MCQ Pediatric 4

"A 1-week-old infant presents to her general pediatrician's office for a well-child visit. She was born at 37 weeks' gestation without complications. Her temperature is 37.0°C 98.6°F, pulse is 130/min, blood pressure is 72/54 mmHg, and respiratory rate is 28/min. She is currently at the 50th percentile for weight and 75th percentile for height. She is acyanotic and has a wide, fixed split S2, with a 2/6 systolic ejection murmur at the left upper sternal border. The remainder of the examination is unremarkable. Which of the following is the most likely diagnosis? A. Atrial septal defect B. Coarctation of the aorta C. Dextratransposition of the great arteries D. tetralogy of Fallot E. ventricular septal defect "

A

"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. Cephalohematoma B. Caput succedaneum C. Cranial meningocele D. Intracranial hemorrhage E. Depressed skull fracture "

A

"A 10-year-old boy has been having "bellyaches" for about 2 years. They occur at night as well as during the day. Occasionally, he vomits after the onset of pain. Occult blood has been found in his stool. His father also gets frequent, nonspecific stomachaches. Which of the following is the most likely diagnosis? A . Peptic ulcer B . Appendicitis C . Meckel diverticulum D . Functional abdominal pain E . Pinworm infestation "

A

"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. Acute lymphoblastic leukemia B. Hodgkin's disease C. Acute myeloid leukemia D. Aplastic anemia E. Immune thrombocytopenic purpura "

A

"A 10-year-old child arrives with the complaint of new-onset bed-wetting. He has had no fever, his urine culture is negative, and he has had no new stresses in his life. He is well above the 95th percentile for weight as is much of his family. Which of the following is most helpful in making a diagnosis? A. Fasting plasma glucose of 135 mg/dL B. Random plasma glucose of 170 mg/dL C. Two-hour glucose during glucose tolerance test of 165 mg/dL D. Acanthosis nigricans on the neck E. Symptoms alone are enough to make the diagnosis"

A

"A 2-month-old boy is examined because he has been straining while passing stool and has a distended abdomen. He is very low on the growth chart for age. The primary care physician suspects that the boy has Hirschsprung disease. Which of the following findings on workup is diagnostic? A. Absence of ganglion cells on full-thickness rectal biopsy 2 cm above the dentate line B. Absence of ganglion cells on full-thickness rectal biopsy 1 cm above the dentate line C. Absence of ganglion cells on suction rectal biopsy 1 cm above the dentate line D. Identification of a transition zone between the sigmoid colon and the distal rectum on barium enema E. Inhibition of the resting anal inhibitory reflex on anorectal manometry "

A

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

A

"A 3-year-old girl with a ventricular septal defect VSD. presents to the emergency department after a 15-minute focal seizure of her left arm and leg. A brief history reveals that the child has no known seizure disorder and has been having a low-grade fever at home for about 4 days. She also has been less active and has had poor appetite. On physical examination, her temperature is 40.2 C 104.3 F, and her pulse is 82/min. She is not responsive to her name, but she is responsive to painful stimuli with withdrawal of her extremities. Cardiac examination is significant for a grade 3 systolic murmur best heard at the left lower sternal border. Neurologic examination reveals anisocoria with a dilated right pupil. After stabilization, which of the following is the most appropriate next step in diagnosis? A. CT of the brain B. ECG C. Electroencephalography D. MRI of the brain E. Complete blood count and blood culture"

A

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

A

"A 5-month-old infant is brought to the office for the evaluation of persistent vomiting, failure to thrive, and developmental delay. His antenatal and postnatal histories are not known to his Caucasian foster parents, who adopted him when he was 4 months old. The physical examination reveals an infant with blonde hair, fair skin and blue eyes. His urine has a peculiar musty odor. His plasma phenylalanine level is 40 mg/dl and tyrosine level is normal. His urinary phenylpyruvic and a-hydroxy phenylacetic acid levels are both increased. What is the most likely etiology of this child's symptoms? A. Classic phenylketonuria B. Benign hyperphenylalaninemia C. Transient hyperphenylalaninemia D. Tyrosinemia E. Alcaptonuria "

A

"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 . Developmental defect of the third/fourth pharyngeal pouches B . In utero infection by human immunodeficiency virus HIV. C . Mutations of an autosomal gene encoding adenosine deaminase D . Mutations of an X-linked gene coding for a cytokine receptor subunit E . Mutations of an X-linked gene coding for a tyrosine kinase "

A

"A 6-month-old male is brought to the office due to fussiness and tugging at his right ear for the past 2 days. He has had a fever of 39.4 C 103 F for the past 2 days. His past medical history is significant for recurrent ear and lung infections, oral candidiasis, and persistent diarrhea by rotavirus. His temperature is 39.4 C 103F., pulse rate is 150/min, respirations are 28/min, and blood pressure is 80/60mm Hg. Physical examination reveals an erythematous, bulging right tympanic membrane with poor mobility on pneumatic otoscopy. His lymph nodes are not palpable, and his tonsils are not visualized. His B and T lymphocyte levels are markedly reduced. The chest x-ray reveals an absent thymic shadow. What is the most likely etiology of this patient's condition? A. severe combined immune deficiency B. Common variable immunodeficiency C. Bruton's agammaglobulinemia D. Wiskott-Aidrich syndrome E. Chronic granulomatous disease "

A

"A 6-year-old Asian boy is brought by his parents to the office due to high-grade fever and rash for the last 9 days. A brick-red, maculopapular rash first appeared on his face and subsequently spread to his trunk and extremities. Prior to the outbreak of the rash, he had a non-productive cough, tearing of eyes, runny nose, sneezing, and intermittent nasal obstruction. Laboratory findings are as follows: Hct 46%, WBC 2,000/mm3, Platelets 160,000/mm3. Which of the following has been shown to reduce the morbidity and mortality rates of patients with this kind of infection? A. Vitamin A B. Vitamin B 6 C.Vitamin B 12 D. Vitamin E E.Vitamin K "

A

"A 6-year-old boy had been in his normal state of good health until a few hours prior to presentation to the ER room. His mother reports that he began to have difficulty walking, and she noticed that he was falling and unable to maintain his balance. Which of the following is the most likely cause for his condition? A. Drug intoxication B . Agenesis of the corpus callosum C . Ataxia telangiectasia D . Muscular dystrophy E . Friedreich ataxia "

