patient?

212) A 6-month-old infant is brought to the clinic for the evaluation of sudden episodes of jerky movements of the neck, arms, and legs onto the trunk for past month. These episodes occur in clusters, last for a few minutes, and are often preceded by a cry. Her development has been normal. Her vital signs are normal. She is at 50th percentile for height, weight and head circumference. She has multiple small 1-2 cm oval irregular hypopigmented macules on her trunk and extremities. A head CT scan reveals cortical tubers in the cerebral cortex and multiple subependymal nodules in the lateral ventricles. Her EEG shows "hypsarrhythmia." What is the best medication for this patient's seizure?

213) A 9-year-old male is brought to the emergency department with respiratory disease after being stung by a bee. He requires resuscitation with epinephrine injection. On discharge, which of the following recommendations to the boy's mother is most appropriate?

214) A 7 -year-old Caucasian boy is brought to the clinic due to malaise and headache for the past 2 weeks. He was apparently normal until 3 weeks ago, when he went on a hiking trip with his family to Connecticut. His father found a tick attached to his thigh; however, a red macule remains at the site where the tick was attached. One week after the tick was removed, a red ring developed around the macule, and then the ring appeared to grow larger by expanding outward, leaving an area of central clearing. What is the most appropriate next step in the management of this patient?

215) You have an 11-day-old term infant in your office for a well-child visit. The mother notes that she received a letter that day from the state’s Department of Health reporting that her child’s newborn screen had come back abnormal, indicating possible galactosemia. Which of the following is the most appropriate management at this point?

216) A 9-year-old boy is being evaluated for a syncopal episode. He was playing basketball when the syncope occurred. The episode lasted about 1-2 minutes, and then the patient woke up on his own. His mother also noticed that he has been having hearing problems. His physical examination is unremarkable. An ECG shows a prolonged Q-T interval, with a QTc of 0.49 sec. Which of the following is the most appropriate management if he has long Q-T syndrome?

217) A 9-year-old girl is seen in the office for unsteady gait and weakness in the lower limbs. On examination she is found to have a wide-based gait with constant shifting of position to maintain her balance. There is decreased vibratory and position sense in the lower extremities, and ankle jerks are absent bilaterally. Her feet are deformed with high plantar arches. MRI of the brain and spinal cord shows marked atrophy of the cervical spinal cord and minimal cerebellar atrophy. Nerve conduction velocity results are within normal limits. EKG shows T-wave inversions in the inferior and lateral chest leads. The mother says that her other child, a 3-year-old boy, is completely normal. He shares toys with his sister and plays well with other children at playschool. He is able to copy squares and crosses but is unable to copy a triangle. Which of the following statements would you advance while discussing the girl's condition with her mother?

218) An 8-year-old African American boy is brought in for evaluation of a mass on the scalp. On examination, he is afebrile and nontoxic. There is a boggy mass on his scalp with alopecia. His posterior cervical lymph nodes are enlarged but nontender. Which of the following is the most appropriate treatment?

219) The parents of a 14-year-old boy are concerned about his short stature and lack of sexual development. By history, you learn that his birth weight and length were 3 kg and 50 cm, respectively, and that he had a normal growth pattern, although he was always shorter than children his age. The physical examination is normal and his growth curve is shown on the next page. His upper-to-lower segment ratio is 0.98. A small amount of fine axillary and pubic hair is present. There is no scrotal pigmentation; his testes measure 4.0 cm3 and his penis is 6 cm in length. In this situation, which of the following is the most appropriate course of action?

220) A 6-year-old boy has multiple, honey-colored, crusted lesions on his face, periungual areas, and forearms. The first lesion appeared 2 weeks ago on his philtrum. Since then the lesions have spread to his hands and arms. Each began as a small pustule on an erythematous base and eventually ruptured to form the crusted lesions now present. His temperature is 100.6 F, pulse is 100/min, and respirations are 14/min. The remainder of the physical examination is unremarkable. Which of the following is the most appropriate treatment?

221) An 11-month-old African American boy has a hematocrit of 24% on a screening laboratory done at his well-child checkup. Further testing demonstrates: hemoglobin 7.8 g/dL; hematocrit 22.9%; leukocyte count 12,200/μL with 39% neutrophils, 6% bands, 55% lymphocytes; hypochromia on smear; free erythrocyte protoporphyrin (FEP) 114 μg/dL; lead level 6 μg/dL whole blood; platelet count 175,000/μL; reticulocyte count 0.2%; sickle-cell preparation negative; stool guaiac-negative; and mean corpuscular volume (MCV) 64 fL. Which of the following is the most appropriate recommendation?

222) A 7-year-old girl presents with hives, which developed after a bee sting. She has no other symptoms. The hives resolve with diphenhydramine. Which of the following is the most appropriate management?

