Pedia 2017 2018

A 3-day-old, full-term baby boy is brought into the emergency department because of feeding intolerance and bilious vomiting. X-rays films show multiple dilated loops of small bowel and a "ground glass" appearance in the lower abdomen. The mother has cystic fibrosis. Which of the following diagnostic tests would also have therapeutic value?

A 3-year-old boy is brought to the office by his Caucasian mother because his speech is difficult to understand. He is a very slow learner, is unusually calm, stays to himself, doesn't hug his parents, prefers to play by himself, speaks in mumbles, and repetitively tries to make towers with cubes. He becomes very aggressive if he is stopped from his activities. He was born at term from an uncomplicated pregnancy and delivery, and his physical growth has been normal. His brother used to be aggressive as a child, and was diagnosed with attention deficit hyperactivity disorder. In the office, the patient is sitting quietly and trying to make towers with cubes. He seems to be oblivious of his environment. On calling his name thrice, he turns towards you once and then resumes playing with the cubes. What do you tell his mother?

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?

A one-week-old infant is admitted to the neonatal intensive care unit due to vomiting, abdominal distension, and poor feeding. His vomitus is bile-stained. There is frank blood in his stools. He has been formula- fed since birth. Physical examination shows diminished bowel sounds. Abdominal x-rays reveal dilated loops of bowel with intramural air Laboratory studies show elevated WBCs. What is the most likely diagnosis?

A 2-year-old child is referred to hospital by the GP after his third visit that week; he now has a rash and the GP is worried he has meningitis. He has had a fever for 5 days up to 39.5°C or above every day and is not eating or drinking well. On examination, he has a temperature of 38.5°C, heart rate of 150, and respiratory rate of 30 and is miserable. He has a blanching macular rash on his torso, swollen hands and feet, red eyes, red cracked lips, large tonsils with no pus, and a left-sided 2 cm× 3 cm cervical lymph node which is mobile. There is no photophobia or neck stiffness. His chest is clear with normal heart sounds and his abdomen is soft with a palpable liver edge. You note his BCG scar is inflamed. What is the most likely diagnosis?

A previously healthy 7-year-old girl comes to the office with complaints of episodic abdominal pain over the past several months. The pain is periumbilical and sharp but does not wake her from sleep or interfere with play. She has no fever, joint complaints, or constipation or diarrhea. Growth and development have been normal. The physical examination is within normal limits. Which of the following is the most likely diagnosis?

A previously healthy 7-year-old child suddenly complains of a headache and falls to the floor. When examined in the emergency room (ER), he is lethargic and has a left central facial weakness and left hemiparesis with conjugate ocular deviation to the right. Which of the following is the most likely diagnosis?

A 2-hour-old male neonate has developed worsening cyanosis over the past few minutes. The infant was vaginally delivered (assisted with forceps) at full term and weighed 4.1 kg (9 lb). The Apgar scores were 7 and 8 at 1 and 5 minutes, respectively. Oxygen is administered by mask but does not relieve the cyanosis. Further examination reveals tachypnea, subcostal retractions, a normal first heart sound, a single and loud second heart sound (S2), and no murmur. Which of the following is the most likely cause of the infant's cyanosis?

A 20-year-old female is brought to the Emergency Room by her college roommate who states that the patient vomited all night. The patient complains of a sore throat and says she has not eaten for the last two days. She admits to a "sugar problem" and quit taking her medication because she has not been eating. Examination reveals an ill-appearing woman. Her temperature is 37.9 C (100.2 F), blood pressure is 118/78 mm Hg, pulse is 160/min, and respirations are 30/min. The patient's lips and mucous membranes are dry. There is a fruity odor noted to the patient's breath. The lung and cardiac examination are unremarkable except for mild tachypnea and tachycardia. Laboratory analysis shows: Sodium 130 mEq/L, Potassium 6.1 mEq/L, Chloride 100 mEq/L, Bicarbonate 8 mEq/L, Urea nitrogen 10 mg/dL, Creatinine 1.0 mg/dL, Glucose 680 mg/dL, pH 7.15, pCO2: 30 mm Hg, pO2: 85 mm Hg, Urinalysis is positive for ketones. Which of the following is the most appropriate initial step in management?

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 examination 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 50th centile and was following that but now he is on the 25th centile for weight. What is the most likely diagnosis?

A mentally retarded 10-year-old boy presents with arthritis, nephrolithiasis, and progressive renal failure. Since his first years of life, he manifested peculiar neurologic abnormalities consisting of self-mutilative biting of the lips and fingers, choreoathetosis, and spasticity. Two male relatives on his mother's side presented with a similar condition and died in their teens. Which of the following is the most likely diagnosis?

A 7 -year-old boy is brought to the office by his mother due to nocturnal enuresis. He has been wetting his bed at night for the past two years. He is otherwise healthy and has no psychological or behavioral problems. His mother is desperately asking for your help because she has tried alarms and bladder training, all to no success. What is the most appropriate next step in the management of this patient?

You see an 18-year-old boy who is the first child of his African parents and was born in Kenya before moving to the UK 1 year ago. He has white skin and pink irises. He was diagnosed with oculocutaneous albinism at birth. He has difficulty with his sight but has recently developed a skin lesion on his face. His mother has brought him to his GP as it has recently started to increase in size. On examination you note is an elevated, 3 cm diameter lump on the left of his nose. It has irregular edges, is firm and immobile and pigmented in areas. What is the likely diagnosis?

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?

A 16-year-old boy presents to the emergency center with a 2-day history of an abscess with spreading cellulitis. While in the emergency center, he develops a high fever, hypotension, and vomiting with diarrhea. On examination you note a diffuse erythematous macular rash, injected conjunctiva and oral mucosa, and a strawberry tongue. He is not as alert as when he first arrived. This rapidly progressive symptom constellation is likely caused by which of the following disease processes?

A 5-year-old boy is brought to the physician because of a malodorous discharge coming from his right nostril. His parents report that the child developed frequent sneezing and mucus discharge for one week. They thought it was a common cold. The child is afebrile and in apparent good health. There is no history of allergic diseases or recurrent infections. Examination reveals obstruction of the right nasal fossa with associated purulent discharge from the right nostril. Which of the following is the most likely diagnosis?

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

A 12-year-old boy is brought to the office by his mother due to a two-week history of generalized edema which is gradually progressing. His past medical history is insignificant. He is not taking any medications. His blood pressure is 110/80 mmHg, pulse is 85/min, respirations are 18/min, and temperature is 36.7C (98F). The laboratory findings are as follows: Serum sodium 140 mEq/L, Serum potassium 3.7 mEq/L, Serum albumin 2.1 g/dl, Serum globulin 6.0 g/dl, Serum creatinine 1.0 mg/dl. Urinalysis reveals proteinuria 3+. What is the best next step in the management of this patient?

A 17-year-old boy comes to medical attention because of recurrent sinusitis and pneumonia, and persistent watery diarrhea due to Giardia lamblia. His parents and a sister are in excellent health. Physical examination reveals enlarged lymph nodes in cervical, axillary and inguinal regions. A lymph node biopsy shows hyperplastic follicles with an absence of plasma cells. Laboratory investigations show: Hematocrit44%, Leukocyte count9, 800/mm3, Neutrophils55%, Lymphocytes30%, Monocytes5%, CD4 T-cell count1000 cells/mm3, Proteins, serum6.2 g/dL, Albumin5.0 g/dL, Globulin1.2 g/dL. Additional studies demonstrate severely depressed levels of serum IgG, with slightly below-normal levels of IgM and IgA. Which of the following is the most likely diagnosis?

A 10-year-old boy presents with recurrent mouth ulcers, abdominal pain, distension and frequent episodes of diarrhoea with mucus. He has been losing weight. On examination he is slim and plotting his growth shows a fall in weight from the 50th centile to below the 9th. His abdomen is soft with generalized discomfort on deep palpation but no masses are present. What is the most likely diagnosis?

An 18-month-old boy presented to the GP with a history of eating soil. He had been in the garden this afternoon as his mother put the washing out. She found him eating the soil and took him straight inside. On examination, he is well and alert but has pale conjunctivae. He is not tachycardic or tachypnoeic. His diet consists of predominantly of breast milk. What is the most likely result of his haemoglobin and haematinics?

A term male infant is found to be cyanotic shortly after birth and requires endotracheal intubation. On physical examination, his blood pressure is 68/34 mm Hg (equal in all four extremities), pulse is 180/min, and respirations are 32/min. His precordium is dynamic, has a grade III systolic murmur, and a single S2. Chest radiography shows a normal heart size and increased pulmonary vascular markings. An arterial blood gas on an FiO2 of 100% shows pH 7.34; PaCO2: 47 mm Hg; PaO2: 46 mm Hg. Which of the following diagnoses is most consistent with these findings?

A 7-year-old boy presents with a rash. His mother states that he was well until 3 days ago when he developed fever and malaise. The next day, the rash started as papules on the trunk, which rapidly changed to vesicles. The lesions have spread all over the body. On physical examination, he has no fever and seems well. You note numerous vesicles all over the body, some of which have crusted over. Which of the following is the most likely diagnosis?

A 7-year-old boy is brought to the physician because of recurrent headaches. The child feels nauseated before and during each attack, and derives some relief from lying down in a dark room. Noises, bright light, and fatigue seem to trigger the episodes. The child frequently complains of headaches at school, and his mother has been occasionally compelled to take him home. The mother is worried about the possibility of a serious illness. She reports that the child's father has similar headaches. The child's growth is normal, and a neurologic examination fails to reveal any abnormality. Which of the following is the most likely diagnosis?

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?

A 1-month-old infant is brought to the office for a routine neonatal visit. His prenatal and birth histories are unremarkable. His vital signs are normal. Examination reveals a harsh, loud holosystolic murmur over the left, lower sternal border. Palpation reveals a thrill over the precordial region. There is no cyanosis, and pulmonary auscultation reveals no rales. Chest radiograph reveals a heart of normal size and a slight increase in pulmonary vascularity. EKG is normal. Which of the following is the most appropriate course of action?

You are called to evaluate a male infant at 50 hours of age because he has not voided. He was born at term and has breastfed poorly, but has passed stool. He appears uncomfortable on physical examination, with a large abdomen and seemingly palpable bladder. There is no respiratory distress. The external genitalia are normal, and both testes descended. Of the following, the MOST appropriate initial step in this infant's evaluation is

A mother brings her 4-year-old son to see you. She seems to be very concerned about her child's bedwetting. As you explore the history, the mother tells you that she started his toilet training when he was 2 years old. The child responded to his mother's efforts and slowly was able to reduce the frequency of bedwetting. However, he did not completely succeed and still occasionally wets his bed. She has become so concerned that she demands you to order tests and prescribe some drugs to resolve the problem. What is your best next step in the management of this boy?

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?

Within 8 hours after birth, an infant has "excessive salivation." Physical examination reveals that she has an imperforate anus, with a small fistula to the vagina. A small, soft nasogastric tube is inserted, and the infant is taken to x-ray. The film shows the tube coiled back on itself in the upper chest, and a normal gas pattern in the gastrointestinal tract. There are no apparent abnormalities of the radius or the vertebral bodies. Which of the following is the most appropriate next step in management?

A 5-week-old infant is brought to the clinic for a 4-week history of noisy breathing that has not improved. She has otherwise been healthy except for a current upper respiratory infection for the past 4 days, which according to the parents, has worsened the noisy breathing. On examination, she has inspiratory stridor. The noisy breathing improves when the infant is asleep. Which of the following is the most likely diagnosis?