A

"A 1-day-old normal-appearing infant develops tetany and convulsions. He was born at 34 weeks' gestation with Apgar scores of 2 and 4 at 1 and 5 minutes, respectively. to a woman whose pregnancy was complicated by diabetes mellitus and pregnancy-induced hypertension. Which of the following serum chemistry values is likely to be the explanation for his condition? A. serum bicarbonate level of 22 mEq/dL B. serum calcium of 6.2 mg/dL C. serum glucose of 45 mg/dL D. serum magnesium level of 5.0 mg/dL E. Intracranial hemorrhage"

B

"A 1-year-old boy is brought to the clinic by his 28-year-oldmother for the evaluation of his eyes. For the past several months, he has been bumping into objects. His perinatal history is unremarkable. Physical examination of the eyes reveals a bilateral white reflex. The retina cannot be visualized properly. Fundal reflection is absent, and the pupil is white. What is the most likely diagnosis? A . Congenital glaucoma B . Congenital cataract C . retinoblastoma D . Pterygium E . glaucoma "

B

"A 1-year-old boy is brought to the pediatrician for a routine visit. While talking to his mother, she reports that they moved into an old house several months ago and are in the process of renovating. The child eats table food and drinks whole milk. His height, weight, and head circumference are all at the 50th percentile for his age. Physical examination is normal. The results of a fingerstick blood test are shown below: Hemoglobin 10.5 g/dL, Hematocrit 30.0%, lead level 12 μg/dL Normal < 10 μg/dL.. Which of the following is the most appropriate next step in management? A. remove the child from the house B. Check a serum lead level C. Initiate chelation therapy with dimercaprol D. Initiate chelation therapy with dimercaptosuccinic acid DMSA, succimer. E. recheck lead level in one month "

B

"A 10-year old girl is brought to the office by her mother for the evaluation of recent changes in behavior. She has been sleeping poorly at night and has started wetting her bed. Her school grades have dropped significantly, and she has become irritable and cranky. She refuses to sleep at night until her father returns home and goes to bed. Her father works as a taxi driver, and is an alcoholic. Her mother is a close friend of yours, and appears very concerned. Prior to this office visit, you have known this girl to be cheerful and lively; however, as you attempt to talk to the young girl in the office, she suddenly bursts into tears. Which of the following should you consider at this point? A . Major depression with melancholic features B . Physical abuse C . Anxiety disorder D . Panic disorder E . Specific phobia "

B

"A 10-year-old boy has a long history of recurrent infections. These have included pneumonia, suppurative lymphadenitis, persistent rhinitis, dermatitis, diarrhea, and perianal abscesses. Involved organisms have included Staphylococcus aureus, serratia, Escherichia coli, and Pseudomonas. Biopsy of skin and lymph nodes have demonstrated granulomatous lesions, even though the only species isolated were those noted above. Immunoglobulin levels are higher than normal. Which of the following findings would be most helpful in establishing the diagnosis? A. Absent B cells and normal numbers of T cells B. Deficient nitroblue tetrazolium dye reduction in neutrophils C. High serum IgM and very low serum IgG D. very low CD11 on the surface of white blood cells E. very low serum calcium levels"

B

"A 10-year-old boy is brought to the emergency department due to abdominal pain and bloody diarrhea. The mother says that he was ""fine"" a few days ago, and then suddenly became ill. Physical examination shows a pale and jaundiced child. There is diffuse abdominal tenderness and 2+ pedal edema. Laboratory studies show anemia, thrombocytopenia and renal insufficiency. What is the most likely cause of the patient's symptoms? A. Vibrio cholera B. Escherichia coli C. Crohn's disease D. Lactose intolerance E. Salmonella poisoning "

B

"A 10-year-old boy, the star pitcher for the Salt Lake City Little league baseball team, had a sore throat about 2 weeks ago but did not tell anyone because he was afraid he would miss the play-offs. Since several children have been diagnosed with rheumatic fever in the area, his mother is worried that he may be at risk as well. You tell her that several criteria must be met to make the diagnosis but the most common finding is which of the following? A. Carditis B. Arthralgia C. Erythema marginatum D. Chorea E. Subcutaneous nodules "

B

"A 12-year-old boy is in a motor vehicle collision in which the car caught fire. He sustains significant inhalation injury and a circumferential burn without fractures or other soft tissue trauma to his left lower extremity during extrication from the burning vehicle. He is intubated and aggressively resuscitated in the intensive care unit. Which of the following is the most appropriate method of assessing for compartment syndrome of the left lower extremity? A. X-ray of the left lower extremity B. Doppler signals of the left lower extremity C. Computed tomography CT. scan of the left lower extremity D. Magnetic resonance imaging MRI. of the left lower extremity E. left lower extremity angiogram"

B

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

B

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

B

"A 2-year-old boy is brought to the emergency department ED. in acute respiratory distress. He is afebrile, and has a heart rate of 100/min, respiratory rate of 80/min, and Sa02 of 84% on room air. He is sitting upright, and has significant nasal flaring and intercostal retractions. He is given supplemental oxygen in the ED. Chest x-ray reveal hyperinflation of the right lung, mediastinal shift to the left, and a severely hypoinflated left lung Which of the following is the most appropriate next step in management? A. Bronchodilator therapy B. Direct laryngoscopy and rigid bronchoscopy C. Direct laryngoscopy and flexible bronchoscopy D. segmental lung resection E. CT scan of the chest "

B

"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. Cervical lymph node biopsy B. Gastric aspiration C. Pleurocentesis D. Sputum induction E. Gastroscopy "

B

"A 2-year-old child is brought to the physician for a routine visit. He is growing and developing appropriately. He drinks 3-4 glasses of whole milk each day. He is starting to put words together into short sentences. His mother has no concerns. Physical examination shows mild pallor. Laboratory studies show the following: Hemoglobin 9.5 g/dL, RDW 21%, MCV 70 fl, Platelet count 284,000/mm3, leukocyte count 6,500/mm3. Which of the following additional findings is most likely in this patient? A. Abnormal hemoglobin electrophoresis B. Low reticulocyte count C. Low serum total iron binding capacity D. High indirect bilirubin E. Positive fecal occult blood test "

B

"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. Barium enema B. Gastrografin enema C. Colonoscopy D. Endoscopic retrograde chokngiopancreatogram ERCP E. Full thickness rectal biopsy"