225) A 9-month-old infant is seen in the pediatrician's office because of failure to gain weight. Her length and weight are both below the 5th percentile at her age. The patient's chart indicates that at the age of age of 6 months, her length and weight were at the 50th percentile. A careful history reveals that the mother returned to work when the infant was 6 months old, and the grandmother assumed the care of the infant most of the time since then. She is receiving 6-8 oz of iron-fortified, cow protein-based formula every 4 hours. Which of the following is the best initial step in the management of this infant?

226) A 5-year-old boy is brought to the physician's office because of a cough. He has episodes of successive and rapid coughs, which seems like he is choking, followed by a deep and loud inspiratory sound. He has vomited a few times after these bursts of cough. His symptoms have been progressively worsening for the past week, and were preceded by congestion, serous rhinorrhea and sneezing. His past medical history is insignificant. His vital signs are stable. Lung auscultation is unremarkable. Which of the following is the most appropriate pharmacotherapy for this patient?

227) A 2-week-old baby girl is brought to the clinic for the evaluation of vaginal discharge. Her mother has noticed a cloudy white vaginal discharge mixed with blood since yesterday. She was born by normal vaginal delivery at term, and no congenital anomalies were noted at birth. Physical examination reveals a blood-stained, odorless vaginal discharge. Her vital signs are stable. What is the most appropriate next step in the management of this patient?

2) A 3-year-old girl is brought to the emergency department because she became partially unconscious, lethargic and febrile. Her mother claims that "she suddenly took a turn for the worse" 24 hours ago after an upper respiratory tract infection. She has no allergies, and was not taking oral antibiotics. Her blood pressure is 60/45mm Hg and pulse is 120/min. On examination, she appears emaciated and lethargic. Despite numerous attempts, it is impossible to start a peripheral intravenous line, and the child's condition continues to deteriorate. What is the best next step to obtain IV access in this patient?

3) An infant who appears to be of normal size is noted to be lethargic and somewhat limp after birth. The mother is 28 years old, and this is her fourth delivery. The pregnancy was uncomplicated, with normal fetal monitoring prior to delivery. Labor was rapid, with local anesthesia and intravenous meperidine (Demerol) administered for maternal pain control. Which of the following therapeutic maneuvers is likely to improve this infant’s condition most rapidly?

4) At 43 weeks’ gestation, a long, thin infant is delivered. The infant is apneic, limp, pale, and covered with “pea soup” amniotic fluid. Which of the following is the best first step in the resuscitation of this infant at delivery?

5) A 15-year-old is participating in high school football practice in August in Texas. He had complained of headache and nausea earlier in practice, but kept playing after a cup of water. He is now confused and combative. He is dizzy and sweating profusely. His temperature is 41°C (105.8°F). Therapy should consist of which of the following?

6) A fully immunized 2-year-old presents to the emergency room with several days of low-grade fever, barking cough, and noisy breathing. Over the past few hours he has developed a fever of 40°C (104°F) and looks toxic. He has inspiratory and expiratory stridor. The family has not noticed drooling, and he seems to be drinking without pain. Direct laryngoscopy reveals a normal epiglottis. The management of this disease process includes which of the following?

7) A 2-year-old boy presents to the emergency department (ED) with severe wheezing. His symptoms have not improved even after three treatments with nebulizers. This is his third trip to the ED in the past two weeks. In the ED, he is given a dose of intravenous steroids, but continues to wheeze. On auscultation, there is no air entry bilaterally. His chest x-ray result is normal. His oxygen saturation is 91% on 6 liters of oxygen. What is the next best step in the management of this patient?

8) A 7 -year-old Caucasian male with a history of peanut allergy develops sudden shortness of breath after lunch at school. On physical examination, his blood pressure is 70/50 mmHg and his heart rate is 130/min. Inspirations and expirations are labored and noisy. Which of the following is the best initial treatment?

9) You admitted to the hospital the previous evening a 1-year-old boy who presented with cough, fever, and mild hypoxia. At the time of his admission, he had evidence of a right upper lobe consolidation on his chest radiograph. A blood culture has become positive in less than 24 hours for Staphylococcus aureus. Approximately 20 hours into his hospitalization, the nurse calls you because the child has acutely worsened over the previous few minutes, with markedly increased work in breathing, increasing oxy- gen requirement, and hypotension. As you move swiftly to the child’s hospital room, you tell the nurse to order which of the following?

10) A previously heathy 3-year-old boy is brought to the emergency department (ED) due to stridor of sudden onset. Last night, he suddenly developed a high fever followed by breathing difficulty. His temperature is 40C (104F), pulse is 130/min, and respirations are 40/min. In the ED, the child is toxic-appearing, sitting up, leaning forward and drooling. His lungs are clear, and oxygen saturation is 85% in room air. What is the most appropriate next step in management?