A 17-year-old teenage girl presents to your office after having problems during sex with her boyfriend. She also states that she has been developing facial hair, which she has constantly been removing. She started her menstruations at the age of 13 and her cycles have always been regular. On examination she is obese but appears a little masculine and has an enlarged clitoris. Initial lab values are as follows: Serum LH 20 IU/L, Serum FSH 10 IU/L, 17 -hydroxyprogesterone 600ng/ml (Normal is 15ng/ml), Serum testosterone Normal, Serum DHEA 4.0ng/ml (Normal is 4.2ng/ml). What is the most likely diagnosis?

A 30 weeks' gestation infant weighing 1,500 g experiences respiratory distress that requires assisted ventilation in the first 4 hours after birth. Some tachypnea with mild subcostal and intercostal retractions is evident on physical examination. Current ventilator parameters are: positive end-expiratory pressure of 4 cm H2O, peak inspiratory pressure of 22 cm H2O, synchronized ventilator rate of 40 breaths/min, and fraction of inspired oxygen of 0.8 to maintain a Pao2 of 60 mm Hg. A chest radiograph reveals low lung volumes, diffuse microatelectasis, and dense ground-glass opacities. You order surfactant for intratracheal administration. Of the following, the MOST expected event in the 4 hours following surfactant administration is

A 10-year-old boy is brought by his parents to the emergency department for the evaluation of headaches, fever, chills, and a rash over his neck, chest, and axillae. The rash appeared today, and for the past two days the child was complaining of a sore throat. He has no history of allergies, and his immunizations are up-to-date. His blood pressure is 112/70 mmHg, pulse is 1 08/min, respirations are 20/min, and temperature is 38.3C (101 F). Examination reveals an erythematous rash with a sandpaper-like texture, and which blanches with pressure. There is submandibular tender lymphadenopathy, and the throat is erythematous with gray-white exudates. What is the most likely diagnosis?

A 15-year-old postmenarcheal girl presents with persistent right lower quadrant discomfort of 6 weeks' duration and a feeling of abdominal fullness. Her last menstrual period was 3 weeks ago. Results of a urine pregnancy test are negative, and urinalysis results are normal. Ultrasonography reveals a well-defined right ovarian cyst filled with multiple echoes. Of the following, the BEST next step for the management of this patient is

An 8-year-old girl is brought to see you, having not opened her bowels in 8 days. She complains of hard painful stools and recurrent abdominal pain for the past 6 months but no vomiting. Her mother thinks that she is avoiding going to the toilet and reports that she has always been a bit irregular opening her bowels, averaging about twice a week. In her past medical history, she passed meconium on day 1 of life and has had no significant medical problems. On examination she is a well-looking, normally grown child. Her abdomen is soft with a palpable indentable mass in the left iliac fossa. The anus is normal, as are her lower limbs. What is the first step in management?

A 3-year-old African-American female is brought to the office due to a swelling in her abdomen. According to her parents, this swelling was first noted when she was 3 months old, and has been increasing in size over the past 6 months. It is more pronounced during crying and coughing. Examination reveals a soft swelling of the umbilical region that is 3 cm in diameter and covered by skin. It can be easily reduced through the fibrous ring at the umbilicus. The child is afebrile and in good health. What is the most likely diagnosis and best course of action for this patient?

A 10-year-old boy is brought to the paediatric outpatient department for a review of his height. He was found to be on the 0.4th centile and his mid-parental height is the 98th centile. He also has widely spaced nipples, wide carrying angle, hypogonadism, pulmonary stenosis and developmental delay. What is the most likely diagnosis?

A 3-year-old-boy ingests 40 of his older sister's chewable vitamin tablets, as well as 3 tablets of 250 mg of acetaminophen. The ingredients in the multivitamin tablets are as follows: Vitamin A 3000 IU, Thiamine 1 mg, Vitamin C 75 mg, Vitamin B6 1 mg, Vitamin D 400 IU, Iron 12 mg, Fluoride 1 mg. The child is brought to the emergency department in no acute distress. Which of the following complications may occur if appropriate therapy is not undertaken?

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 hear 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 finding, you wish to exclude DiGeorge’s syndrome. What is the best diagnostic test?

A 14-year-old boy presents with sudden onset of pain and swelling of his right testicle. There was no history of trauma, he is not sexually active, and denies any history of penile discharge. On examination, the scrotum is swollen and tender. The cremasteric reflex is absent. A testicular flow scan shows a “cold spot” or absent flow to the affected side. Which of the following is the most likely cause?

The mother of a 4-year-old child takes her daughter to a pediatrician because she is "scratching all the time." Physical examination demonstrates multiple areas of excoriation, which are worst on the shoulders, buttocks, and abdomen. In the areas where the scratching has occurred, scattered tiny red punctate lesions are also seen. Careful examination of the clothing reveals small, ovoid, grayish-white structures attached to threads on the seams. Which of the following is the most likely causative agent?

A 13-year-old patient with sickle-cell anemia presents with respiratory distress; she has an infiltrate on chest radiograph. The laboratory workup of the patient reveals the following: hemoglobin 5 g/dL; hematocrit 16%; white blood cell count 30,000/µL; and arterial blood (room air) pH 7.1, PO2: 35 mm Hg, and PaCO2: 28 mm Hg. These values indicate which of the following?

An 8-year-old boy is seen in the pediatrician's office for a routine health supervision visit. The mother states that he has Duchenne muscular dystrophy. On examination, he is found to have hip waddle and enlargement of both calves. He is ambulatory, but his muscle strength is diminished symmetrically. Which of the following signs is most consistent with Duchenne muscular dystrophy?

A 9-year-old boy is brought to the pediatrician's office for bed-wetting. His mother states that he has never been dry at night. Occasionally, he has problems controlling his bladder during the day. On physical examination, his blood pressure is 98/56 mm Hg. Both his weight and height are below the 5th percentile for his age. His bladder is enlarged and palpable above the symphysis pubis. Which of the following is the most likely cause of his problem?

A frail 6-year-old child who has cystic fibrosis is transported by ambulance to the emergency department. She has had hemoptysis for the past 4 hours, yielding approximately 10 mL of bright red blood. She has had increased cough over the past 3 days. Physical examination findings include a respiratory rate of 38 breaths/min, heart rate of 90 beats/min, oxygen saturation of 92% on room air, blood pressure of 100/70 mm Hg, and temperature of 98.6ºF (37ºC). She is awake and alert but seems breathless when she tries to speak. On auscultation, you note diffuse crackles throughout her lung fields. Of the following, the BEST next step in the management of this patient is to

A child can walk well holding on to furniture but is slightly wobbly when walking alone. She uses a neat pincer grasp to pick up a pellet, and she can release a cube into a cup after it has been demonstrated to her. She tries to build a tower of two cubes with variable success. She is most likely at which of the following age?

A 7 year old with a 3-day history of upper respiratory tract infection is brought to accident and emergency by his mother because he suddenly went pale and sweaty and seems to be working hard to breath. The triage nurse calls you to see him urgently because his heart rate is 200 beats per minute. You take him round to the resuscitation area, give him oxygen and connect him to the cardiac monitor. The electrocardiogram (ECG) shows a narrow complex tachycardia with a rate of 180 beats per minute. He remains alert, with a respiratory rate of 40. What is the most appropriate initial diagnosis?

A 41-year-old woman who is 32-weeks pregnant presents to the emergency department in labor. Her pregnancy has been complicated by gestational hypertension. Her medical history is otherwise unremarkable. She does not abuse alcohol, tobacco, or illicit drugs. Despite medical interventions, her preterm labor cannot be arrested, and she delivers a 2 kg (41b, 5oz) female infant. Both she and the child do well after delivery. Which of the following is true regarding immunizations for this infant?

A 1-week-old infant presents for his first newborn evaluation. He had been discharged apparently well and thriving at 48 hours of age. He now exhibits grouped vesicles on an erythematous base that were not present at birth. Wright stain of scrapings from the floor of the vesicles reveals multinucleated giant cells and balloon cells. Of the following, the MOST likely diagnosis is

A 6-week-old male infant, who was born at 32 weeks' gestation with a birth weight of 1500 g, has had an average weight gain of 8 g/day since birth. He takes an iron-fortified formula that is 24kcal/oz. His calorie intake is about 125kcal/day. It is noted that his stool is poorly formed and bulky. Which of the following dietary modifications will most likely result in decreased steatorrhea and improved weight gain?

A one-and-a-half-year-old Caucasian child is referred to paediatrics for failure to thrive. On examination he is a clean, well-dressed child who is quite quiet and withdrawn. He is pale and looks thin with wasted buttocks. His examination is otherwise unremarkable. What is the most likely cause of this child’s growth failure? His growth chart shows good growth along the 50th centile until 6 months followed by weight down to the 9th, height down to 25th and head circumference now starting to falter at 1.5 years.

A previously healthy 18-month-old has been in a separate room from his family. The family notices the sudden onset of coughing, which resolves in a few minutes. Subsequently, the patient appears to be normal except for increased amounts of drooling and refusal to take foods orally. Which of the following is the most likely explanation for this toddler’s condition?

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

A child presents for her 2-week evaluation after being delivered by a midwife at home. The parents are concerned that they have never seen her turn her head, which makes it difficult for her to feed at the breast. They also note that her back does not appear normal. On physical examination, her hairline appears low posteriorly. You confirm that she does not turn her head, and when placed prone, does not turn her head to the side. Her right scapula appears to be higher than the left, and you note that the spine does not appear to be perfectly straight, suggesting congenital scoliosis. You obtain an anteroposterior radiograph of the cervical spine, which shows multilevel segmentation anomalies (Item Q165A) in the mid to lower cervical spine. Of the following, the condition that is MOST consistent with these findings is

A 21-year-old woman presents to the emergency room in active labor. She has had no prenatal care, but her last menstrual period was approximately 9 months prior. Her membranes are artificially ruptured, yielding no amniotic fluid. She delivers an 1800-g (4-lb) term infant who develops significant respiratory distress immediately at birth. The first chest radiograph on this infant demonstrates hypoplastic lungs. After this infant is stabilized, which of the following is the most appropriate next step for this infant?

A 28-year-old Caucasian female comes to the office due to fever for the past 3 days. She also complains of pain and swelling in her right breast for the past 4 days. She exclusively breastfeeds her 3-month-old baby. On examination, she is febrile. Her right breast is engorged, red and tender, without any area of fluctuation. She wants to know if she can continue to breastfeed. Which of the following is a contraindication to breastfeeding?

A mother brings her infant into the clinic for a routine visit. Examination reveals nothing abnormal. Developmental assessment shows that the infant sits well unsupported, enjoys looking around, babbles, and has a raking grasp. What is the most likely age of this infant?

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?

A 15-year-old girl is admitted to the hospital with a 6-kg weight loss, bloody diarrhea, and fever that have occurred intermittently over the previous 6 months. She reports cramping abdominal pain with bowel movements. She also reports secondary amenorrhea during this time. Stool cultures in her physician’s office have shown only normal intestinal flora. A urine pregnancy test was negative, while an erythrocyte sedimentation rate (ESR) was elevated. Her examination is significant for the lack of oral mucosal ulcerations and a normal perianal examination. Anti-Saccharomyces cerevisiae antibodies (ASCA) are negative, while anti-neutrophil cytoplasm antibodies (pANCA) are positive. You confirm your presumptive diagnosis with a rectal biopsy. In counseling her about her disease, which of the following statements would be true?