B

"A 3-month old infant is brought to a pediatrician's office because of increased lethargy and irritability. The parents state that the child rolled off the couch and fell on the floor one day prior to presentation. His parents report that the child has been previously healthy and is up to date on his vaccinations. He has been meeting his development milestones. His fontanelles are full. While in office the patient develops a tonic-clonic seizure. Which of the following is the next appropriate step? A. Obtain a head computerized tomography scan B. Perform a retinoscopic examination C. Check serum levels of ammonia D. Administer intravenous benzodiazepines E. Perform a lumbar puncture"

B

"A 3-year-old child presents to your office for an evaluation of constipation. The mother notes that since birth, and despite frequent use of stool softeners, the child has only about one stool per week. He does not have fecal soiling or diarrhea. He was born at term and without pregnancy complications. The child stayed an extra day in the hospital at birth because he did not pass stool for 48 hours, but has not been in the hospital since. Initial evaluation of this child should include which of the following? A. A child psychiatry evaluation for stool retention and parenting assistance B. A barium enema and rectal manometry C. Plain films of the abdomen D. Dietary log and observation E. Beginning oral antispasmodic medication"

B

"A 3-year-oldboy is brought to the emergency department with sudden onset of difficulty walking. His mother reports that his right hand also seems ""clumsy."" The boy's past medical history is significant for a hospitalization one year ago for severe upper extremity pain and hand swelling. On physical examination, he has a blood pressure of 90/60 mmHg, heart rate of 120/min, temperature of 36.7°C 98°F, and respiratory rate of 22/min. Which of the following would be most helpful in diagnosing his condition? A. Carotid ultrasonography B. CBC and reticulocyte count C. Antineutrophil cytoplasmic antibodies D. temporal artery biopsy E. Lumbar puncture"

B

"A 30-hour-old infant has not passed meconium since birth. He was full term with a birth weight of 3856 g 8 lb 8 oz. The pregnancy was uncomplicated. The baby appears well with no respiratory distress. Slight abdominal distention is noted. rectal examination reveals a slightly tight rectum and results in a greenish gush of stool. Which of the following tests will probably confirm the likely diagnosis? A. A stool culture B. A rectal biopsy C. A barium enema D. An alpha1-antitrypsin level E. A serum TSH level "

B

"A 4-year-old child comes in for a health maintenance visit. His mother is concerned that he is not doing some things that other kids in his preschool do. Which of the following skills would be expected of a 4-year-old? A . Building a 10-cube staircase B . Drawing a square C . Drawing a triangle D . Drawing a person with six parts E . Repeating five digits "

B

"A 4-year-old girl is brought to the physician because of a crusted honey-colored erythema resulting from rupture of tiny vesicles and pustules. Her temperature is 37.7 C 102 F. Skin lesions are distributed over the face and extremities. Physical examination reveals enlargement of lymph nodes in the cervical and axillary regions. Which of the following is the most frequent pathogen of this skin infection? A. Human herpes virus 7 B . Staphylococcus aureus C . Streptococcus pyogenes D . Propionibacterium acnes E . Trychophyton fungi "

B

"A 4week-old male infant is brought to the office due to several episodes of projectile vomiting for the last few days. The vomitus contains milk and doesn't contain bile or blood. The child's appetite has increased for the last few days. He has been fed with goat's milk since birth, but doesn't seem to tolerate it anymore for the last few days. He vomits a few minutes after feedings. He appears dehydrated, and abdominal examination reveals no mass. blood tests reveal macrocytosis. What is the most appropriate next step in the management of this patient? A. Barium swallowing B. Ultrasound of the abdomen C. Substitute goat's milk with another form of milk D. Divide his feedings E. Add folic acid to relieve his vomiting"

B

"A 5-year-old pedestrian is hit by a car in a mall parking lot and he is brought to the emergency department. There was loss of consciousness for less than 1 minute. On evaluation, the child has no neurologic deficits and a CT scan of the head reveals no intracranial abnormalities and no obvious skull fractures. The parents want to know what possible long-term problems there might be. You remember that problems after head trauma may include the development of seizures and that the risk of developing posttraumatic epilepsy is increased by which of the following? A. a brief loss of consciousness B. an acute intracranial hemorrhage C. retrograde amnesia D. posttraumatic vomiting E. a small linear skull fracture "

B

"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 alsohad 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. Normal physiologic changes occurring in red cells in the neonatal period B. Hereditary spherocytosis C. Hereditary elliptocytosis D. Autoimmune hemolytic anemia due to warm antibodies E. Isoimmune hemolytic disease of the newborn, due to ABO incompatibility "

B

"A 1-day-old infant who was born by a difficult forceps delivery is alert and active. She does not move her left arm spontaneously or during a Moro reflex. Rather, she prefers to maintain it internally rotated by her side with the forearm extended and pronated. The rest of her physical examination is normal. This clinical scenario most likely indicates which of the following? A. Fracture of the left clavicle B. Fracture of the left humerus C. left-sided Erb-Duchenne paralysis D. left-sided Klumpke paralysis E. Spinal injury with left hemiparesis "

C

"A 1-day-old male infant has bilious vomiting after every feeding. He hasn't passed any stools yet. He had no prenatal care, and was delivered vaginally at term. He weighs 3kg 6.61b., and his APGAR scores were 6 and 7 at 1 min and 5 min, respectively. On examination, the neonate is hypotonic, he has a flat facial profile, short ears with downfolding ear lobes, a single palmar crease, and a depressed fontanel. There is abdominal distention which is most prominent in the upper abdomen. His abdominal x-rays show gastric and duodenal gas distension with no air distally. What other anomaly can also be expected in this infant? A.Sigmoid volvulus B.Meconium plug syndrome C. Hirschsprung's disease D. Intussusception E.Meckel's diverticulum "

C

"A 1-month-old baby boy has bloody diarrhea. No infectious agent is identified, but the baby is found to be profoundly thrombocytopenic. The baby is also noted to have a skin rash, and a dermatologist diagnoses eczema. By three months of age, the baby begins to develop recurrent respiratory infections. If this child survives until adolescence, he is at particularly high risk of developing which of the following? A. Congestive heart failure B. Crohn disease C. Lymphoma D. Rheumatoid arthritis E. Wilms tumor "

