Pediatrics
Youngdoctor - 04-06-07 22:43

1)A 1 1/2 -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 follow
fielderstreet: 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

2)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. Atrial septal defect
B. Hypoplastic left heart syndrome
C. Patent ductus arteriosus
D. Tetralogy of Fallot

3)A 5-year-old boy develops a headache, cough, myalgia and a fever. He has been a healthy child with all immunizations up to date. He is given a decongestant and an aspirin for his symptoms with some relief. However, 4 days later, he is brought back by his parents because of persistent vomiting and irritability. On physical examination, he is found to be semicomatose, becoming combative on stimulation. Which of the following levels should be measured to aid in the diagnosis of this patient?
A. Serum ammonia level
B. Serum blood urea nitrogen level
C. Serum calcium level
D. Serum opiate level
E. Serum sodium level

4)A 14-year-old boy is brought to the emergency department because of persistent midepigastric
pain for two days. The pain is getting no better, yet it is no worse, and radiates to his back. The
boy also has had fever, as well as nausea and vomiting that is worse when his temperature
rises in the afternoon. On examination, his temperature is 39.2 C (102.5 F) and there is
marked upper abdominal tenderness with guarding. Mild abdominal distention is present with
no audible bowel sounds. A complete blood count reveals an elevated leukocyte count and a
normal serum amylase. Which of the following is the most likely diagnosis?
A. Fitz-Hugh-Curtis syndrome
B. Intussusception
C. Wilms tumor
D. Pancreatitis
E. Pyelonephritis

5)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. Acute appendicitis
B. Acute cholecystitis
C. Crohn disease
D. Functional abdominal pain
E. Irritable bowel syndrome

6)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. Chronic lead intoxication
B. Fragile-X syndrome
C. Gout
D. Huntington disease
E. Lesch-Nyhan syndrome

7)A full-term infant is brought to the office on her 6th day of life because her mother noted that she looked "yellow". The mother states that the infant is strictly breast-fed and has been eating every 2-3 hours. On examination, she is noted to be jaundiced over her trunk and face. There is no scleral icterus. She is otherwise healthy. Both the mother and baby are Rh positive. Which of the following is the most likely cause of this infant's jaundice?
A. Breast-feeding jaundice
B. Glucose-6-phosphate dehydrogenase (G6PD) deficiency
C. Hypothyroidism
D. Physiologic jaundice
E. Rh incompatibility

8)A 6-year-old boy with mental retardation has recently been diagnosed with Fragile X syndrome. His 9-year-old sister appears to be of normal intelligence but has symptoms of attention deficit hyperactivity disorder (ADHD). What is the first test that is indicated in her work-up for ADHD?

A. EEG
B. Cytogenetic testing
C. MRI
D. Intelligence quotient (IQ) test
E. Urine for metabolic screen