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

In examining a 4-year-old girl who is new to your practice, you discover that she has rudimentary thumbs and is well below the 5th percentile for both weight and height. You also observe irregular hyperpigmentation on the trunk and anogenital areas. Of the following, the MOST likely hematologic disorder associated with these findings is

A 4-year-old girl is brought to the office due to easy bruising and a rash for the past 3 days. She had an upper respiratory infection approximately two weeks ago. She has never had palpable bruises, hemarthroses, or deep muscle bleeding in the past. She has 2 older brothers, neither of which has had bleeding symptoms before. Her family history is negative for any bleeding disorders. Her vital signs are normal. Physical examination reveals a diffuse petechial rash on her neck, trunk, extremities and groin. There is no hepatosplenomegaly. Nonpalpable ecchymoses of varying ages are present on the shins and arms. The laboratory findings are as follows: Hemoglobin 12 .8 g/dl, Hematocrit 38.5%, WBC 6,000/mm3, Platelets 5,000/mm3, PT 12.0 sec, PTT 30 sec. Peripheral smear shows normal morphology of the red and white blood cell lines. The platelets are reduced in number, and majority of them are increased in size. What is the most likely diagnosis?

A 6-year-old girl presents to accident and emergency with a fever. She has no history of cough, cold, vomiting, diarrhoea, rash, headache or joint pain. On examination, she is tachycardic at 150 bpm and there are two petechial spots on her right ankle. Her capillary refill time is 4 seconds and she has cold feet. All her other observations are normal. What is the most appropriate course of action?

A 16-year-old girl presents to your office with a recent diagnosis of peptic ulcer. The gastroenterologist has prescribed sucralfate. Of the following, the condition that is a relative contraindication to sucralfate treatment is

A young black male with sickle cell trait is preparing for his travel to West Africa from North America. While obtaining his visa, he is advised by the health inspector to obtain some immunizations, although his chances of getting a certain tropical disorder are low. What disease has the sickle cell trait been shown to convey protection against?

An 8-year-old boy presents to your office for a second opinion. He has a 2-year history of intermittent vomiting, dysphagia, and epigastric pain. His father reports he occasionally gets food “stuck” in his throat. He has been on a proton pump inhibitor for 18 months without symptom relief. His past history is significant only for eczema and a peanut allergy. Endoscopy was performed 6 months ago; no erosive lesions were noted and a biopsy was not performed. You arrange for a repeat endoscopy with biopsy. Microscopy on the biopsy sample reveals many eosinophils. Treatment of this condition should include which of the following?

You see a boy in outpatients whose parents are concerned he is not talking yet. You do a developmental assessment and find he is walking well and able to build a tower of three blocks. He will scribble but does not copy your circle. He is able to identify his nose, mouth, eyes and ears as well as point to mummy and daddy. You do not hear him say anything but his parents say he will say a few single words at home such as mummy, daddy, cup and cat. He is a happy, alert child. Parents report him to be starting to feed himself with a spoon and they have just started potty training but he is still in nappies. What is the child’s most likely age?

A 12-year-old male is brought to your office by his mother because of a several month history of back pain. He also experienced bed wetting recently. His past medical history is insignificant. He tried acetaminophen for pain relief. He does not smoke or consume alcohol. His temperature is 36.7 C (98 F), blood pressure is 110/65 mmHg, pulse is 80/min, and respirations are 16/min. Physical examination reveals a palpable "step off" at the lumbosacral area. Straight-leg raising test is negative on both sides. Perianal sensation tested by pinprick test is decreased, but anal reflex is normal. What is the most probable diagnosis in this patient?

A 7-year-old boy is brought to the physician because of a persistent mucopurulent nasal discharge for 2 weeks following a common cold. The mother also reports that the child has had frequent cough during the day and occasional temperatures up to 38.0 C (100.5 F). The child does not appear critically ill, but he complains of mild pain in the maxillary region and nasal obstruction. Rhinoscopic examination reveals a rivulet of purulent fluid coming from the inferior meatus. The rest of the physical examination is normal. Which of the following is the most likely diagnosis?

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?

A young mother claims that her 4-week-old child sleeps best on his stomach. You tell her that the safest sleep position for infants is which of the following?

A 16-year-old boy presents with a very swollen, painful right knee. He is a soccer player, but there is no history of recent injury. During the interview, you notice the boy has injected conjunctivae. Of the following, further evaluation MOST likely will reveal

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?

A primiparous woman whose blood type is O positive gives birth at term to an infant who has A-positive blood and a hematocrit of 55%. A total serum bilirubin level obtained at 36 hours of age is 12 mg/dL. Which of the following additional laboratory findings would be characteristic of ABO hemolytic disease in this infant?

A 4-year-old previously well African American boy is brought to the office by his aunt. She reports that he developed pallor, dark urine, and jaundice over the past few days. He stays with her, has not traveled, and has not been exposed to a jaundiced person, but he is taking trimethoprim sulfamethoxazole for otitis media. The CBC in the office shows a low hemoglobin and hematocrit, while his “stat” serum electrolytes, blood urea nitrogen (BUN), and chemistries are remarkable only for an elevation of his bilirubin levels. His aunt seems to recall his 8-year-old brother having had an “allergic reaction” to aspirin, which also caused a short-lived period of anemia and jaundice. Which of the following is the most likely cause of this patient’s symptoms?

A 7 -day-old male infant is brought by his mother complaining of decreased movements of his right arm. She denies any trauma or fall. Pregnancy was uneventful, but delivery was complicated with shoulder dystocia. Examination reveals the presence of crepitus and bony irregularity over the clavicular area and Mora reflex is absent on the right. Which of the following is the most appropriate management?

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?

A 12-year-old African American male with known sickle cell disease presents with a 2 hours history of right-sided arm weakness ans slurred speech. He has been hospitalized before for pain crises and pneumonia. He takes hydroxyurea, oxycodones as needed, and folic acid. His temperature is 36.6 C, BP is 153/83 mmHg and HR is 112/min with regular rhythm. On physical examination, he has right arm weakness and mild dysarthria. His laboratory values are the following: Hemoglobin 8.2 mg/dL, WBC count 14, 000.mm3, Platelet count 210, 000/mm3, Creatinine 0.9 mg/dL. CT of the head shows no evidence of intracranial bleeding. Which of the following is the best initial management for this patient?

A 10-year-old male fell while riding his scooter down a steep hill. In the Emergency Department, his injuries included a fractured wrist and a lacerated spleen, which required surgical removal. Two years later he is diagnosed with bacterial pneumonia. Which of the following bacterial agents is the most likely pathogen for this patient's pneumonia?

A mentally retarded 14-year-old boy has a long face, large ears, micropenis, and large testes. Chromosome analysis is likely to demonstrate which of the following?

You are evaluating a newborn boy who has lax abdominal musculature and bilateral undescended testes. Other findings on physical examination are normal. Of the following, the MOST likely urologic abnormality in this boy is

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?

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?

An 8-year-old immigrant from rural Central America presents with complaints of weakness, facial swelling, muscle pain, and fever. A CBC reveals marked eosinophilia. Which of the following parasites is most likely to be responsible?

An 8-year-old girl presents with well-controlled, moderately persistent asthma. Her therapies consist of occasional use of short-acting β-agonists, daily inhaled steroids, and a leukotriene inhibitor. She presents with white patches on her buccal mucosa. You recommend which of the following?

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 x-ray 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?

A 1-year-old presents for a well-child checkup, but the parents are concerned about giving the child his immunizations. Which of the following is a true contraindication to the administration of the fourth DTaP (diphtheria and tetanus toxoid and acellular pertussis) vaccine?

A 3-year-old boy is brought to the emergency department because of a worsening cough over the past week. His temperature is 38.9 C (102 F), and inspiratory stridor is noted. A plain film of the neck reveals subglottic swelling. He is noted to have copious thick secretions and a barking cough. He has not had such events previously, and his parents deny recent contact with sick children. The patient is in respiratory distress and is noted to be retracting his subcostal muscles to breathe. Which of the following is the next most appropriate step in management?

A 2-month-old girl presents to her pediatrician's office for well-child care. Her mother complains of excessive tearing of the baby's left eye for the past 4 weeks. Each morning, a yellow crusty discharge is noted along the lashes of the left eye. The conjunctiva appears uninflamed. The right eye is not affected. On physical examination, the infant is otherwise well and achieving adequate weight gain on an exclusive breast milk diet. She is developmentally appropriate, including visually tracking 180 degrees. Which of the following is the most likely diagnosis?

A 3-week-old baby is brought to the ‘prolonged jaundice clinic’. His mother reports he has poor feeding, is not gaining weight appropriately and is more sleepy compared to her previous child. He opens his bowel once a day and is being mix breast and bottle fed. He is floppy, jaundiced, has a large, protruding tongue and a hoarse cry. He had a newborn blood spot screening test done at birth which was normal and he has no dysmorphic features. What is the most likely diagnosis of this child?

A 14-year-old girl is brought to the physician’s office because of irregular menstrual periods. She had her menarche at age 13, and since then her periods have been irregular with the cycles varying from 3 to 6 weeks. She has no other symptoms. Physical examination is unremarkable. She has age appropriate secondary sexual characteristics. A urine pregnancy test is negative. Serum prolactin and thyroid stimulating hormone levels are normal. Administration of micronized oral progesterone results in withdrawal bleeding in 3 days. Which of the following most likely explains her irregular periods?

A 4-week-old infant presents with tachycardia, tachypnea, and poor weight gain. His arterial blood gas shows a pH of 7.34, a PaCO2 of 41 mm Hg, and a PaO2 of 74 mm Hg. A chest radiograph shows cardiomegaly. Echocardiography reveals a structurally normal heart, left ventricular dilatation, a left ventricular ejection fraction of 20%, and mild mitral and tricuspid regurgitation. IV administration of which of the following medications is the best initial step in management of this patient?

A 12-year-old boy comes to the emergency department at midnight with a complaint of severe scrotal pain since 7 PM. There is no history of trauma. Which of the following is the most appropriate first step in management?

A 3-year-old girl presents to the pediatrician's office. The mother states that the girl has been having big, bulky stools that float in the toilet. She also has intermittent diarrhea. On examination, her height is 88 cm (34.6 in, <5th percentile) and weight is 15.8 kg (34.8 lb, <5th percentile). In addition, she has an uncle who died of recurrent lower respiratory infections. Which of the following would be most effective for alleviating the gastrointestinal symptoms of this patient?

Examination of the cerebrospinal fluid (CSF) of an 8-year-old, mildly febrile child with nuchal rigidity and intermittent stupor shows the following: WBCs 85/µL (all lymphocytes), negative Gram stain, protein 150 mg/dL, and glucose 15 mg/dL. A computed tomographic (CT) scan with contrast shows enhancement of the basal cisterns by the contrast material. Which of the following is the most likely diagnosis?

A 3-year-old boy is brought to the physician for help with toilet training. He recently started day care and screams "no" when teachers try to place him on the toilet. He has bowel movements every other day and strains when he passes hard, pellet-like stools. The boy is a picky eater but loves milk and drinks up to 30 oz of chocolate milk daily. He has no medical problems and takes no medications. His weight and height have been tracking along the 75th percentile. Examination shows a cooperative, well-nourished boy. He runs well and can climb onto the examination table independently. He speaks in short sentences that are mostly understandable. The boy's abdomen is soft, nontender, and nondistended. He has normal Tanner I male genitalia. A small fissure is noted on the anal verge. Which of the following is the best next step in management of this patient?