C

"A 1-month-old infant is seen in a well-baby clinic. The mother states that the baby is constipated and feeds poorly. On examination, he is jaundiced, has a large posterior fontanel and an umbilical hernia, and exhibits poor muscle tone. He has gained only 300 g since discharge from the normal newborn nursery. Which of the following is the most likely diagnosis? A. Alpha1-antitrypsin deficiency B. Biliary atresia C. Congenital hypothyroidism D. Pyloric stenosis E. Syphilis "

C

"A 1-year-old child presents with failure to thrive, frequent large voids of dilute urine, excessive thirst, and three episodes of dehydration not associated with vomiting or diarrhea. Over the years, other family members reportedly have had similar histories. Which of the following is the most likely diagnosis? A . Water intoxication B . Diabetes mellitus C . Diabetes insipidus D . Child abuse E . Nephrotic syndrome "

C

"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. Gonococcal conjunctivitis B. Chemical conjunctivitis C. Congenital chlamydial infection D . Congenital rubella infection E . Adenovirus infection "

C

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

C

"A 10-year-old boy from the Connecticut coast is seen because of discomfort in his right knee. He had a large, annular, erythematous lesion on his back that disappeared 4 weeks prior to the present visit. His mother recalls pulling a small tick off his back. Which of the following is a correct statement about this child's likely illness? A . The tick was probably a Dermacentor andersoni B . The disease is caused by a rickettsial agent that is transmitted by the bite of a tick C . In addition to skin and joint involvement, CNS and cardiac abnormalities may be present D . Therapy with antibiotics has little effect on the resolution of symptoms E . The pathognomonic skin lesion is required for diagnosis "

C

"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. Kawasaki disease B. Staphylococcal scalded skin syndrome C. Scarlet fever D. Stevens-Johnson syndrome E. Mononucleosis "

C

"A 12-year-old girl has a solitary thyroid nodule found on routine examination; she has no symptoms. Which of the following is the most appropriate next step for this patient? A. Fine needle aspirate B. CT scan of the neck C. serum thyroid function tests D. Trial of suppressive T4 treatment to look for nodule shrinkage E. Excisional biopsy"

C

"A 12-year-old male child comes to the office after being referred for a medical evaluation. His schoolteacher says that he has a problem concentrating during class. He stares in space for a few seconds several times a day, and appears totally absorbed in his thoughts. He is not disruptive in class, but appears forgetful. There is no history of trauma, infection or problem at birth. On examination, the child is alert with stable vital signs. There is no loss of motor or sensory perceptioin. Which of the following can confirm the patient's diagnosis? A. CT scan of the head B. EMG studies C. EEG studies D. Psychiatric evaluation E. Lumbar puncture"

C

"A 12-year-old male child comes to the office after being referred for a medical evaluation. His schoolteacher says that he has a problem concentrating during class. He stares in space for a few seconds several times a day, and appears totally absorbed in his thoughts. He is not disruptive in class, but appears forgetful. There is no history of trauma, infection or problems at birth. On examination, the child is alert with stable vital signs. There is no loss of motor or sensory perception. Which of the following can confirm the patient's diagnosis? A. CT scan of the head B. EMG studies C. EEG studies D. Psychiatric evaluation E. Lumbar puncture"

C

"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. serum calcium, phosphorus, and magnesium levels B. analysis of cerebrospinal fluid CSF. C. cranial computed tomography CT. scan D. serum ammonia level E. serum acetaminophen level "

C

"A 2-year-old asymptomatic child is noted to have a systolic murmur, hypertension, and diminished femoral pulses. Which of the following should be performed as part of the preoperative workup and management of this child's disorder? A. Administration of indomethacin if there is a patent ductus arteriosus B. Ligation of a patent ductus arteriosus C. Echocardiography D. Aortogram with bilateral lower extremity runoffs E. Cardiac catheterization "

C

"A 3-year-old boy is admitted for seizure-like activity. He has been a healthy child and has been meeting all development milestones. His immunization schedule is up-to-date. Examination is notable for an erythematous throat and fever. His convulsions require IV administration of a benzodiazepine. serum analysis reveals a normal white cell count with mild basophilic stippling. The lumbar puncture reveals elevated CSF pressure. Head CT scan is notable for cerebral edema. Which of the following is the next diagnostic step? A. Antistreptolysin O titer B. Electroencephalography C. Protoporphyrin level D. Rapid slide Monospot. test E. Spinal fluid culture "

C

"A 3-yearold boy is brought to the ER with a two-day history of decreased appetite, neck swelling, and irritability. He keeps his head rotated slightly to the right side. He resists passive flexion of the neck and rotation to the left side. Which of the following is the best next step in managing this patient? A . Direct laryngoscopy B . Soft neck collar C . X-ray of the neck D . Lumbar puncture E . Botulinum toxin injection"

C

"A 4-year-old child presents in the clinic with an illness notable for swelling in front of and in back of the ear on the affected side, as well as altered taste sensation. Correct statements about this condition include which of the following? A . Arthritis is a common presenting complaint in children B . The disease could have been prevented by prior immunization with killed whole cell vaccine C . Involvement of the central nervous system CNS. may occur 10 days after the resolution of the swelling D . Orchitis can occur and is almost exclusively seen in prepubertal males E . Subendocardial fibroelastosis is a common complication in a child of this age "

C

"A 5-month-old boy is brought in for a routine check-up. He was born at 37 weeks' gestation and has had persistent wheezing since shortly after birth despite inhaled bronchodilator and oral corticosteroid therapy. His diet consists of 32 ounces of iron-fortified cow's milk-based formula daily. On physical examination, the child appears well-nourished and happy. You note moderate relief of his wheezing with neck extension. Which of the following is the most likely mechanism of this infant's wheezing? A . Allergic reaction to cow's milk B . Aspiration of a foreign body C . Compression of the airway by a vascular ring D . Chronic upper respiratory tract infection E . Asthma "

C

"A 6-month-old infant has been exclusively fed a commercially available infant formula. Upon introduction of fruit juices, however, the child develops jaundice, hepatomegaly, vomiting, lethargy, irritability, and seizures. tests for urine-reducing substances are positive. Which of the following is likely to explain this child's condition? A . Tyrosinemia B . Galactosemia C . Hereditary fructose intolerance D . α1-Antitrypsin deficiency E . Glucose-6-phosphatase deficiency "