11) An 18-month-old infant is found with the contents of a bottle of drain cleaner in his mouth. Which of the following treatment options is most appropriate?

12) A 2 week post mature baby is born and immediately exhibits severe respiratory distress. Previously, green-tinged meconium was noted in the amniotic fluid. Which of the following is the most appropriate next step?

13) A 2-year-old arrives in the emergency center after having swallowed a button battery from one of her toys. She is breathing comfortably, without stridor. Radiographs show the battery to be lodged in the esophagus. Which of the following is the correct next step?

14) A 9-month-old infant is brought to the emergency department with lethargy and tachypnea. He was healthy before developing fever and diarrhea four days ago. He has been taking some formula, but has had two to three episodes of diarrhea with each bottle. He has lost three pounds (1 .4 kg) since his routine check-up two weeks ago. He has had one wet diaper in the past twenty four hours. On examination, his temperature is 102.5F (39.1c), pulse is 200/min, respiratory rate is 42/min, and blood pressure is 70/45 mm Hg. He is lethargic with decreased tone and decreased deep tendon reflexes. His mucous membranes are dry. Cardiopulmonary exam reveals tachycardia and tachypnea. His abdominal exam is unremarkable. Capillary refill is four seconds. Laboratory results are shown below. Chemistry panel: Serum sodium 165 mEq/L, Serum potassium 4.5 mEq/L, Chloride 108 mEq/L, Bicarbonate 14 mEq/L, Blood urea nitrogen (BUN) 20 mg/dl, Serum creatinine 0.8 mg/dl, Calcium 10.0 mg/dl, Blood glucose 98 mg/dl. Which of the following fluids should be used as a bolus in the resuscitation of this infant?

15) A 5-year-old boy is brought into the ER immediately after an unfortunate altercation with a neighbor’s immunized Chihuahua that occurred while the child was attempting to dress the dog as a superhero. The fully immunized child has a small, irregular, superficial laceration on his right forearm that has stopped bleeding. His neuromuscular examination is completely normal, and his perfusion is intact. Management should include which of the following?

16) Aunt Mary is helping her family move to a new apartment. During the confusion, 3-year-old Jimmy is noted to become lethargic. The contents of Aunt Mary’s purse are strewn about on the floor. In the ER, the lethargic Jimmy is found to have miosis, bradycardia, and hypotension. He develops apnea, respiratory depression, and has to be intubated. His condition would most likely benefit from which of the following therapies?

17) An infant is born at terrn to a 27-year-old Caucasian female. The prenatal course was uncomplicated. The amniotic fluid is clear. Immediately after the delivery, the infant starts crying and is moving all four extremities actively. Heart rate is 140/min. He is making a grimace on the attempt to put the suction catheter into his nostrils. His body is pink, but extremities are cyanotic. Which of the following is the next best step in the management of the infant?

18) A 15-year-old boy with Duchenne muscular dystrophy is brought to the emergency department with increasing respiratory distress and cyanosis. On examination, he is diaphoretic, with gasping respirations, poor air entry, and diminished responsiveness. He is tachycardic at 160 beats/min. His chest x-ray film shows a lingular pneumonia, and he is intubated. He improves over the next 10 days with antibiotics but is not extubated secondary to hypoventilation on weaning because of muscle weakness. Which of the following modalities will most likely help wean him off the ventilator?

19) A 6-year-old Hispanic boy is brought to your office by his mother because of severe pain in his right hip and refusal to walk for the last 2 days. His blood pressure is 100/70 mm Hg, pulse is 90/min, respirations are 18/min, and temperature is 38.7C (102.7F). His WBC count is 19,000/mm3 and ESR is 55 mm at one hour. On examination, he keeps his right hip externally rotated, and cries out in pain on any movement involving the right hip. Joint aspiration reveals a turbid fluid with total WBC: 90,000/mm3 and numerous bacteria. What is the most appropriate management in this patient?

20) A newborn infant becomes markedly jaundiced on the second day of life, and a faint petechial eruption, first noted at birth, is now a generalized purpuric rash. Hematologic studies for hemolytic diseases are negative. Acute management should include which of the following?

21) A 14-year-old girl, angry at her mother for taking away her MP3 player, takes an unknown quantity of a friend’s pills. Within the first hour she is sleepy, but in the emergency center she develops a widened QRS complex on her electrocardiogram (ECG), hypotension, and right bundle branch block. The therapy you would initiate for this ingestion is which of the following?

22) You are called to a delivery of a woman with no prenatal care; she is in active labor but has no history of amniotic rupture. The biophysical profile done in the emergency center revealed severe oligohydramnios. When you get this infant to the nursery, you should carefully evaluate him for which of the following?