An 18-month-old boy has received 5 days of amoxicillin for otitis media. He continues to have fever, and on physical examination, the right tympanic membrane is bulging with purulent fluid behind it. Which of the following is the best antibiotic to use?

A 12-year-old boy presents with an intensely pruritic rash for 3 days. He just went on a camping trip, during which he wore only shortsleeve shirts and short pants. His temperature is 37.6 C (99.7 F), blood pressure is 96/62 mm Hg, pulse is 65/min, and respirations are 12/min. There are numerous erythematous papules and vesicles on both arms and legs. Most of them are in a linear array. Which of the following is the most appropriate pharmacotherapy?

A 3-year-old girl is brought to the pediatrician with complaints of abdominal pain and fever. Her mother states that the fever started 2 days ago, with the highest temperature being 39.0 C (102.2 F). She has had no vomiting or diarrhea. The mother states that her daughter has been complaining of pain on urination. On examination, she is tender in her lower abdomen, and there is some right-sided costovertebral angle tenderness. A urinalysis confirms the suspicion of a urinary tract infection. Which of the following would be the most appropriate diagnostic procedure?

The mother of a 2-year-old boy comes to the physician because her child awakens at night, with a blank gaze, screaming in bed without recognizing his parents. These episodes have occurred three times in the past 2 weeks, always in the first few hours of the night. The child goes back to sleep and seems to retain no memory of the episode the next morning. Which of the following is the most appropriate next step in management?

A nurse asks you to examine a 26-hour-old infant who recently developed a rash. On physical examination, you note erythematous macules over the trunk, face, and proximal extremities. Most of the macules have tiny central pustules. The infant is breastfeeding well, and the remainder of the physical examination findings are normal. No lesions were present at birth. Of the following, analysis of the pustular contents is MOST likely to reveal

A previously healthy 4-year-old child is brought to the physician for a school physical for kindergarten. His mother has brought in his immunization record and reports that received all his immunizations prior to age 2. He has received only a yearly influenza vaccine since age 2. His physical examination is unremarkable. Which of the following immunizations should he be given during this visit?

A mother brings her 3-year-old son to the pediatrician because he has had 7 days of fever and a painful swollen lymph node in his right groin. This is the boy's sixth episode of lymph node swelling; previous episodes resolved after drainage and prolonged antibiotic therapy. The child also has a past medical history significant for pneumonia at 12 months of age that required chest tube placement for drainage. His maternal uncle died in childhood from recurrent infections. On examination, the boy is at the 5th percentile for both height and weight His temperature is 38.5 c (101.3 F). There is an enlarged, tender lymph node in the right inguinal area with erythema and warmth of the overlying skin. There are several healed incisions in the inguinal regions bilaterally, as well as around the neck from old drainage sites. Laboratory analyses reveal the following: Hematocrit 35%, Platelet count 350,000/mm3, Leukocyte count 17000/mm3, Segmented neutrophils 65%, Bands 10%, Lymphocytes 25%. Gram stain of fluid aspirated from the affected lymph node reveals numerous bacteriafilled segmented neutrophils. Cultures grow S. aureus. What is the most likely mechanism underlying these findings?

A woman gives birth to twins at 38 weeks’ gestation. The first twin weighs 2800 g (6 lb, 3 oz) and has a hematocrit of 70%; the second twin weighs 2100 g (4 lb, 10 oz) and has a hematocrit of 40%. Which of the following statements is correct?

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 100% oxygen, chest compressions, intravenous epinephrine, 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 mmol/L. 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:

A 4-month-old is brought to clinic by his parents for evaluation of bilateral droopy eyes. His mother believes this has developed just over the last week. The child recently started taking cereal in addition to breastfeeding and has been constipated. Physical examination reveals droopy eyelids and 1+ deep tendon reflexes diffusely. Of the following, the MOST likely diagnosis is

A blood type B infant born to a blood type O mother has clinically significant fetal-maternal blood group incompatibility with mild anemia and a weakly positive Coombs test. The infant develops jaundice a few hours after birth, with a bilirubin (measured at 12 hours after birth) of 12 mg/dL (predominately unconjugated) compared with 3.5 mg/dL in cord blood. The physician is concerned that the rising bilirubin levels will damage the infant's nervous system. Which of the following sites is most vulnerable to this injury?

You are evaluating a 2-year-old boy in your office for recurrent cellulitis of his right thigh. The patient has had three episodes in the last 4 months. According to his mother, all the episodes start with a "red bump" that progressively enlarges and, in most cases, drains spontaneously. He has received two courses of cephalexin in the past, but there was no clinical improvement until the abscess spontaneously drained. On one occasion, an incision and drainage procedure had to be performed. Except for pain with walking, the patient has been afebrile and experienced no other systemic symptoms. On physical examination, you note a 6x6 cm area of induration and erythema on the lateral right thigh that is warm, firm, and tender to palpation. There is no active drainage from the site. Of the following, the MOST appropriate antibiotic for treatment of this patient is

A child is brought to your clinic for a routine examination. She can put on a T-shirt but requires a bit of help dressing otherwise. She can copy a circle well but has difficulty in copying a square. Her speech is understandable and she knows four colors. She balances proudly on each foot for 2 seconds but is unable to hold the stance for 5 seconds. Which of the following is the most likely age of this child?

A one-year-old girl is brought to the office for the evaluation of sickle cell anemia. Both her parents have sickle cell trait, and her elder sister has sickle cell anemia. Her vital signs are stable. Her height and weight are appropriate for age. On examination, she appears healthy and normal. Her birth and past medical histories are insignificant. Hemoglobin electrophoresis reveals 40% hemoglobin S. What is the most common presentation of this condition?

A 10-year-old boy presents to the emergency room with chief complaints of fever and increasing fatigue. He was well until 2 weeks ago when he had an upper respiratory illness (URI). He has a decreased appetite and has lost 2 lbs over the last 2 weeks. He has some shortness of breath when he climbs the stairs. His past medical history is unremarkable. On examination his vital signs are normal, and his height and weight are at the 80th percentile. His conjunctiva are pale, and he has bilateral, mobile (about 1-2cm) and non-tender nodes in the cervical, axillary, and inguinal regions. Hepatosplenomegaly is present, and skin shows no lesions. Labs are Hemoglobin 7.3g/dl, platelet count 20,000/mm3, WBC count 42,100/mm3, with 24% lymphoblasts, 70% lymphocytes, and 6% atypical lymphocytes. Chest X-rays show clear lung fields but a wide mediastinum. Which of the following is the most likely diagnosis in this patient?

A 5-year-old boy suffers from a condition characterized by recurrent fungal and viral infections, thymic hypoplasia, tetany, and abnormal facies. Serum levels of immunoglobulins are mildly depressed, and lymph node biopsy shows lymphocyte depletion of T-dependent areas. Which of the following is the underlying pathogenetic mechanism?

A 16-year-old African American male with sickle cell anemia presents complaining of a 1-week history of exertional dyspnea, fatigue, and generalized weakness. He denies fevers, chills, night sweats, or cough. His only medication is oxycodone for chronic pain. On physical examination, he weighs 68 kg (150 lbs) and is 168cm (66 in) tall His temperature is 36.7C (98F), blood pressure is 120/70 mm Hg, pulse is 76/min, and respirations are 18/min. All organ systems appear normal Laboratory studies show: Hematocrit 20%, Mean corpuscular volume 110 fl, Reticulocyte count 1.0%. Which of the following is the most likely mechanism underlying these findings?

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

A 3-year-old boy is brought to the office by his parents for the evaluation of dry eyes and photophobia. He has some difficulty in adapting to darkness. He is a very poor eater, and his diet consists mainly of canned foods, and very rarely, fresh vegetables or milk. Examination reveals dry, scaly skin, follicular hyperkeratosis in the extensor surfaces of the extremities, and dry, silver-gray plaques on the bulbar conjunctiva. What is the most likely diagnosis of this patient?

A 20-year-old man presents to the infectious diseases department with a large 7 cm× 8 cm swollen painful lump in the left anterior triangle of his neck. He has night sweats, 10 kg weight loss and a dry cough for the last month. He was treated with surgery and radiotherapy for a high grade astrocytoma when he was 8 years old. Which of the following is not a recognized complication of his childhood condition and its treatment?

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?

A 5-year-old is referred to paediatrics due to concerns initially raised by his school teacher that he is weak and clumsy. On examination he has wasting of his quadriceps and walks in a waddling gait. His blood creatine kinase is 1600 mmol/L (normal is 24–190). What is the most likely diagnosis?

A 7-year-old girl is brought to the physician because of an exanthematous rash associated with malaise and headache for 2 days. On examination, the child shows a fiery red facial rash with a characteristic "slapped cheek" pattern and pallor around the mouth. There is no fever. In immunocompromised patients, the pathogen that causes this condition may result in which of the following manifestations?

A 17-year-old adolescent comes to your office seeking help for “heavy” menses. Your review of systems also reveals weekly epistaxis. Her only significant past history includes a tonsillectomy at age 6 after which she required blood transfusion for excessive bleeding. Her family history includes several people who seem to bleed and bruise more easily than others. The patient’s mother required a hysterectomy after child birth for excessive hemorrhage. You order a variety of laboratory tests. The patient has a hemoglobin of 6.5 mg/dL with an MCV of 60%; her platelet count is 350,000/µL. Her von Willebrand antigen and her von Willebrand factor (vWF) activity (ristocetin cofactor activity) are decreased. Her vWF is reported as normal but in decreased amounts. You have been unable to reach her to report the findings, but when she calls about 1 week later she reports she is having a mild to moderate nosebleed. You initiate therapy with which of the following?

A 2-month-old baby is brought in by the babysitter because he has been crying since she arrived to look after him and his right leg looks swollen. He is the only child living in the household. She does not think he is moving it and is worried it is injured. On examination he is miserable, his heart rate is 160, respiratory rate of 56, and capillary refill is less than 2 seconds. He has a swollen right thigh. He cries more when that leg is examined. You note a yellow bruise on his left thigh and two purple bruises on either arm. X-rays show a fracture of the right femur but the arms appear intact. A chest x-ray shows three healing posterior rib fractures. You are highly suspicious of non-accidental injury. What is the most appropriate management?

A 2-month-old female infant born at term is brought to the office for a well-baby visit. Her antenatal and birth histories are unremarkable. Her developmental milestones are all normal for her age. Ophthalmoscopic examination reveals a white reflex in the right eye. The rest of the examination is normal. What is the most appropriate next step in the management of this patient?

You have been the primary practitioner for a 15-year-old girl since she was 3 years old. She never has been hospitalized, and her history, vital signs, and physical examination findings on this health supervision visit are normal. At each of the past two yearly visits, urinalysis revealed no abnormalities except 2+ protein. Her urinalysis today again reveals 2+ protein with no other abnormalities. Her complete blood count, measurements of serum electrolytes and serum complements, and antinuclear antibody test results are all normal. Two successive 24-hour urine collections reveal 321 and 387 mg protein. You now refer the girl to a pediatric nephrologist. Of the following, the MOST important next step for the pediatric nephrologist is to

The accident and emergency triage nurse asks you to look at a 3-year-old child with a short history of waking up this morning unwell with a cough and fever. She looks unwell, heart rate is 165, respiratory rate 56, saturations of 96 per cent in air, temperature of 39.3°C and central capillary refill of 4 seconds. She has a mild headache but no photophobia or neck stiffness and you notice a faint macular rash on her torso and wonder if one spot is non-blanching. You ask the triage nurse to move her to the resuscitation area and call your senior to review her. Fifteen minutes later your senior arrives and the spot you saw on the abdomen is now non-blanching and there is another spot on her knee. What are the three most important things to give her immediately?