C

"A 6-year-old child has had repeated episodes of otitis media. She undergoes an uneventful surgical placement of pressure-equalization PE tubes. In the recovery room she develops a fever of 40C 104F., rigidity of her muscles, and metabolic and respiratory acidosis. Which of the following is the most likely explanation for her condition? A . Otitis media B . septicemia C . Malignant hyperthermia D . Dehydration E . Febrile seizure "

C

"A 1 -year old child is brought in for a well-baby check-up. His parents report that he has been of good health and began walking a few weeks earlier. They are concerned that he tends to bump into things and falls more than his older sister did. Family history is significant for retinoblastoma. On examination, the pediatrician notes leukocoria of the left eye. No significant lymphadenopathy is present, and there is no enlargement of the liver or spleen. The child's height and weight are normal for age. Which of the following is the most appropriate next step in management? A. Explain to parents what leukocoria is and reassure them that it is temporary B. return visit in 1 month C. refer to neurologist D. refer to ophthalmologist E. Treat the eye with erythromycin ophthalmic ointment for 10 days "

D

"A 1 and half -year-old girl is sent to a children's hospital for evaluation following a nosebleed which was so severe as to require nasal packing and transfusion of platelet concentrates. When a blood sample had been drawn in the emergency room for serum chemistry studies, the local hospital laboratory had noted that the clot that formed was unusual in that it failed to retract. Peripheral blood smear obtained by finger puncture showed an appropriate number of normal-sized platelets, all of which were individual, without clumping. At the children's hospital, it was noted that the child's parents were cousins. Special platelet studies showed that the child's platelet's failed to aggregate with any physiologic aggregating agent, including a high concentration of exogenous ADP. Which of the following is the most likely diagnosis? A . Bernard-Soulier syndrome B . Chediak-Higashi syndrome C . May-Hegglin anomaly D . Thrombasthenia E . Von Willebrand disease "

D

"A 1-day-old full-term boy is in the neonatal intensive care unit with cyanosis. His BP is 80/40 mmHg in all 4 extremities, HR is 140/min, and respirations are 55/min. Pulse oxymetry shows 80% and does not improve with 100% inspired oxygen by face mask. He is breathing comfortably, but his fingertips and oral mucosa are blue. A continuous machine-like murmur is heard on auscultation. Chest x-ray shows clear lung fields bilaterally. Which of the following is the best next step in management of this patient? A. Furosemide B. Intubation with 100% FiO2 C. Propranolol D. Prostaglandin E1 E. red blood cell transfusion"

D

"A 1-day-old infant who received silver nitrate eye drops in the delivery room is suffering from bilateral purulent conjunctival discharge. Which of the following is the most likely cause of this child's condition? A. N. gonorrhoeae infection B. herpes simplex infection C. nasolacrimal duct obstruction D. chemical irritation E. Pseudomonas infection "

D

"A 1-month-old male infant is brought to the emergency department by his mother due to a 3-day history of projectile vomiting. He has been formulafed since birth, and his bowel movements are normal. He has never been ill before. His parents and 4-year-old sister are all healthy. One of his maternal uncles is mentally retarded. He weighs 3.1 kg 7 Ib. Physical examination reveals fair hair and skin, blue eyes, eczematous rash, and mild signs of dehydration. His urine has a mousy odor. What is the most appropriate screening test for this patient's early diagnosis and treatment? A. Aminolevulinic acid and porphobilinogen in the urine B. serumTSH C. blood level of alfa 1-antiprotease D. Guthrie test in urine E. serum LDH "

D

"A 1-month-old, previously healthy infant develops forceful projectile vomiting. No bile is seen in the vomitus. After the infant feeds, gastric peristaltic waves are visible crossing the epigastrium from left to right. several minutes later, the projectile vomiting occurs. Which of the following is the most likely diagnosis? A. Diaphragmatic hernia B. Duodenal atresia C. Esophageal atresia D. Hypertrophic pyloric stenosis E. Meconium plug syndrome "

D

"A 1-year-old girl is brought to the emergency department by her mother because the child's ""eyes and feet are dancing."" On physical examination, the girl is well developed and in no acute distress. Her temperature 37.0 C 98.6 F., blood pressure is 100/55 mm Hg, pulse is 100/min, and respirations are 20/min. The patient has opsoclonus, myoclonus, and ptosis of the right eye. On history, the mother notes the child was born ""looking like a blueberry muffin"" and has had a persistent cough since the age of 2 months. Which of the following is the most likely diagnosis? A. Astrocytoma B. Glioblastoma multiforme C .Hyperthyroidism D. Neuroblastoma E . Wilms tumor "

D

"A 1-year-old 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. osteogenesis imperfecta B. scurvy C. congenital syphilis D. rickets E. chondrodystrophy "

D

"A 10-year-old patient with sickle cell disease comes to the physician for a routine visit. The patient has a history of multiple pain crises and pneumonias. He was started on hydroxyurea one year ago and has not had any further pain crises since then. He has had no recent illnesses or hospitalizations. His physical examination is unremarkable. The patient's laboratory results are shown below. Complete blood count: Hemoglobin 9.0 g/L, Erythrocyte count 2.2 mln/mm3, MCHC 32%, MCV 105 fl, reticulocytes 2.0%, Platelets 212,000/mm3, leukocyte count 9500/mm3, Neutrophils 56%, Eosinophils 3%, Lymphocytes 36%, Monocytes 5%. Which of the following best describes the role of hydroxyurea in the treatment of patients with sickle cell disease? A. removes sickled red blood cells from the circulation B. Lyses microthrombi in the circulation C. Protects against encapsulated bacterial infections D. Increases fetal hemoglobin E. Chelates iron to prevent iron toxicity "

D

"A 2-year-old boy has been doing well despite his diagnosis of tetralogy of Fallot. He presented to an outside ER a few days ago with a complaint of an acute febrile illness for which he was started on a "pink antibiotic." His mother reports that for the past 12 hours or so he has had a headache and is more lethargic than normal. On your examination he seems to have a severe headache, nystagmus, and ataxia. Which of the following would be the most appropriate first test to order? A. Urine drug screen B. blood culture C. Lumbar puncture D. CT or MRI of the brain E. Stat echocardiogram "

D

"A 2-year-old presents to the emergency center with several days of rectal bleeding. The mother first noticed reddish-colored stools 2 days prior to arrival and has since changed several diapers with just blood. The child is afebrile, alert, and playful, and is eating well without emesis. He is slightly tachycardic, and his abdominal examination is normal. Which of the following is the best diagnostic study to order to confirm the diagnosis? A. Exploratory laparotomy B. Barium enema C. Ultrasound of the abdomen D. Radionucleotide scan E. Stool culture"