23) Parents bring a 5-day-old infant to your office. The mother is O negative and was Coombs positive at delivery. The term child weighed 3055 g (6 lb, 1 oz) at birth and had measured baseline hemoglobin of 16 g/dL and a total serum bilirubin of 3 mg/dL. He passed a black tarlike stool within the first 24 hours of life. He was discharged at 30 hours of life with a stable axillary temperature of 36.5°C (97.7°F). Today the infant’s weight is 3000 g, his axillary temperature is 35°C (95°F), and he is jaundiced to the chest. Parents report frequent yellow, seedy stool. You redraw labs and find his hemoglobin is now 14 g/dL, and his total serum bilirubin is 13 mg/dL. The change in which of the following parameters is of most concern?

24) A 20-month-old child is brought to the ED because of fever and irritability and refusal to move his right lower extremity. Physical examination reveals a swollen and tender right knee that resists passive motion. Which of the following is the most likely to yield the diagnosis in this patient?

25) A 12-year-old boy presents to his pediatrician with frequent episodes of headache, nausea, blurry vision, and sweating. On physical examination, his temperature is 37.4 C (99.3 F), blood pressure is 148/94 mm Hg, pulse is 92/min, and respirations are 18/min. The rest of his examination is unremarkable. His 24-hour urinary vanillylmandelic acid (VMA) and metanephrines are increased. An abdominal CT reveals an extrarenal mass above the left kidney. Which of the following is the most appropriate pharmacotherapy?

26) A 12-year-old girl with mild asthma comes to the office for a health maintenance visit. Her mother states that she is using her albuterol inhaler 2-3 times a week and that she has a cough that wakes her up at night about 3 times a month. On physical examination, she has diffuse inspiratory and expiratory wheezes. She has no accessory muscle use. Pulse oximetry shows 95% oxygen saturation on room air. Which of the following is the most appropriate treatment for her at this time?

27) A 2-week postmature neonate exhibits severe respiratory distress immediately after birth. Previously, greentinged meconium was noted in the amniotic fluid. Which of the following is the most appropriate next step in management?

28) A 3-month-old, previously well male infant presents to the emergency department in January with a 2-day history of clear rhinorrhea, low-grade fever, and poor appetite, but no cough. On physical examination, there are mild subcostal retractions, coarse breath sounds heard throughout the lung fields, and scattered expiratory wheezes. The child receives an intravenous fluid bolus in the emergency department and is admitted for observation. Which of the following is the most severe, life-threatening complication of this child's illness?

29) A father brings his 18-month-old daughter to the office for the evaluation of fever for the past 10 days. The father, who works as a phlebotomist, says that the temperature seems higher in the morning and again at night, often reaching 40.5C (105F), and is sometimes associated with shaking chills. He remembers that the child developed severe respiratory distress and skin rash when treated with penicillin in the past. He has heard about the development of 'liver toxicities' in children treated with aspirin, and he is interested in knowing everything about his daughter's condition. On examination, the child is in no acute distress. Her temperature is 39.4C (103F), pulse rate is 90/min, and blood pressure is 100/60 mmHg. Her sclerae are anicteric. There is bulbar conjunctival injection, and the oropharynx shows diffuse mucosal injection with fissuring and crusting of the lips. There are numerous hyperemic macules over her entire body, and her palms appear edematous. Based on the above description, which of the following is the most accurate statement?

31) A 6-month-old boy presents to the Emergency Department with a three-day history of cough, congestion and low grade fever. The mother states that the baby has not been feeding well and has used only two diapers over the past 24 hours. Physical examination reveals a pale infant with a temperature of 37.8 C (100.1 F), pulse of 170/min, respirations of 60/min and oxygen saturation of 88% on room air. The patient exhibits nasal flaring, subcostal and intercostal retractions. Lung examination reveals diffuse wheezing. Cardiac examination reveals a regular but tachycardic rhythm with no murmur. Central capillary refill is four seconds. The remainder of the examination is normal. Which of the following is the most appropriate initial step in management?

33) A 15-year-old Caucasian male is brought to the office by his mother for the evaluation of a six-month history of unstable gait and speech difficulty which are getting worse over time. His past medical history is insignificant. He is not taking any medications, and denies smoking or alcohol consumption. His blood pressure is 120/70 mmHg and pulse is 80/min. Musculoskeletal examination showed scoliosis and feet deformity with 'hammer toes.' The neurologic examination showed dysarthria, dysmetria, nystagmus, and absence of deep plantar reflexes on lower extremities. What is the most common cause of death in this patient population?