During the health supervision visit of a 2-year-old boy who is new to your practice, you note a productive cough that his mother says has been present for several months. His weight is below the 5th percentile. Fine crackles are present in his lungs bilaterally, and he has mild clubbing of the fingers. The remainder of his physical examination findings are unremarkable. A chest radiograph reveals poorly defined bronchovascular markings and a few cystic spaces bilaterally, prompting you to order computed tomography scan of the chest, which reveals diffuse bronchiectasis. Of the following, the MOST likely diagnosis is

A 4-day-old infant is brought to the physician for an outpatient follow-up visit. The mother's pregnancy and delivery were uncomplicated. The infant weighed 3.4kg (7 .5 lb) and was 19 in (48.2 cm) long at birth. He did well in the newborn nursery and was discharged from the hospital on day 2 of life. Today his mother reports that he is exclusively breastfed, and nurses for 10 minutes every 3 hours. He has 3-4 wet diapers a day, and has not had a bowel movement for two days. On examination, he weighs 2.95 kg (6.5 lb) and is 19 in (48.2 cm) long. He appears jaundiced on the face and chest. The remainder of the physical examination is unremarkable. Laboratory values are shown below. Total bilirubin 15 mg/dl, direct bilirubin 1 mg/dl, Infant's blood type O positive, Mother's blood type A positive. Which of the following is the most likely cause of this infant's hyperbilirubinemia?

A 2-year-old child has had red, weeping, crusted lesions of the face, scalp, diaper area, and extremities since about age 2 months, with multiple periods of exacerbation and improvement. Attempts to remove potentially irritating substances have not modified the course of the rashes. The child is noted to be constantly scratching and rubbing involved areas. There is a strong family history of hay fever and asthma. Which of the following is the most likely diagnosis?

You are asked to evaluate an infant born vaginally 3 hours previously to a mother whose only pregnancy complication was poorly controlled gestational diabetes. The nursing staff noticed that the infant was breathing abnormally. On examination, you find that the infant is cyanotic, has irregular, labored breathing, and has decreased breath sounds on the right side. You also note decreased tone in the right arm. You provide oxygen and order a stat portable chest radiograph, which is normal. Which of the following studies is most likely to confirm your diagnosis?

A 4196 g (9 lb 4 oz) infant is delivered via vaginal delivery to a 31-year-old mother with gestational diabetes. The delivery was complicated by shoulder dystocia. He is taken to the newborn nursery where his initial plasma glucose level is 20 mg/dL. The initial spun hematocrit is 65%. Which of the following congenital anomalies is this baby most likely to have?

A 12-year-old girl presents to the emergency department with abdominal pain. Her parents report that she awakened with a temperature of 101°F (38.4°C) this morning, complained of abdominal pain, and has vomited twice. There is no diarrhea, and there are no sick contacts. She reports nausea and no interest in food or drink. She was previously entirely healthy. Urinalysis reveals a specific gravity of 1.015 and is otherwise normal. Of the following, the finding that MOST indicates the need for immediate surgical intervention is

A previously healthy, active, 18-month-old African American child presents with unilateral nasal obstruction and foul-smelling discharge. The child’s examination is otherwise unremarkable. Which of the following is the most likely diagnosis?

A 10-hour-old newborn is noted to have a swelling in the scalp which was not present at birth. The swelling is limited to the surface of one cranial bone. There are no visible pulsations, indentations of the skull, or discoloration of the overlying scalp. What is the most likely diagnosis?

A 7-year-old boy presents with tenderness and erythema of one knee joint. He has had troubles with infections since about 3 months of age. A brother and a maternal uncle both died of infectious disease at an early age. A detailed immunologic evaluation performed at 2 years of age demonstrated plasma IgG less than 50 mg/100 mL. Normal numbers of circulating T cells and normal cellular immunity were found. The boy had been treated monthly since then with IV immunoglobulin. This therapy had markedly reduced, but not eliminated, the boy's infection rate. Which of the following is the most likely pathogen to cause infectious arthritis in this patient?

A 2-week-old baby was referred to the prolonged jaundice clinic by the community midwife. The pregnancy was unremarkable, and she was born at term with no antenatal abnormalities on ultrasound (US) scans or blood serology. She is now 17 days old and has been jaundiced since day 5 of life and never required phototherapy. She is breastfed and feeds 3-hourly for 20–25 minutes. She is afebrile and not lethargic. Her mother reports that the stools are pale and she has dark coloured urine. The bilirubin is 300 µmol/L, and conjugated bilirubin 100 µmol/L. What is the most important diagnosis to exclude?

A 1-year-old African American infant is in for well-child care. He is primarily breast-fed. His parents do not give him much solid food because he has no teeth. He receives no medications or supplements. His parents are concerned about his bowed legs. On examination, you note some other bony abnormalities including frontal bossing, enlargement of the costochondral junctions, a protuberant sternum (pigeon chest), and severe bowing of the legs. You obtain x-rays to confirm your clinical diagnosis and also note a healing fracture of the left femur. Which of the following is the most likely diagnosis?

A woman comes to an emergency department because she is in labor. She has had no prenatal care. Her baby is delivered and appears to be of about 32 weeks' gestation. The newborn is very pale and shows severe, generalized edema. Cord-blood hematocrit is 22%, and cord-blood bilirubin is 7 mg/dl. Ultrasound examination demonstrates pleural effusions, ascites, cardiomegaly, and hepatomegaly. Which of the following is the most likely diagnosis?

A 7 -year-old boy is brought to the emergency department after falling on his outstretched hand. On examination, there is minimal swelling at the right elbow. The skin on the right forearm appears tense, and severe pain is present. The brachial pulses are good bilaterally, and sensations are intact in both upper limbs. X-rays of the right upper limb show a displaced anterior fat pad. Which of the following statements is true about this condition?

A 16-year-old girl who was treated successfully for leukemia that presented as a mediastinal mass at 2 years of age comes to your office complaining of a "lump" in her neck of 3 weeks' duration. On physical examination, you note a firm, hard, fixed mass that seems to be part of the left lobe of the thyroid and scattered shotty lymphadenopathy on the left. Of the following, the MOST informative laboratory study for this patient is

Your 6-year-old son awakens at 1:00 AM screaming. You note that he is hyperventilating, is tachycardic, and has dilated pupils. He cannot be consoled, does not respond, and is unaware of his environment. After a few minutes, he returns to normal sleep. He recalls nothing the following morning. Which of the following is the most likely diagnosis?

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

An otherwise healthy 7-year-old girl is brought to your office by her father because she has some acne, breast development, and fine pubic hair. Which of the following is the most likely etiology for her condition?

A school teacher calls you to report that his 7-year-old student had about 10 episodes of eye blinking and gabbling today, which lasted approximately 30 seconds each. The child sat in his chair and did not have any postictal confusion. The teacher noticed several similar episodes during the last month, as well as a decline in the child's school performance. What type of seizure does this child have?

You are examining a newborn infant in the well-baby nursery. The infant was the product of a benign pregnancy and vaginal delivery; he appears to be in no distress. Interestingly, your measurement of fronto- occipital head circumference is about 2 cm larger than the initial measurement done several hours before. Your examination otherwise is significant for tachycardia and a “squishy” feel to the entire scalp. You can elicit a fluid wave over the scalp. Management of this condition should include which of the following?

A one-day-old infant with Down syndrome has developed persistent vomiting. He was delivered vaginally at 34 weeks without any complications. On examination, he appears dehydrated and slightly tachypneic. His abdomen is soft and not distended. Abdominal x-ray reveals two large distinct air bubbles, but there are no dilated bowel loops or air fluid levels. What is the most likely diagnosis of this patient?

A 3-year-old boy is brought by his father to the Emergency Department with fever, headache and neck pain that developed over the past several hours. The father states he is not the birth father, and that he and his wife adopted the boy at 18 months of age after his birth mother abandoned him. Physical examination reveals a lethargic male with a temperature of 39.7 C (103.5 F). There is photophobia, and mildly injected conjunctiva are appreciated. Pupils are equal and reactive and funduscopic examination is unremarkable. The patient has neck stiffness with a positive Kernig's sign. A complete blood count reveals a leukocyte count of 24,000/mm3 with 64 segmented neutrophils and 25 bands. A lumbar puncture is performed that reveals elevated CSF pressure, decreased glucose, and elevated protein. A Gram's stain shows gram-negative pleomorphic rods. There is no growth on blood agar. Growth on chocolate agar reveals white colonies. Which of the following is the most likely pathogen?

A week-old infant presents blood in his stools. He was born at home, with the father assisting in the delivery; no physician or midwife was present. He has been breast-fed and has been nursing well. On examination, you also note some blood in his nose. He is not jaundiced; a rectal examination and guaic test of the stool confirms that blood is present. His examination is otherwise normal. He is on no medications. Which of the following is the most likely diagnosis?

A 9-year-old girl is stung on the left leg by a hornet while playing outside. Within 10 minutes, she experiences diffuse pruritus, and a large area of erythema develops at the sting site. Five minutes later, extensive raised welts appear on her trunk and extremities. Her parents rush her to the emergency department, where intravenous antihistamines and steroids are administered. During this episode, she denies difficulty breathing or swallowing. No tongue or uvula edema is evident during examination, and her symptoms gradually resolve over 4 hours. Of the following, the NEXT recommended step in her management is to

A 3-month-old boy is brought to the pediatrician because of a red growth on his arm. The pregnancy had been uncomplicated, and the infant has been meeting all development milestones. He has been healthy so far and has received all scheduled immunizations. He is currently being breast-fed. His skin was clear at birth, but when he was 2 months old, his mother noted a light red growth on his arm. Within the past month, it has increased in size and has turned bright red. Which of the following is the most appropriate treatment for this disorder?

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?

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 widebased 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?

A 9-year-old boy presents with a 3-month history of multiple episodes of sudden awakening at night. His mother states that when he wakes up suddenly, he screams, "Go! Get away! Go!" and does not respond to the parents. His eyes are wide open, and he sweats heavily and looks scared. The parents have had to struggle to awaken him. After the episodes, he has no memory of what happened. Which of the following is the most likely diagnosis?

A 4-year-old boy presents to your clinic for a second opinion. He has a 3-week history of diarrhea, abdominal pain, and tenesmus. The parents state that he seems to be getting worse, and nobody has been able to help them despite "a bunch of tests on his poop." His stool output has increased from four to five per day to eight to ten per day during the past week, and he now has a temperature of 102°F (38.9°C). They are starting to see what appears to be blood in the toilet after he goes to the bathroom. According to the parents, the boy was in good health until 1 week after they returned from a fishing trip on the Amazon river. Physical examination reveals a moderately illappearing boy who has diffuse abdominal pain. During your examination, he passes a very foul-smelling stool that appears to be a mixture of blood and pus, which you send to the laboratory for analysis. Of the following, the MOST appropriate next test is

A 6-year-old boy is brought to the office by his parents after hearing that chicken pox is infectious and can occur in unimmunized contacts. One week ago, he and his family visited relatives, and one of his cousins had chicken pox at that time. He and his two other siblings are apparently healthy, and never had any "chicken pox shots." What is the best next step in the management of this patient?