D

"A 20-month-old male is brought to the emergency department for evaluation of rectal bleeding after his parents discovered a substantial amount of maroon colored stool when changing his diaper. He has no history of hematochezia. The patient has been otherwise healthy and has not appeared to be in any pain. He has been eating and drinking well. His temperature is 37 C 98.6 F, blood pressure is 85/50 mmHg, pulse is 130/min, and respiratory rate is 20/min. On examination, the abdomen is soft, nondistended, and nontender. On rectal examination, there are no fissures or masses present. A fecal occult blood test is positive. The remainder of the physical examination is normal. Which of the following is the best test to confirm the diagnosis? A. Abdominal radiography B. Superior mesenteric arteriography C. Barium enema D. technetium-99m pertechnetate scan E. Abdominal CT scan"

D

"A 24-month-old pale child is brought to the office by his mother, who says, ""Doc, I think he is under some weird spell. He acts bizarre and always seems tired. He likes to eat wooden, painted toys."" The child and her mother live in a relatively poor neighborhood. CBC reveals:WBC 8,600 /mm3, Hemoglobin 7.1 g/dl, Hematocrit 25%, Platelets 166,000 /mm3. His blood lead levels are elevated. Which of the following is most likely seen in this child's peripheral blood smear? A. Megaloblastic anemia and basophilic stippling B. tear-drop RBCs and hypochromic, microcytic anemia B. Loss of concavity of the RBC and basophilic stippling D. Basophilic stippling and microcytic, hypochromic anemia E. Normochromic, normocytic anemia and basophilic stippling"

D

"A 3-year-old boy of African descent is brought to your office by his stepfather because of easy bruising. He says that the child bruises easily even without trauma. The child started playing games by himself recently. He has a past history of clavicular fracture, which the stepfather attributes to a fall down a set of stairs. The history of the biological father is unknown. On examination, there is a right knee effusion with decreased range of motion, and multiple soft tissue hematomas on the thigh. What is the most appropriate diagnostic step in management? A. Contact child protective services B. Obtain type 1 collagen assay C. Obtain prothrombin time and liver function tests D. Obtain factor VIII level E. Obtain bleeding time"

D

"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. CT scan of the abdomen and pelvis B. reassure the parents that microhematuria from minor trauma will resolve spontaneously C. serial hemoglobin and hematocrit determinations D. Urologic workup, starting with a sonogram E. retrograde ureterogram and cystogram "

D

"A 6-week-old child arrives with a complaint of "breathing fast" and a cough. On examination you note the child to have no temperature elevation, a respiratory rate of 65 breaths per minute, and her oxygen saturation to be 94%. Physical examination also is significant for rales and rhonchi. The past medical history for the child is positive for an eye discharge at 3 weeks of age, which was treated with a topical antibiotic drug. Which of the following organisms is the most likely cause of this child's condition? A. Neisseria gonorrhoeae B. Staphylococcus aureus C. Group B streptococcus D. Chlamydia trachomatis E. Herpesvirus "

D

"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 . Add pancreatic enzymes to the formula B . change to a lactose-free formula C . Increase calorie intake to 175 kcal/day by increasing volume per feed D . Substitute medium-chain triglycerides for long-chain triglycerides E . Supplement with vitamins A and E "

D

"A 6-year-old Caucasian boy is hospitalized for acute sinusitis that was accompanied with intensive nasal bleeding. Past medical history is significant for recurrent pulmonary infections and several hospitalizations for parenteral antibiotic therapy. The sweat chloride test is positive. The blood tests reveal a prothrombin time PT. of 20 seconds. Which of the following coagulation factors is most likely to be deficient in this patient? A . Fibrinogen B . Hageman factor C . Factor VIII D . Factor VII E . Factor V "

D

"A 6-year-old boy is brought to the clinic for the evaluation of a large, red, circular rash on his left thigh which has been present since two weeks and has been enlarging. He has a mild headache and myalgia, but is afebrile. Three weeks ago, he and his family visited relatives at a rural farm in Connecticut and went hiking in the woods. His temperature is 37.2 C 99 F. and pulse is 90/min. He is alert, active, appears non-toxic, and not in distress. On the anterior surface of his left thigh, there is a red ring that is 7 cm in diameter with central clearing, and a central brownish-red macule that is 3 mm in diameter. Which of the following measures would have prevented this condition? A. Childhood vaccinations as recommended by American Association of Pediatrics B. Careful sanitary measures in food preparation C. Avoiding water intake from streams D. Wearing light-colored clothing. Long-sleeved shirts and tucking pants into socks or boot tops E. No wearing light-colored clothing. Short-sleeved shirts and tucking pants into socks or boot tops"

D

"A 1-cm carcinoma of the breast is diagnosed by an excisional biopsy in a 36-year-old woman at 14 weeks' gestation. The axillary nodes are negative. Which of the following is the best management of this patient? A. terminate the pregnancy immediately and treat the breast cancer B. monitor the mass throughout pregnancy with serial breast ultrasounds C. induce labor at 34 weeks' gestation, then give chemotherapy D. perform a cesarean delivery at 36 weeks and treat the breast cancer E. modified radical mastectomy at the time of diagnosis"

E

"A 1-month old boy is brought to the emergency department by his mother, who states that he has been having what she describes as ""projectile vomiting"" for the past several days. She states that he vomits every time she feeds him, and the situation seems to be getting worse, although he does not seem to be in pain. She describes the vomitus as non-bilious, and he has had normal stools with no blood in them. On examination, the infant appears to be mildly dehydrated, his abdomen is soft, and there is a palpable, olive-sized, firm moveable mass in the right upper quadrant. Which of the following is the most likely diagnosis? A . Duodenal atresia B . Intussusception C . Hirschsprung disease D . Midgut volvulus E . Pyloric stenosis "

E

"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? A. PGE 1 administration B. Oxygen administration C. Digoxin and diuretic therapy D. Surgical repair E. reassurance"