Subsequently diagnosed with otitis media and treated with oral amoxicillin. Today, in the ED, his temperature is 40.0C(104F), pulse is 80/min, respirations are 40/min and irregular, blood pressure is 100/60 mm Hg, and weight is 8kgs ( 15 lbs). He is lethargic and arousable only to painful stimuli. His anterior fontanel is full and tense. His tympanic membranes are red and bulging. His pupils are reactive, but his eyes do not focus well on his parents. What is the most appropriate next step in the management of this patient?

35) A 17-year-old girl is found unconscious in a bathroom after ingesting a large amount of unknown sleeping pills. On arriving at the emergency department, she is somnolent; her temperature is 37.2 C (98.9 F), blood pressure is 120/74 mm Hg, pulse is 68/min, and respirations are 12/min. She responds to painful stimuli and to hearing her name loudly by opening her eyes briefly. However, she returns to the somnolent state immediately afterward. Which of the following is the most appropriate initial intervention?

37) A 6-year-old child, attempting to pet a neighbor's domestic dog while the dog is eating, is bitten in the hand. The dog has been vaccinated regularly. Which of the following steps are needed for rabies prophylaxis in this case?

1) The parents of a 3-year-old patient followed in your clinic recently took their child on quickly planned 5-day trip to Africa to visit an ill grandparent. Everyone did well on the trip, but since their return about 10 days ago the boy has been having intermittent, spiking fevers associated with headache, sweating, and nausea. The parents had not been too concerned since he was relatively well, except for being tired, between the fevers. Today, however, they feel that he looks a bit pale and his eyes appear “yellow.” Which of the following is likely to reveal the source of his problem?

2) An 11-month-old girl is brought by her mother to the office due to fever, fussiness, and irritability. The girl has a tendency to hold and pull on her left ear. For the past few days, she has had rhinorrhea and nasal congestion. Her medical history reveals nothing particular. Her temperature is 38.9C (102F). Otoscopic examination reveals a bulging, erythematous tympanic membrane with decreased mobility on air compression. What is the most likely organism responsible for the patient's condition?

3) A 1-day-old normal-appearing infant develops tetany and convulsions. He was born at 34 weeks’ gestation with Apgar scores of 2 and 4 (at 1 and 5 minutes, respectively) to a woman whose pregnancy was complicated by diabetes mellitus and pregnancy-induced hypertension. Which of the following serum chemistry values is likely to be the explanation for his condition?

4) A neonate is noted to have aniridia of the right eye on physical examination. He was born by spontaneous vaginal delivery after an uncomplicated full-term pregnancy. The remainder of the physical examination is normal. Which of the following is the most appropriate next step before the infant is released from the hospital?

5) An 11-month-old boy is brought to the emergency department by his parents. The child has a fracture of the right femur. The father reports this was sustained as a result of falling out of the crib. The child is also noted to have bruises on his shoulders and back. The rest of his examination is unremarkable. Which of the following is the most appropriate next step in diagnosis?

6) A 7-year-old boy is brought to the clinic for a lifetime history of bedwetting. He has otherwise been completely healthy and has met all development milestones. His parents deny a history of trauma, and the history is not consistent with abuse. The patient has been wetting every night but not during the daytime. He has no incontinence. Which of the following is the most appropriate next step in his evaluation?

7) A 4-year-old child has mental retardation, shortness of stature, brachydactyly (especially of the fourth and fifth digits), and obesity with round facies and short neck. The child is followed by an ophthalmologist for subcapsular cataracts, and has previously been noted to have cutaneous and subcutaneous calcifications, as well as perivascular calcifications of the basal ganglia. This patient is most likely to have which of the following features?

8) A 30-hour-old infant has not passed meconium since birth. He was full term with a birth weight of 3856 g (8 lb 8 oz). The pregnancy was uncomplicated. The baby appears well with no respiratory distress. Slight abdominal distention is noted. Rectal examination reveals a slightly tight rectum and results in a greenish gush of stool. Which of the following tests will probably confirm the likely diagnosis?

10) A 10-year-old child arrives with the complaint of new-onset bed-wetting. He has had no fever, his urine culture is negative, and he has had no new stresses in his life. He is well above the 95th percentile for weight as is much of his family. Which of the following is most helpful in making a diagnosis?

11) A 2-year-old child is brought to the physician for a routine visit. He is growing and developing appropriately. He drinks 3-4 glasses of whole milk each day. He is starting to put words together into short sentences. His mother has no concerns. Physical examination shows mild pallor. Laboratory studies show the following: Hemoglobin 9.5 g/dL, RDW 21%, MCV 70 fl, Platelet count 284,000/mm3, Leukocyte count 6,500/mm3. Which of the following additional findings is most likely in this patient?