A routine prenatal ultrasound reveals a male fetus with meningomyelocele. The 24-year-old primigravid mother is told the infant will require surgery shortly after birth. You counsel her about the etiology of this defect and the risk of further pregnancies being similarly affected, and state which of the following?

A 3 year old is brought into accident and emergency by ambulance following a generalized tonic clonic seizure that lasted 2 minutes. She did not require any treatment to stop the seizure but on arrival the ambulance crew measured her temperature as 39.2 °C and gave paracetamol. She is now apyrexial with a heart rate of 140, respiratory rate of 30 and capillary refill less than 2 seconds. On examination she has red enlarged tonsils with no pus, no neck stiffness or rash. What is the most appropriate management?

A 3 month old is brought into accident and emergency with a generalized tonic clonic seizure. She is apyrexial and the seizure stopped after 15 minutes with rectal diazepam given by the ambulance crew. Her heart rate is 130, respiratory rate of 36 and capillary refill is less than 2 seconds. On examination she is drowsy, has a port wine stain on her forehead but is otherwise normal on examination. What is the most likely cause of her seizure?

The parents of a 10-year-old girl with newly diagnosed, generalized tonic-clonic epilepsy come to the physician seeking advice regarding what they should do when the child has a seizure. Which of the following suggestions is appropriate?

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 98.1ºF (36.7ºC) 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

A new mother complains that her 6-week-old infant frequently regurgitates small volumes of formula during and after feedings. Physical examination demonstrates a happy baby who has gained half a pound since his last visit. No abdominal masses are noted. Which of the following is the best next step in management?

A 12-year-old boy is brought to the emergency department with a temperature of 39.1 C (102.4 F) at home, difficulty speaking, and odynophagia for 2 days, Physical examination reveals marked erythema of the right tonsil pillar and edema of the uvula with deviation to the left. In addition to anaerobic bacteria, which of the following organisms is most likely to be isolated from a tonsillar pillar aspirate?

A 4-year-old child has been losing weight recently and has been vomiting for the past 24 hours, unable to eat anything. His mother has brought him into accident and emergency out of concern as he seems confused. The triage nurse has taken him to the resuscitation room and asked for your help. On examination he is drowsy, has a heart rate of 150, respiratory rate of 60 and a central capillary refill of 5 seconds. He has subcostal recessions and good air entry bilaterally with no added sounds. He moans when you examine his abdomen but there are no masses. You put in a canula and take bloods. The venous blood gas shows: pH 7.12, PCO2 2.3 kPa, PO2 6.7 kPa, HCO3– 15.3 mmol/L, BE –8.6, Glucose 32.4 mmol/L. What is the most likely diagnosis and what is the first management step?

You are called to examine a one-day-old male neonate who gradually developed cyanosis over the past few hours. The infant was delivered vaginally at full term, assisted with forceps, and weighed 9 lbs. The Apgar scores were 7 and 8 at 1 and 5 minutes, respectively. Oxygen administration by mask does not relieve the cyanosis. Further examination reveals tachypnea, subcostal retractions, a normal S 1, single and loud S2, and no murmur. Which of the following is the most probable cause of the infant's cyanosis?

A 12-year-old basketball player has complained of right knee pain immediately after games for 2 weeks. Although he had complained of knee pain intermittently earlier in the season, there is no history of swelling or injury of the knee. Physical examination findings of the knee are remarkable for full range of motion no effusion, and swelling over the proximal right tibia that is tender to palpation. Of the following, the MOST likely diagnosis is

A 3-year-old boy who has spina bifida has a history of recurrent urinary tract infections (UTIs). He is currently being treated for a UTI. Monitoring laboratory evaluation shows a blood urea nitrogen of 23 mg/dL (8.2 mmol/L) and a creatinine concentration of 1.1 mg/dL (97.2 mcmol/L). Of the following, the class of antibiotics that is MOST commonly associated with renal toxicity is

A 9-year-old boy is brought to the pediatric clinic by his mother, who noticed that the left side of his mouth has started to droop over the past several days. In addition, he is unable to close his left eye completely and complains of it burning. Review of systems reveals a cold approximately two weeks ago and recent decreased taste sensation. Physical examination reveals a well-nourished male with normal vital signs. There is left eye ptosis and mild erythema of the left conjunctiva. His smile is asymmetrical on the left. Laboratory evaluation, including a complete blood count and chemistry profile, are normal. Which of the following infections is most closely associated with this patient's condition?

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:

A 7-year-old boy who has had pain in his right leg for 4 months is being evaluated. The pain is worse at night and is unrelenting, but it can usually be relieved with ibuprofen. On physical examination, there is localized tenderness over the anterior aspect of the right thigh. There is also mild atrophy of the affected limb. A radiograph of the femur reveals a radiolucent nidus with surrounding reactive sclerotic bone. Which of the following is the most likely diagnosis?

A 7-year-old boy has a history of repeated urinary tract infections that have been treated by the empiric use of antibiotics. The parents are not satisfied with the care the child is receiving, and they take him to a pediatric urologist. Evaluation by voiding cystourethrogram shows that the patient has vesicoureteral reflux without ureteral or upper tract dilatation (grade one reflux). Which of the following is the appropriate management for this child?

A 5-year-old girl is being evaluated for generalized swelling. Her blood pressure is 98/60 mm Hg. Her laboratory results show: Creatinine 0.7 mg/dl, Albumin 1.6 g/dL, Cholesterol 360 mg/dL, Triglycerides 400 mg/dL, C3 complement 120 mg/dL (normal, >80 mg/dL), Antinuclear antibody Negative, Urinalysis 1 RBC/hpf, protein 400 mg/dL. Which of the following is the most likely diagnosis?

A 1-week-old black infant presents to you for the first time with a large, fairly well-defined, purple lesion over the buttocks bilaterally, as shown in the photograph. The lesion is not palpable, and it is not warm nor tender. The mother denies trauma and reports that the lesion has been present since birth. This otherwise well-appearing infant is growing and developing normally and appears normal upon physical examination. Which of the following is the most appropriate course of action in this infant?

As part of your clinic responsibilities, you supervise allergy shots for patients who have allergic rhinitis. The nurse calls you about a 12- year-old girl who received her allergy shot 30 minutes ago and now is experiencing warmth and erythema over the injection site. On physical examination, the patient appears healthy and in no distress. Vital signs include a temperature of97.5°F (36.4°C), heart rate of 90 beats/min, and respiratory rate of 18 breaths/min. At the injection site, there is a 4 x 4 cm raised, erythematous, warm area. There is no edema of the tongue or uvula, and findings on her pulmonary, cardiovascular, and skin examinations are otherwise normal. Of the following, the NEXT most appropriate action is to

A baby is born to a mother who is positive for hepatitis B surface antigen (HBsAg). Your plan is to do which of the following?

A vomiting infant is brought to the emergency room. The blood work results reveal a normal blood count, but a hyponatremic, hypochloremic, metabolic alkalosis. Which of the following would be consistent with these findings?

A 12-hour-old baby on the post-natal ward has just had a seizure lasting 2 minutes. It resolved spontaneously and was generalized in nature. Her mother had gestational diabetes and poor glucose control in pregnancy. The baby’s birth weight was 5 kg. There were no abnormalities noted on antenatal US scans or maternal serology. On examination she has no dysmorphic features and handles well. What initial blood tests would you do for the baby?

A 21-year-old woman has just delivered a term infant. She has had only one visit to her obstetrician, and that was at about 6 weeks of pregnancy. She provides her laboratory results from that visit. The delivered infant is microcephalic, has cataracts, a heart murmur, and hepatosplenomegaly. Your further evaluation of the child demonstrates thrombocytopenia, mild hemolytic anemia, and, on the echocardiogram, patent ductus arteriosus and peripheral pulmonary artery stenosis. Which of the following maternal laboratory tests done at 6 weeks gestation is likely to explain the findings in this child?

A 6-year-old boy with a history of asthma and eczema is brought in to accident and emergency from a local restaurant. He is on high flow facial oxygen with significant facial oedema and generalized erythema. On auscultation there is widespread wheeze for which the ambulance crew gave a salbutamol nebulizer. What is the next step in management?

A 1-month-old baby attends accident and emergency with a 2-day history of fever to 38.8°C measured at the GP surgery. He has been vomiting, with no diarrhoea, rash, cough or coryza. A clean catch urine has leukocytes +++ and ketones, no nitrites, blood or protein. An urgent microscopy shows >200 cells/ µL white cells. What is the most appropriate course of action?

A 9-month-old boy is taken to the emergency room because of high fever. Breath sounds are diminished in the lungs, and a chest x-ray film shows lobar pneumonia. Probable streptococcal pneumonia is demonstrated in Gram's stain of sputum and then later confirmed by culture. The child responds to antibiotic therapy. A detailed history is taken during the admission, which reveals that this is the third episode of pneumonia in this young child; the two previous episodes occurred at 6 and 7.5 months of age. One of the mother's brothers had died of infection at age 9. Immunoglobulin studies demonstrate the following: IgG 80 mg/dL [normal 723-1685 mg/dL], IgA 60 mg/dL [normal 81-463 mg/dL], IgM 20 mg/dL [normal 48-271 mg/dL]. Studies of the lymphocyte population demonstrate normal numbers of T cells and markedly decreased B cells. Which of the following is the most likely diagnosis?

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?

An otherwise healthy 5-year-old boy is brought to the emergency department of a small hospital because of a simple 3-cm laceration in his forehead. The patient is crying and frightened. The practitioner decides to perform conscious sedation before suturing the laceration. Support personnel and equipment are available for monitoring the patient's vital status and carrying out resuscitation measures if needed. Which of the following is the most appropriate pharmacologic agent to achieve a safe level of conscious sedation in this situation?

You are asked to see a 14-year-old girl who developed pubic hair at age 11 years and breast buds at age 12 years, but has not reached menarche. She is a gymnast who practices 2 hours a day. Breast tissue is Sexual Maturity Rating (SMR) 2 and pubic hair is SMR 4. She is 57 in tall and weighs 86 lb. The results of gonadotropin laboratory studies are a luteinizing hormone concentration of 18 mIU/mL (18 IU/L) (normal adult female, 2 to 70 mIU/mL [2 to 70 IU/L]) and a follicle-stimulating hormone concentration of 40 mIU/mL (40 IU/L) (normal adult female, 1 to 30 mIU/mL [1 to 30 IU/L]). Of the following, the MOST likely cause of the primary amenorrhea in this patient is

A 6-year-old girl has presented to her GP with a rapid increase in growth. Her mother is also concerned that she seems to have developed pubic and axillary hair and breast development prior to this but thought it would go away. She has no history of trauma and has reported problems with her vision. Her levels of gonadotrophin-releasing hormone (GnRH), follicle-stimulating hormone (FSH), luteinizing hormone (LH) and oestrogen are high. You are concerned that she may have a pituitary tumour. What is the likely visual field defect?

A very concerned mother brings a 2-year-old child to your office because of two episodes of a brief, shrill cry followed by a prolonged expiration and apnea. You have been following this child in your practice since birth and know the child to be a product of a normal pregnancy and delivery, to be growing and developing normally, and to have no chronic medical problems. The first episode occurred immediately after the mother refused to give the child some juice; the child became cyanotic, unconscious, and had generalized clonic jerks. A few moments later the child awakened and had no residual effects. The most recent episode (identical in nature) occurred at the grocery store when the child’s father refused to purchase a toy for her. Your physical examination reveals a delightful child without unexpected physical examination findings. Which of the following is the most likely diagnosis?