E

"A 1-year-old boy is brought to your office with necrotic periodontal infection. The past medical history is significant for recurrent cutaneous and sinus infections revealing S. aureus and P. aeruginosa isolates. The separation of the umbilical cord was delayed 4 weeks.. CBC shows leukocytosis and increased neutrophils. The number of peripheral lymphocytes is normal. Gamma-globulin level is increased on plasma protein electrophoresis. The Nitroblue tetrazolium NBT. test is normal. Which of the following is the most likely defect present in this patient? A. Adenosine deaminase deficiency B. Complement deficiency C. Opsonization defect D. Destruction ofT lymphocytes E. Impaired leukocyte adhesion "

E

"A 1-year-old child has repeated episodes of vomiting and abdominal distention. An x-ray shows obstruction at the second portion of the duodenum. Laparotomy is performed and an annular pancreas is discovered. For a symptomatic partial duodenal obstruction secondary to an annular pancreas, which of the following is the operative treatment of choice? A. A Whipple procedure B. Gastrojejunostomy C. Vagotomy and gastrojejunostomy D. Partial resection of the annular pancreas E. Duodenostomy "

E

"A 1-year-old child is brought in for a regular ""well baby"" check-up. The child appears to have strabismus. The reflection of a bright light from the ceiling of the examination room comes from a different place in each eye. The family explains that the child has always looked that way, and there has been no recent change in the appearance of his eyes. Which of the following is the most effective management? A. No treatment unless the condition has not resolved spontaneously by age 7 B. Corrective lenses C. Each eye patched for a month at a time, alternating sides D. Surgical correction whenever he is old enough to decide whether he wants it for cosmetic reasons E. Surgical correction as soon as it is practical to do it "

E

"A 1-year-old child is brought to the physician for a routine visit. He was born full term with a birth weight of 71b 8oz 34 kg. and a birth length of 20 in 50.8cm. He has had no major illnesses or hospitalizations His parents report that he was breastfed exclusively for 6 months. He now eats a variety of baby foods and is being transitioned to whole milk. He can pull up to stand and cruise around holding onto objects, but cannot walk independently yet. He can feed himself small pieces of table food with his thumb and first finger. The only words he knows are mama, dada, and ball. His parents are concerned about his growth because some of the other children in his day care class are bigger than him. On physical examination, he weighs 221bs 10 kg. and is 30 in 76.2cm. long. A complete examination is unremarkable. Which of the following should you tell his parents? A. His weight is normal, but his height is less than expected. B. His weight is less than expected, but his height is normal. C. His growth is normal, but he has delayed motor development. D. His growth is normal, but he has delayed speech development. E. The child's growth and development are normal "

E

"A 1-year-old female infant is brought to the clinic by his 30-year-old mother due to feeding problems since birth. She still cannot walk nor speak. She began to sit when she was 8 months old. Her weight is in the 15th percentile, height is in the 20th percentile, and head circumference is in the 100th percentile for her age. She has multiple freckles in her armpit and groin area. She has cafe-au-lait spots on her skin, and the diameter of at least 20 of these spots is greater than 1.5mm. What is the most likely diagnosis? A . Neurofibromatosis type 2 B. Down syndrome C. Fetal alcoholic syndrome D. Normal development E. Neurofibromatosis type 1 "

E

"A 10-month-old boy develops an upper respiratory tract infection 2 days before presentation. On the day of presentation, he has a generalized tonic-clonic seizure lasting 30 seconds. His temperature is 40.0 C 104 F, blood pressure is 90/60 mm Hg, and respirations are 22/min. He is alert and smiling. He has rhinorrhea, and his neck is supple. He has bruises below his knees. Which of the following is the most likely diagnosis? A. Child abuse B. Idiopathic epilepsy C. Infantile spasms D.Meningitis E. Simple febrile seizure "

E

"A 10-month-old infant has poor weight gain, a persistent cough, and a history of several bouts of pneumonitis. The mother describes the child as having very large, foul-smelling stools for months. Which of the following diagnostic maneuvers is likely to result in the correct diagnosis of this child? A. CT of the chest B. serum immunoglobulins C. TB skin test D. Inspiratory and expiratory chest x-ray E. Sweat chloride test"

E

"A 10-month-old infant on long-term aspirin therapy for Kawasaki disease develops sudden onset of high fever, chills, diarrhea, and irritability. A rapid swab in your office identifies influenza A, adding her to the long list of influenza patients you have seen this December. Over the next few days, she slowly improves and becomes afebrile. However, 5 days after your last encounter you hear from the hospital that she has presented to the emergency center obtunded and posturing with evidence of liver dysfunction. Which of the following statements about her current condition is correct? A. With proper supportive care, the overall mortality rate is low. B. With her progressiveliver dysfunction, increased total serum bilirubin is anticipated. C. Administration of N-acetylcysteine is first-line therapy. D. seizures are uncommon with this condition. E. Death is usually associated with increased intracranial pressures and herniation. "

E

"A 10-year-old boy is brought in with a chief complaint of multiple colds. On further questioning, you elicit a history of chronic, clear nasal discharge with no seasonal variation. Other symptoms include sneezing, itching of the nose and eyes, as well as tearing and occasional eye redness. Some relief is obtained with an over-the-counter cold medicine containing antihistamine and a decongestant. His history suggests which of the following? A. nasal foreign body B. immunologic deficiency C. rhinitis medicamentosa D. chronic sinusitis E. allergic rhinitis "

E

"A 10-year-old boy is brought to clinic because of increasing weakness and dyspnea over the past 6 months. He has been previously healthy and is on no medications. There is no significant family history of illness. On examination, he appears pale. His hematocrit is 20%, mean corpuscular volume MCV is 60/mm3, and iron level is normal. Smear reveals basophilic stippling. Hemoglobin electrophoresis is consistent with the absence of one beta-globin gene. Which of the following is the most likely diagnosis? A. EUiptocytosis B. Hemoglobin S-C disease C. Porphyria D. Sickle cell disease E. Thalassemia "

E

"A 10-year-old boy is brought to the emergency room by his mother with right hemiplegia of sudden onset. The mother says that the child fell on a pencil in his mouth, ten hours before the onset of symptoms. His past medical history is insignificant. Family history is not significant. His blood pressure 110/60 mmHg and heart rate is 90/min. Physical examination reveals dense right hemiplegia, right hemianesthesia, and mild motor aphasia. Cardiac auscultation reveals S3. What is the most probable cause of this patient's condition? A. Antiphospholipid antibodies B. Migraine C. Epilepsy D. Congenital heart disease E. Internal carotid artery dissection "