12) A 2-month-old infant is brought to the emergency department with irritability and lethargy. The parents state that he was well until he rolled off the couch on to the floor yesterday. On examination, he is inconsolable and afebrile. The fontanels are full and tense. He has a generalized tonic-clonic seizure. Which of the following is the most important initial diagnostic study to order?

13) A 6-month-old male infant is brought to the physician's office by his mother due to failure to thrive. According to the mother, the child always regurgitates shortly after feedings, and has even had a few episodes of blood-stained projectile vomiting. During the vomiting episodes, the child tilts his head to the left and arches his back. On examination, the child is afebrile. His abdomen is soft and non-tender to palpation. Laboratory evaluation shows hypochromic microcytic anemia. What is the most appropriate test to confirm the most likely diagnosis of this child?

14) Which of the following is the most appropriate evaluative procedure for an otherwise normal 7-day-old boy with perineal hypospadias?

15) A 10-year-old boy has a long history of recurrent infections. These have included pneumonia, suppurative lymphadenitis, persistent rhinitis, dermatitis, diarrhea, and perianal abscesses. Involved organisms have included Staphylococcus aureus, Serratia, Escherichia coli, and Pseudomonas. Biopsy of skin and lymph nodes have demonstrated granulomatous lesions, even though the only species isolated were those noted above. Immunoglobulin levels are higher than normal. Which of the following findings would be most helpful in establishing the diagnosis?

16) A previously healthy 15-month-old girl is brought to the emergency department after she had several episodes in which she lost consciousness after crying. Her parents state that the episodes always occur when the child gets frustrated or upset. She has no cyanosis or incontinence. The episodes last about 45 seconds each. After the episode, she is alert and appropriate. Physical examination is unremarkable. What is the next best step in the management of this patient?

17) A male infant born at term is found to have bilateral colobomas, choanal atresia, ear anomalies, and cryptorchidism. There is no history maternal drug or alcohol abuse during pregnancy. There is no family history of similar congenital defects. Which of the following is the most appropriate initial test to exclude any associated abnormalities?

18) A 10-year-old patient with sickle cell disease comes to the physician for a routine visit. The patient has a history of multiple pain crises and pneumonias. He was started on hydroxyurea one year ago and has not had any further pain crises since then. He has had no recent illnesses or hospitalizations. His physical examination is unremarkable. The patient's laboratory results are shown below. Complete blood count: Hemoglobin 9.0 g/L, Erythrocyte count 2.2 mln/mm3, MCHC 32%, MCV 105 fl, Reticulocytes 2.0%, Platelets 212,000/mm3, Leukocyte count 9500/mm3, Neutrophils 56%, Eosinophils 3%, Lymphocytes 36%, Monocytes 5%. Which of the following best describes the role of hydroxyurea in the treatment of patients with sickle cell disease?

19) A 3-year-old boy is brought to the ER with a two-day history of decreased appetite, neck swelling, and irritability. He keeps his head rotated slightly to the right side. He resists passive flexion of the neck and rotation to the left side. Which of the following is the best next step in managing this patient?

20) The parents of a 2-month-old baby boy are concerned about his risk of coronary artery disease because of the recent death of his 40-year-old maternal uncle from a myocardial infarction. Which of the following is the most appropriate management in this situation?

21) A 5-month-old infant is brought to the physician for a routine visit. The child was born full term without complications. The infant has been seen several times for poor weight gain. The infant takes 5 ounces of formula every 4 hours, and has been tried on increased calorie formula without success. Family history is significant only for nephrolithiasis. On examination, the infant's weight is <5%, height is at the 20% and head circumference is at the 20%. The infant appears thin, but the remainder of the physical examination is unremarkable. Screening labs are shown below. Chemistry panel: Serum sodium 140 mEq/L, Serum potassium 3.0 mEq/L, Chloride 113 mEq/L, Bicarbonate 15 mEq/L, Blood urea nitrogen (BUN) 10 mg/dl, Serum creatinine 0.5 mg/dl, Calcium 9.0 mg/dl, Blood glucose 98 mg/dl. The urine pH is 7.9 and urinary sodium and potassium are within normal limits. What is the most likely cause for this patient's failure to thrive?

22) A 6-year-old boy has had a fever for 8 days. He just finished a 5-day course of amoxicillin for otitis media. On examination, his temperature is 38.6 C (101.4 F). He has meningismus and palsy of the left sixth cranial nerve. Cerebrospinal fluid (CSF) analysis reveals 200 white cells per mL with 80% lymphocytes and 20% polymorphonuclear leukocytes, glucose of 18 mg/dL, protein of 260 mg/dL, and a negative Gram stain. There is basilar enhancement without focal lesions on CT. Which of the following CSF tests will most likely identify the cause of meningitis?

23) An 18-month-old boy is brought to the clinic for a checkup. As part of his routine care, a serum lead level is obtained. It is 25 g/mL. Which of the following is the most appropriate next step in his management?