The mother and father of a newborn come in for the 2-week check-up. The mother complains of “colic” and asks if she can switch to goat’s milk instead of breast milk. Which of the following should be your main concern about using goat’s milk instead of breast milk or cow’s milk?

A 2-day-old male infant presents with multiple episodes of bilious vomiting over the past 24 hours. He has a prominent tongue, flat occiput and slanting eyes. His hands are short, and there are wide gaps between his first and second digits. His abdomen is soft and without any distention, guarding or rigidity. On auscultation of the chest, there is a systolic ejection murmur along the left sternal border, with a wide and fixed splitting of S2. Abdominal x- rays show air trapped in the first portion of the duodenum and stomach. What is the most likely cause of the child's symptoms?

A 24-month-old child is seen in the pediatrician's office for a regular health supervision visit. He has no history of developmental delay. He was born by an uncomplicated normal vaginal delivery at term, and he has not had any significant illness or injury prior to this visit. Which of the following motor milestones is most consistent with his age?

A 6-day-old female neonate is admitted to the hospital for the evaluation of jaundice. She was born to a 17-year-old German mother at 39 weeks gestation. Her mother's blood type is B+, and was treated for newborn jaundice due to presumed ABO incompatibility. Her other family members also had neonatal jaundice. The infant's vital signs are normal. On examination, she is visibly jaundiced, and her spleen tip is palpable. Her total bilirubin level is 25mg/dl and direct bilirubin level is 0.4mg/dl. Her hemoglobin was initially 15.7g/dl, but is now 10.7g/dl. Her reticulocyte count is 4% and platelet count is 230,000/mm3. The peripheral smear shows moderate schistocytes with burr cells and moderate spherocytes. Her blood type is B+ and Coombs' test is negative. Her stools are negative for occult blood. Her mother's incubated red cell osmotic fragility study is abnormal. What is the most likely cause of this neonate's symptoms?

A 7-year-old child is scheduled for an elective tonsillectomy. The most important instruction to the parents should be to make sure that the child does which of the following?

A 16-year-old girl presents with lower abdominal pain and fever. On physical examination, a tender adnexal mass is felt. Further questioning in private reveals the following: she has a new sexual partner; her periods are irregular; she has a vaginal discharge. Which of the following is the most likely diagnosis?

Friends are considering adopting a “special needs” child from another country. The family has few details, but the information they have received so far suggests the 4-year-old child has had surgery for an endocardial cushion defect, is short for his age, and had a history of what sounds like surgically repaired duodenal atresia at birth. You are suspicious this child may have which of the following syndromes?

You are admitting to the hospital a 3-month-old infant who has been having poor feeding, emesis, and diarrhea for 3 days. In the emergency center, her electrolytes were found to be: sodium 157 mEq/L, potassium 2.6 mEq/L, chloride120 mEq/L, bicarbonate 14 mEq/L,creatinine1.8 mEq/L, blood urea nitrogen(BUN) 68 mEq/L, and glucose195 mEq/L. She was given a fluid bolus in the emergency center and has subsequently produced urine. Which of the following is the most appropriate next step in her management?

You are called to see a hospitalized 9-year-old girl who suddenly has become dystonic, with her neck hyperextended, and is unable to move her eyes, now superiorly deviated. The nurses relate that this girl has non-Hodgkin lymphoma and has been receiving highly emetogenic chemotherapy. Of the following, the drug MOST likely to have caused this girl's symptoms and signs is

A newborn infant is brought to the nursery for evaluation after delivery. The mother reports that the pregnancy was uncomplicated, but she had only two prenatal visits. The infant was born via normal spontaneous vaginal delivery and required no resuscitation. The infant has hepatosplenomegaly on examination. While in the hospital, the infant requires treatment for anemia and hyperbilirubinemia. On subsequent examinations, the infant has clear rhinorrhea and ulcerative lesions on his feet. Which of the following congenital infections is most likely in this patient?

A 3 year old male has sudden onset of dry cough with a small amount of bright red blood produced when he coughs. He has had no fevers, runny nose, or vomiting. In the Emergency Department a chest x-ray shows hyperexpansion of the right lung and clear lung fields. Which of the following is the most likely etiology of this child's symptoms?

A 12-year-old child is struck in the right eye by a baseball. He now complains of double vision when he looks to the left. On physical examination, you note marked swelling and bruising circumferentially around the boy's right eye. His pupillary reflexes are normal, he has no blood in the anterior chamber of the eye, and his optic disc margins are sharp. He has dysconjugate gaze to the left but not to the right. His vision is 20/20 out of the left eye and 20/30 out of the right eye. Other findings on his neurologic examination are normal. Of the following, the MOST likely diagnosis is

A 6-year-old girl presents with a 1-year history of periumbilical, nonradiating abdominal pain. The pain occurs at least three times per week and lasts up to 30 minutes. There is no history of heartburn, constipation, or diarrhea. Physical examination, complete blood count, erythrocyte sedimentation rate, and urinalysis yield normal results. A Helicobacter pylori serology (immunoglobulin G antibody) is positive. Of the following, a TRUE statement regarding this patient is that:

A 2-year-old boy who emigrated from Eastern Europe 1 year ago is brought to the physician because of fever, cough, and night sweats for 3 weeks. The child's grandmother, who lives with him, has similar symptoms. The child's temperature is 39.2 C (102.6 F), Wood pressure is 110/65 mm Hg, pulse is 90/min, and respirations are 28/min. A Mantoux test is reactive, and a chest x-ray film shows a right middle lobe infiltrate and hilar lymphadenopathy. Which of the following is the most appropriate next step in diagnosis?

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 running 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

A 5-month-old boy is brought to the office for a mass in his left groin area. The infant is on the examination table, quietly sucking on his pacifier. On examination, there is a fluid-filled sac that does not reach the inguinal ring and transilluminates well. Which of the following is the most likely diagnosis?

A premature neonate with respiratory distress syndrome is maintained on mechanical ventilation in a neonatal intensive care unit. Two weeks after delivery, the nurses in the intensive care unit notice that higher ventilation settings are needed and that more secretions are being suctioned from the endotracheal tube. A chest x-ray film shows questionable new infiltrates. Which of the following is the most likely pathogen?

You are evaluating a 13-year-old boy who has a body mass index of 32 kg/m2 and a blood pressure of 140/80 mm Hg. Initial laboratory studies reveal fasting serum triglyceride levels of 180 mg/dL (2.0 mmol/L) (normal, 40 to 100 mg/dL [0.45 to 1.1 mmol/L]). You diagnose metabolic syndrome. Of the following, the MOST appropriate evaluation in addition to fasting blood glucose is

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 90% 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 findings are normal. Of the following, the pathogen that is MOST likely responsible for his symptoms is

A 42-year-old primigravida is in her 16th week of pregnancy. She is concerned about the possibility of her child having myelomeningocele. Of the following, the MOST useful diagnostic evaluation is

A newborn female has loose neck skin and nonpitting edema of the lower extremities. Of the following, the MOST appropriate evaluation for this infant is

A 4-year-old boy, who has a ventriculoperitoneal shunt for congenital hydrocephalus, develops fever, headache, irritability, lethargy, photophobia, and vomiting. His temperature is 39.6 C (103.2 F). He is noted to have nuchal rigidity, with the presence of both Kernig's and Brudzinski's signs. The shunt tract is erythematous on the surface. A lumbar puncture is performed and shows a WBC of 40,000/mm3 with 85% neutrophils, a glucose concentration of 48 mg/dL, and a protein concentration of 169 mg/dL. Which of the following is the most likely pathogen?

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?

An 8-year-old boy presents to the pediatrician's office with a headache for the past 3 weeks. His mother also states that he has been more tired and has had frequent nose bleeding for the past month. On physical examination, his height and weight are both below the 5th percentile for his age. His blood pressure is 152/86 mm Hg in all four extremities. His pulse is 74/min, and respirations are 16/min. His heart examination is normal with no murmur. His peripheral pulses are strong and symmetric. Urinalysis and serum electrolytes are ordered. Which of the following is the most appropriate next step in diagnosis?

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?

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?

A 5-day-old male term neonate has been passing maroon-colored stools for the past three days, along with frequent episodes of vomiting. He is being breastfed by his mother, who is an asthmatic. His vital signs are within normal limits. The physical examination is unremarkable. Stool examination is positive for RBCs and eosinophils. What is the most likely cause of this patient's findings?

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

An 11-year-old girl is brought to the office by her mother due to headaches for the last 4 hours. The headaches are bifrontal, and are accompanied by nausea, vomiting, and sensitivity to light and noise. It is her second episode, and both episodes were preceded by dark spots in her visual field. The first episode was 2 weeks ago. She doesn't report any numbness or tingling in her extremities, and no behavioral change or decline in school performance was noted. Her vital signs are stable, and she is afebrile. The physical examination is normal. What is the most appropriate next step in the management of this patient?

An anxious 16-year-old girl presents to the emergency department complaining of a sudden onset of feeling that she is unable to breathe. She also complains of tingling around her lips and fingertips. Upon further questioning, you learn that she has had frequent similar episodes in the past. She has no history of asthma, and she has no chest pain or fever. Physical examination reveals a respiratory rate of 30 to 40 breaths/min, oxygen saturation on room air of 99%, no intercostal retractions, and no fever. No wheezing or crackles are evident on chest auscultation; breath sounds are good bilaterally. The balance of findings on the physical examination, including a complete neurologic evaluation, are normal. Of the following, the symptom that BEST distinguishes the correct diagnosis from compensatory hyperventilation is

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

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

A 14-year-old girl was seen in accident and emergency following her third collapse this year and referred to cardiology for review of a low rumbling murmur heard at the left upper sternal edge. Her ECG in accident and emergency was normal. Her blood sugar was 5.3 mmol/L. Urea and electrolytes were normal. The most recent collapse occurred at school while waiting for exam results to be given out. Previously they occurred while watching a parade all afternoon standing in a crowded street, and at a party. On all three occasions she felt dizzy beforehand, was unconscious for less than 10 seconds and fully alert following the episode, but did feel nauseous. Her echocardiogram today is normal. What is the most likely diagnosis?

A pediatrician examines a 2-month-old infant who had been born at term. The pediatrician hears a continuous murmur at the upper left sternal border. The peripheral pulses in all extremities are full and show widened pulse pressure. Which of the following is the most likely diagnosis?

A 12-year-old girl has had a sore throat over 2 days. She now has a fever of 39.5°C and has difficulty opening her mouth, swallowing, or speaking. Her throat can be visualized with difficulty, the right tonsil is significantly more enlarged than the left, and the uvula is displaced to the left side. Which of the following is the most likely diagnosis?

A healthy 1-year-old child comes to your office for a routine checkup and for immunizations. His parents have no complaints or concerns. The next day, the CBC you performed as customary screening for anemia returns with the percentage of eosinophils on the differential to be 30%. Which of the following is the most likely explanation?

A 22-year-old woman has just delivered a male infant at 41 weeks of gestation. Her medical history is normal and her pregnancy was uncomplicated. She is an assistant in a veterinary clinic. Examination of the infant reveals jaundice, hepatosplenomegaly, and generalized lymphadenopathy. During the examination, he suddenly begins to have tonic-clonic seizures. The CT scan reveals active inflammatory lesions, hydrocephalus and intracranial calcifications. What is the most likely diagnosis of this patient?