E

"A 10-year-old boy is brought to the office by his mother after having a seizure this morning. All he can recall before the episode is ""seeing funny little lights."" According to his mother, his body went stiff; he lost consciousness, and then had jerky movements of the entire body. He bit his tongue, and started to drool. The seizure lasted for about one minute. After the seizure, he appeared confused for several minutes, and passed urine. He has been complaining of a headache for the past two hours. The neurological examination is normal. What type of seizure did this patient experience? A. Childhood absence seizure B. Status epilepticus C. Simple partial seizures D. Complex partial seizures E. Tonic clonic seizure "

E

"A 10-year-old boy is brought to the office by his parents because he is developing dark facial hair, deepening of the voice and a rapid height increase. He also complains of persistent headaches which are resistant to over-the-counter analgesics for the past 2 weeks. He denies nausea, vomiting or visual disturbances. His medical history is unremarkable. Physical examination reveals coarse pubic and axillary hair. Other pertinent findings include lid retraction, paralysis of upward and downward gaze, and poor pupillary reaction to light. His deep tendon reflexes are normal, Babinski sign is negative, and there are no focal neurologic signs. What tumor is most likely responsible for this child's symptoms? A. Medulloblastoma B. Neuroblastoma C. Oligodendroglioma D. Craniopharyngioma E. Pinealoma"

E

"A 2-year-old girl is brought to the emergency department with a fever, chills, poor appetite, and vomiting. On examination, she is irritable and diaphoretic. Her temperature is 39.2 C 102.5 F, blood pressure is 80/48 mm Hg, pulse is 88/min, and respirations are 17/min. She is tender at the left costovertebral angle. Initial laboratory tests show the following: leukocyte count 16,300/mm3, Hemoglobin 12.5 g/dL, Platelet count 245,000/mm3, blood urea nitrogen 6 mg/dL, Creatinine 0.5 mg/dl. Urinalysis is positive for leukocyte esterase and nitrite, with 150 white blood cells/hpf. After TV antibiotic administration and stabilization, what is the most appropriate diagnostic study? A. CT of the abdomen and pelvis B. IV pyelography C. Plain abdominal radiography D. Radionuclide imaging of the kidneys E. Voiding cystourethrography"

E

"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? A. Cystoscopy B. Dimercaptosuccinic acid DMSA scan in 1-2 months C. Intravenous pyelogram D. Voiding cystourethrogram VCUG now E. VCUG in 1-2 months "

E

"A 4-year-old male is brought to the physician with fever and headache. His symptoms began two days ago with low-grade fever, cough, and congestion. Last night, he developed a temperature of 102 F 38.9 C and became fussy and less active. Today, he is crying and complaining of a headache. His parents report that he has vomited twice today. In the office, his temperature is 102.5 F 39 C., pulse is 110/min, and respiratory rate is 20/min. On examination, he is irritable and shows signs of photophobia. His oropharynx is erythematous. Nuchal rigidity is present and when the neck is flexed, the patient flexes his lower extremities. The remainder of the physical examination is normal. Lumbar puncture is performed and the results are shown below CSF: Glucose 60 mg/dL, Protein 80 mg/dL, RBC 10/mm3, WBC 100/mm3, Neutrophils 10%, Lymphocytes 70%, Monocytes 20%, Gram stain negative. Which of the following organisms is most likely responsible for this patient's presentation? A. Streptococcus pneumoniae B. Mycobacterium tuberculosis C. Epstein-Barr virus D. Neisseria meningitidis E. Echovirus "

E

"A 4-year-old previously wellboy 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. Hepatitis B B. Hepatitis A C. Hemolytic-uremic syndrome D. Gilbert syndrome E. Glucose-6-phosphate dehydrogenase deficiency "

E

"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 . Aniridia B . Cleft palate C . Macroglossia D . Omphalocele E . Small left colon "

E

"A 6-year-old African-American child is brought in by his father for complaints of easy fatigability and pallor. These symptoms occurred after the son was treated with ""some medication"" for a recent diarrhea. Physical examination is normal except for pallor and multiple petechiae. Laboratory values are as follows: Hb 8.0 g/dL, WBC 12,000/cmm, Platelets 50,000/cmm, blood glucose 118 mg/dL, serum Na 135 mEq/L, serum K 5.3 mEq/L, Chloride 110 mEq/L, Bicarbonate 18 mEq/L, BUN 38 mg/dL, serum creatinine 2.5 mg/dL, Total bilirubin 3 mg/dL, Direct bilirubin 0.5 mg/dL, PT 12 seconds, APTT 30 seconds, LDH 900 IU/L, reticulocyte count 6%. A peripheral blood smear reveals giant platelets and multiple schistocytes. What is the most likely underlying pathophysiology for this boy's pallor? A . Sickle cell anemia B . Thalassemia C . Vitamin B 12 deficiency D . Folate deficiency E . Microangiopathic hemolytic anemia "

E

"A 6-year-old African-American child is brought in by his father for complaints of easy fatigability and pallor. These symptoms occurred after the son was treated with ""some medication"" for a recent diarrhea. Physical examination is normal except for pallor and multiple petechiae. Laboratory values are as follows: Hb 8.0 g/dL, WBC 12,000/mm3, Platelets 50,000/mm3, blood glucose 118 mg/dL, serum Na 135 mEq/L, serum K 5.3 mEq/L, Chloride 110 mEq/L, Bicarbonate 18 mEq/L, BUN 38 mg/dL, serum creatinine 2.5 mg/dL, Total bilirubin 3 mg/dL, Direct bilirubin 0.5 mg/dL, PT 12 seconds, APTT 30 seconds, LDH 900 IU/L, reticulocyte count 6%. A peripheral blood smear reveals giant platelets and multiple schistocytes. What is the most likely underlying pathophysiology for this boy's pallor? A . Sickle cell anemia B . Thalassemia C . Vitamin B12 deficiency D . Folate deficiency E . Microangiopathic hemolytic anemia "

E

"A 6-year-old boy is seen in the office for evaluation of polyuria. Further questioning reveals several months of headache with occasional emesis. Your physical examination reveals a child who is less than 5% for weight. He has mild papilledema. His glucose is normal, and his first urine void specific gravity after a night without liquids is 1.005 g/mL. Which of the following might also be expected to be seen in this patient? A . Sixth nerve palsy B . Unilateral cerebellar ataxia C . Unilateral pupillary dilatation D . Unilateral anosmia E . Bitemporal hemianopsia "

E