24) A 3-month old infant is brought to a pediatrician's office because of increased lethargy and irritability. The parents state that the child rolled off the couch and fell on the floor one day prior to presentation. His parents report that the child has been previously healthy and is up to date on his vaccinations. He has been meeting his development milestones. His fontanelles are full. While in office the patient develops a tonic-clonic seizure. Which of the following is the next appropriate step?

25) A 4-year-old girl was diagnosed of left-side otitis media about 10 days ago and was prescribed oral amoxicillin, 40 mg/kg/day for 7 days. She has since developed bloody diarrhea with mucus, crampy abdominal pain, and fever. On physical examination, her temperature is 39.4 C (102.9 F), pulse is 88/min, and respirations are 16/min. She has normal bowel sounds and is diffusely tender to palpation. Which of the following is the most appropriate initial step in diagnosis?

26) A 4-week-old infant is brought to the office due to several episodes of vomiting over the past week. The episodes have progressively become more frequent and forceful, but the vomitus never contains any blood or bile. The infant has been breastfed since birth. His vital signs are stable, and he is afebrile. An olive-shaped mass is palpated just to the right of the umbilicus, and peristaltic waves are seen in the upper abdomen. Lab studies reveal a sodium level of 135 mEq/L, potassium level of 3.3 mEq/L, chloride level of 92 mEq/L and bicarbonate level of 30 mEq/L. Which of the following is the most appropriate diagnostic test for this patient?

27) A 5-year-old boy presents to the emergency department with 2-day history of fever, anorexia, loose stools, and yellow skin color. He attends a large daycare center. On physical examination, his temperature is 38.1 C (100.7 F), blood pressure is 88/56 mm Hg, pulse is 74/min, and respirations are 15/min. Initial laboratory evaluation reveals a total bilirubin of 1.8 mg/dL and alanine aminotransferase of 764 U/L. Which of the following is the most appropriate diagnostic test?

28) A 6-year-old child is hospitalized for observation because of a short period of unconsciousness after a fall from a playground swing. He has developed unilateral pupillary dilatation, focal seizures, recurrence of depressed consciousness, and hemiplegia. Which of the following is the most appropriate management at this time?

29) A 2-year-old boy has been doing well despite his diagnosis of tetralogy of Fallot. He presented to an outside ER a few days ago with a complaint of an acute febrile illness for which he was started on a “pink antibiotic.” His mother reports that for the past 12 hours or so he has had a headache and is more lethargic than normal. On your examination he seems to have a severe headache, nystagmus, and ataxia. Which of the following would be the most appropriate first test to order?

30) In an adolescent presenting with pityriasis rosea, which of the following would be an appropriate blood test to order?

31) A 2-year-old boy with cough and difficulty breathing is brought to the emergency department by his mother. She says that he was well and playing with his toys until 2 hours prior to presentation. He is healthy, but his 6-year old brother has a peanut allergy. The patient's temperature is 36.7 C (98 F), blood pressure is 92/48 mmHg, pulse is 114/min, and respirations are 48/min. The patient's pulse oximetry shows 91 % on room air. Physical examination shows nasal flaring and grunting with both subcostal and intercostal retractions. Wheezing is heard in the right lung field; the left field is clear to auscultation. No rales or rhonchi are noted. The remainder of the physical examination is within normal limits. Supplementary oxygen is applied. Chest x-ray reveals mild hyperinflation of the right lung. Which of the following is the most appropriate next step in management of this child?

32) An 8-year-old boy is brought to the emergency department by his mother. Two weeks ago, he developed a low-grade fever followed by a persistent cough. He occasionally has severe paroxysms of cough that are precipitated by eating and do not resolve with antitussive medications. On physical examination, extensive subcutaneous emphysema over the anterior chest is noted. What is the most appropriate next step in the management of this patient?

33) During a routine yearly checkup, a 10-year-old boy is found to have 2+ proteinuria on urinalysis. Which of the following would be the most appropriate diagnostic test?

34) An infant has had repeated pneumonias and middle ear infections that began at about 5 months of age. At 1 year of age, serum electrophoresis demonstrated hypogammaglobulinemia. T cell function was normal. By 2 years of age, the child's infection rate has decreased, and repeat serum electrophoresis is normal. Which of the following immunoglobulins was likely decreased in this child during the period of increased susceptibility to infection?