A 16-year-old girl comes to the physician's office because she has not begun menstruating yet. Both her mother and an older sister started menstruation at age 12. She takes no medication and denies strenuous exercise or excessive dieting. Her height is at the 50fh percentile for age; her weight is at the 60fh percentile. Both her breast and pubic hair development are at Tanner stage 4. Pelvic ultrasonography reveals a normal uterus and ovaries. Which of the following is the most likely diagnosis?

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

A 5-year-old boy is brought to the emergency department by his mother. One week ago, he developed a low grade fever followed by persistent bouts of coughing that last 20-30 minutes each. The coughing spells are severe, unresponsive to antitussive medications, and appear to worsen with eating and drinking cold liquids. On physical examination, there is subconjunctival hemorrhage and extensive subcutaneous emphysema over the anterior chest. What is the most likely organism responsible for this child's illness?

A 12-year-old girl with a history of asthma has been admitted to intensive care units two times in the past and has had three emergency department visits during the past 12 months. Her only medication is inhaled albuterol as needed, and she uses it two to three times a day. She has nocturnal symptoms about two times a week. She is free of symptoms now but reports that she gets short of breath easily. Which of the following pharmacologic interventions is most appropriate?

A 17-year-old girl presents to the office with a 5-day history of a malodorous vaginal discharge. She is sexually active and uses condoms for sexual intercourse. On examination, a thin, white discharge is seen. A "fishy" odor is produced when KOH is added to the discharge. The vaginal fluid has a pH of 5. Which of the following is the most likely finding on a microscopic examination of the vaginal fluid?

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?

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?

A 16-year-old female presents to the emergency room with severe right- sided abdominal pain. Her last menstrual period was 2 weeks ago and felt to be normal. She displays painful difficulty in positioning herself on the examination table. Her temperature is 38.5 C (101.3 F), blood pressure is 128/75 mm Hg, pulse is 80/min, and respirations are 22/min. Examination of the throat reveals mild pharyngitis. Her abdomen is diffusely tender, especially the lower abdomen. Rectal examination reveals tenderness anteriorly on the right side. Stool guaiac is negative. A pelvic examination is performed, and there is evidence of cervical tenderness and questionable fullness in the right adnexal area. Which of the following is the most likely diagnosis?

A 3-day-old newborn is ready to be discharged from the neonatal nursery, when his mother reports that a rash has appeared on his abdomen and chest. He otherwise appears healthy. He has no fever and no risk factors for infection. Examination reveals a nontoxic neonate with erythematous papules and vesicles surrounded by patches of erythema. Microscopic evaluation of the pustules reveals numerous eosinophils. What is the most likely diagnosis?

A 4-year-old boy falls from the jungle gym at preschool. He sustains minor abrasions and contusions, and is taken care of by the school nurse. His parents take him that same afternoon to his regular pediatrician and demand "a thorough check-up" for possible internal injuries. The pediatrician complies, and a complete physical examination is normal. His hemoglobin is 14 g/dL, and a urinalysis shows the presence of microhematuria. Which of the following is the most appropriate next step in management?

A 3-year-old child is rushed to the emergency department after the mother found her with an open and empty bottle of acetaminophen. The mother has no idea how many tablets were in the bottle. She estimates that no more than 1 hour has passed since the child ingested the tablets. The child began to vomit during the trip to the emergency department, and has vomited three times more since her arrival. The child is awake and alert but clearly unhappy, crying even in her mother's arms. She appears pale and diaphoretic. Her heart rate is 110 beats/min, respiratory rate is 26 breaths/min, temperature is 98.6°F (37°C), and blood pressure is 90/60 mm Hg. Of the following, the MOST appropriate statement about acetaminophen toxicity is that:

A 15-month-old boy presents to the emergency department following the acute onset of nausea, vomiting, and abdominal pain. He appears agitated. His heart rate is 160 beats/min, but other vital signs are normal. His mother, who has asthma, reports finding her theophylline bottle lying empty on the floor in her bedroom. His capillary blood gas measurement is normal, and his blood glucose concentration is 190 mg/dL (10.5 mmol/L). Of the following, the MOST appropriate management of this patient is

A 5-year-old African-American boy is brought to the office by his mother due to a painful right knee, fever and chills over the last 2 days. The child was diagnosed with pharyngitis 2 weeks ago, and was treated with a 10-day course of penicillin G. His temperature is 38.8 C (102F) and pulse rate is 102/min. On examination, his right knee joint is swollen and erythematous, with a decreased range of motion. The laboratory findings are significant for leukocytosis and an elevated ESR. X-rays of both the right hip and knee joints are normal. What is the most appropriate treatment for the patient's condition?

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?

A 10-day-old infant is brought to the office by her mother for the evaluation of purulent discharge from both eyes. The discharge appeared three days ago, after which the child developed a cough. On examination, the infant's eyes appear normal, except for the presence of purulent discharge. Auscultation reveals diffuse rales without wheezing. Chest radiograph shows a hyperinflated thorax. What is the most likely diagnosis?

A full-term newborn develops cyanosis a few hours after birth. Oxygen administration does not improve color or oxygen saturations. Which of the following is the most likely diagnosis?

A 16-year-old girl is being evaluated for primary amenorrhea. Her birth history is significant for low birth weight and bilateral swelling of the hands and feet. Her blood pressure is 170/96 mmHg. She has a short stature and webbed neck. Her karyotype examination results show 22 pairs of autosomes, one X, and one Y chromosome. Which of the following statements is true regarding this patient's condition?

A 6-year-old boy presents in clinic for a routine visit. Examination reveals coarse, dark pubic hair, an enlarged penis and testes, and acne of the face and upper back. His mother notes that he has a body odor similar to that of her teenage son after playing sports. The child is in file 99th percentile of height for his age group. Which of the following is the most likely diagnosis?

A 4-year-old boy was diagnosed with nephrotic syndrome 6 months ago and has required a long course of oral corticosteroids to maintain remission of the condition. He has developed truncal obesity and you are concerned he may be developing Cushing’s syndrome. Which of the following is not a complication of Cushing’s syndrome?

A mother brings her daughter into the clinic for a routine health-care visit. On examination, you note that she walks alone, knows two words, throws objects, and comes when called. What is the most likely age of this infant?

A 2-month-old infant is evaluated by a pediatric cardiologist. The infant was noted at birth to have an upper left sternal border ejection murmur. The infant at that time was not cyanotic, but slowly developed cyanosis over the next two months. At the time of the pediatric cardiologist's examination, an ECG showed right axis deviation and right ventricular hypertrophy. A chest x-ray film showed a small heart with a concave main pulmonary artery segment and diminished pulmonary blood flow. Which of the following is the most likely diagnosis?

A 15-year-old girl comes to accident and emergency complaining of sudden right arm weakness and double vision. Last week she was incontinent of urine twice. She is normally fit and well. On examination she has a left-sided 6th nerve palsy and four out of five power in her right arm. The examination is otherwise unremarkable. An MRI head shows multiple hyperintense, inflammatory, white matter lesions. What is the most likely diagnosis?

A 10-year-old presents with 2 months history of heavy menstrual-like bleeding. Menarche occurred 6 months ago and this first menses consisted of spotting for 3-4 days without cramps. Subsequent periods were light in flow but lasted 6 to 8 days. Which of the following is the most likely cause of her bleeding?

A mother with known placenta praevia with heavy vaginal bleeding was rushed into the labour ward and delivered by emergency caesarean section at 35 weeks’ gestation. Pre-delivery the fetus was bradycardic and after birth APGARs were three at 1 minute, five at 5 minutes and nine at 10 minutes. Thirty-six hours later on the special care baby unit the baby is irritable and requiring nasogastric tube feeds as he is not sucking well. The tone in his upper limbs is reduced and an EEG showed seizure activity which has been controlled by intravenous phenobarbitone. His cranial ultrasound is normal. His blood sugar monitoring is between 3.5 and 5 mmol/L, C-reactive protein (CRP) was less than 5 mg/L and is 7 mg/L today. He is apyrexial. What is the most likely diagnosis?

An infant born at term to a 22-year-old woman has a weak cry and is cyanotic at birth. His fingertips and oral mucosa appear blue. His blood pressure is 80/40 mmHg and his heart rate is 140/min. Chest x-ray shows clear lung fields bilaterally. PaO2 measured by arterial cannulation is 38 mmHg, and improves only minimally on 100% inspired oxygen. Which of the following is the best next step in managing this patient?

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?

An 8-year-old boy is brought to his physician by his mother, who is worried by the child's frequent episodes of daydreaming, which have apparently resulted in a decline in school performance. The child's psychomotor development appears normal. EEG recording reveals bilateral and symmetric 3 Hz spike-and-wave discharges, which begin and end abruptly on a normal background. Which of the following is the most likely diagnosis?

You receive a telephone call from the mother of a 4-year-old child with sickle-cell anemia. She tells you that the child is breathing fast, coughing, and has a temperature of 40°C (104°F). Which of the following is the most conservative, prudent course of action?

A 6-year-old girl presents to hospital with a large right-sided abdominal mass. It does not cross the midline. On further questioning she has had macroscopic haematuria and weight loss of 4kg over the last 4 months. She has reduced appetite and lethargy. Her blood pressure is 125/73 mmHg, heart rate 120 bpm. Which of the following is not a complication of this malignancy?

A 4-week-old boy has been vomiting all of his feedings for the last 2 days. His mother reports decreased urine output but no fever. On physical examination, he is alert, appears hungry, and is moderately dehydrated. Abdominal examination reveals a small mass in the epigastrium. When he is offered a bottle, he takes it vigorously and then vomits forcefully. You order laboratory tests. Of the following, the MOST likely abnormality to expect is

Over the past week, you and your colleagues have seen a number of previously healthy children younger than 2 years of age who present with fever, coryza, cough, vomiting, and diarrhea. Several of the children have required hospitalization for respiratory distress and pneumonia. You suspect that influenza virus infection is the cause of these illnesses. Of the following, the MOST rapid test to confirm the diagnosis in these patients is

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?

A 5-day-old baby is brought to see the GP because she has had a rash for the past 3 days which started on her chest, is spreading to her face and getting worse. On examination she handles well and is alert. There is an erythematous rash on her face, torso and right arm with little pustules. What is the most likely diagnosis?

A 3-week-old female is brought into the emergency department with a fever and irritability. She was born after a normal pregnancy and delivery. Her mother had routine prenatal care and has no history of sexually transmitted infections. The infant's mother is 14 years old and the father is 17 years old. They are not married, and the father is not involved in the care of the infant. The infant lives with her mother and maternal grandparents at the maternal grandparents' home. You are concerned about meningitis and decide to do a lumbar puncture. The mother and maternal grandparents are present in the emergency department. Informed consent should be obtained from which of the following individuals?

A 2-year-old child is brought to the office by his parents for the evaluation of failure to thrive and chronic diarrhea. His diarrhea is "almost continuous", and he also has recurrent episodes of fever. He was initially quite well and was on the 60th percentile for weight; however, for the past seven months, he didn't seem to grow and failed to gain weight. Over the past three months, he fell to the 3rd percentile for weight. His parents deny any use of drugs, but they drink occasionally. His mother mentions that her former boyfriend was a heroin addict. Physical examination reveals oral thrush, generalized lymphadenopathy and eczema. What is the most likely diagnosis in this patient?