35) A 12-year-old, previously healthy girl presents to her physician with a chief complaint of early morning headaches. She states that these headaches wake her up from sleep 2-3 days a week. She also complains of some vomiting associated with the headaches. The headaches have been getting progressively worse for the past 2 months. She denies any photophobia, dizziness, or blurred vision. There is no history of a recent respiratory infection, runny nose, or cough. There is no history of recent trauma. In the office, her vital signs are within normal limits. Her examination shows pupils that are equal, round, and reactive, with no maxillary or frontal sinus tenderness. Her tympanic membranes are clear and intact. Her neck is supple with full range of motion. Neurologic examination shows a positive Romberg sign. Which of the following tests would most likely confirm the diagnosis?

36) A 4-year-old child manifests symptoms of fever, sore throat, and swollen lymph nodes. The spleen tip is palpable. Throat culture and rapid slide (Monospot) test results are negative. The next logical diagnostic procedure would involve which of the following?

37) A 17-year-old adolescent female is 6 weeks postpartum. She presents to the emergency room with the complaints of increased jaundice, abdominal pain, nausea, vomiting, and fever. Her examination is remarkable for jaundice, pain of the right upper quadrant with guarding, and a clear chest. Chest radiographs appear normal. Which of the following tests is most likely to reveal the cause of this pain?

38) An 8-year-old is accidentally hit in the abdomen by a baseball bat. After several minutes of discomfort, he seems to be fine. Over the ensuing 24 hours, however, he develops a fever, abdominal pain radiating to the back, and persistent vomiting. On examination, the child appears quite uncomfortable. The abdomen is tender, with decreased bowel sounds throughout, but especially painful in the midepigastric region with guarding. Which of the following tests is most likely to confirm the diagnosis?

39) A 10-month-old baby boy, recently adopted from Guyana, has a 5-hour history of crying, with intermittent drawing up of his knees to his chest. On the way to the emergency room he passes a loose, bloody stool. He has had no vomiting and has refused his bottle since the crying began. Physical examination is noteworthy for an irritable infant whose abdomen is very difficult to examine because of constant crying. His temperature is 38.8C (101.8F). The rectal ampulla is empty, but there is some gross blood on the examining finger. Which of the following studies would be most helpful in the immediate management of this patient?

40) A 5-month-old male infant has a urine output of less than 0.1 mL/kg/hr shortly after undergoing major surgery. On examination, he has generalized edema. His blood pressure is 94/48 mm Hg, pulse is 140/min, and respirations are 20/min. His blood urea nitrogen is 38 mg/dL, and serum creatinine is 1.4 mg/dL. Initial urinalysis shows a specific gravity of 1.018 and 2+ protein. Microscopic examination of the urine sample reveals 1 WBC per high-power field (HPF), 18 RBCs per HPF, and 5 granular casts per HPF. His fractional excretion of sodium is 3.2 %. Which of the following is the most appropriate next step in diagnosis?

41) A young Hispanic couple brings their first child to their physician for a well-child examination. The child is a 3-month-old healthy-appearing infant, whose weight is at the 45th percentile of the normal growth curve. During examination, the physician observes an area of blue-black pigmentation over the buttocks. The parents say that it was present from the time of birth. Which of the following is the most appropriate next step in management?

42) A 2-year-old boy is brought to the clinic because of a swelling at the base of his neck on the left side. The family indicates that since he was born, they suspected he had some kind of a mass in his left supraclavicular area and behind the sternomastoid on that same side, but the area felt soft and mushy, was not always evident, and seemed to be painless, so they did nothing about it. Two weeks ago the child had an upper respiratory infection, and within a day or two the mass became larger and quite obvious. On physical examination he indeed has a soft, mushy, ill-defined mass occupying the entire left supraclavicular area and extending into the posterior triangle of the neck. He has no enlarged lymph nodes anywhere, and his spleen and liver are not palpable. Which of the following is the most appropriate next step in the evaluation?

43) A 9-month-old infant is brought to the pediatrician's office because his weight is persistently below the 10th percentile. His mother states that the infant seems to be hungry all the time and usually consumes 8-12 oz of formula every 2-3 hours in addition to some table food. He also has frequent, bulky, and malodorous stools. A malabsorption syndrome is suspected. The results of which of the following tests will most likely be abnormal?

44) A 6-week-old child is being evaluated for a fever of unknown etiology. As part of the laboratory evaluation, a urine specimen was obtained that grew E coli with a colony count of 2000/μL. These findings would be definite evidence of a urinary tract infection if which of the following is true about the sampled urine?

45) An obese Caucasian father brings his 1 0-year-old obese son to your office. The father states that he was recently diagnosed with high cholesterol levels and coronary artery disease. Even though his son is asymptomatic, he is worried about the obesity. Which of the following best explains the next best step in management?

46) A full-term infant is born after a normal pregnancy; delivery, however, is complicated by marginal placental separation. At 12 hours of age, the child, although appearing to be in good health, passes a bloody meconium stool. For determining the cause of the bleeding, which of the following diagnostic procedures should be performed first?