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1-Incontinentia pigmenti or Bloch-Sulzberger syndrome is an X-linked dominant genodermatosis. Which is NOT a criterion for diagnosis? A. B. C. D. E. Neonatal vesicular rash with eosinophilia Multiple congenital nevi Blaschkoid hyperpigmentation on the trunk fading in adolescence Linear, atrophic hair less lesions Family history 2-All of the following medications are definite contraindications to breastfeeding EXCEPT: A. B. C. D. E. cyclophosphamide cyclosporine phenobarbitone methotrexate heroin 3-Breast-feeding is NOT advisable if one or more of the following conditions are present EXCEPT: A. B. C. D. E. an infant diagnosed with galactosemia a mother with mastitis and fever of 39°C a mother taking cancer chemotherapy agents a mother infected with HIV virus a mother suffering of untreated active tuberculosis 4-Transient neonatal pustular melanosis is characterized by vesicles, superficial pustules and pigmented papules. Which following statement is WRONG? A. B. C. D. It is present at birth Its etiology is unknown It is more frequent in pigmented population A Tzank smear will reveal a predominance of neutrophils without evidence of bacteria E. It needs immediate septic work-up and antibiotherapy 5- A six hour-old Full-Term infant has cloudy corneas on Examination. Re-exam at 72h of age shows no change in ocular findings. Which of the following is the most appropriate management? A. B. C. D. E. Evaluate the infant for increased ocular pressure Treat the infant with propranolol Reassure the parents that these findings are benign Evaluate the infant for retinoblastoma Evaluate the infant for renal abnormalities 1 6-All of the following are factors influencing the immunologic properties of human milk EXCEPT: A. B. C. D. E. Ig A Macrophages Lysosyme Long chain polyunsaturated fatty acids Lactoferrin 7- Organic acidemias should be suspected in all of the following clinical presentations except: A. B. C. D. E. A newborn with sudden onset of lethargy that progressed to coma A 2-year-old child with splenomegaly and dysmorphic features A 1-year-old infant with progressive developmental delay A newborn with wide anion-gap metabolic acidosis A 6-month-old infant with sweaty-feet odor of urine 8- Hypoglycemia is a common problem in newborns, infants and children. All of the following statements are true Except: A. Hypoglycemia, hepatomegaly and hyperlipidemia suggest glycogen storage disease B. Hypoketotic hypoglycemia suggests fatty acid oxidation defect C. Hypoglycemia, hepatosplenomegaly and large tongue suggest BeckwithWiedemann syndrome D. Hypoglycemia and metabolic acidosis suggest urea cycle disorder E. Hypoglycemia and ketosis in a child after prolonged overnight fasting suggest ketotic hypoglycemia 9-All of the following matches between each vitamin deficiency state and its characteristic clinical findings are true Except: A. B. C. D. E. Vitamin A Night blindness Vitamin C Scurvy Vitamin D Craniotabes d. Niacin Pellagra Pyridoxine Beriberi 10- Which of the following best describes the external genitalia of a newborn male infant with adrenogenital syndrome? A. ambiguous genitalia B. virilized male phallus C. normal male genitalia D. penile-scrotal transposition E. micropenis 2 11-Of the following verbal skills, which is most likely to be apparent in the normal 2-yeal-old: A. B. C. D. E. Two-word sentences and 50 words vocabulary Ability to name 4 colors consistently Ability to demonstrate 3 prepositional commands Ability to say his full name and sex 5-word sentences 12- A male infant was found to have low T4 levels in newborn screening. Further evaluation reveals normal free T4 and TSH levels. All the following are true except A. B. C. D. E. atients are usually asymptomatic It is an x-linked disorder The level of thyroid binding globulin is high It requires no treatment It may be a source of confusion with hypothyrodism 13-In Kawasaki syndrome the coronary involvement in nontreated children is estimated to be: A. B. C. D. E. 5%. 8%. 10%. 20% 40% 14-. A FT AGA male infant with HC = 41 Cm, + transillumination over the frontal & parietal parts of the skull. Mom states that 2 of her brothers who died in infancy had large heads. Which of the following is the most likely diagnosis? A. B. C. D. E. Normal varient Familial macrencephaly Aqueductal stenosis Dandy-Walker syndrome Tay-Sachs disease 15-Ebstien anomaly can be characterized by all of he following except: A. B. C. D. E. right axis on the ECG. right to left shunt. tricuspid regurgitation. atrialized right ventricle. huge heart on x-ray. 3 16-Snowman sign on a chest- X- Ray is typical of: A. B. C. D. E. Total pulmonary venous anomaly. Tetralogy of Fallot. Transposition of great arteries. Truncus arteriosus. None of the above 17-Disproportionate dwarfism is an important feature of one of the following Conditions:A. B. C. D. E. Achondroplasia. Constitutional short stature. Nutritional deprivation. Familial short stature. Psychological deprivation. 18-Which malignancy you expect to find in a child with aniridia and congenital genitourinary abnormalities? A. B. C. D. Lymphoma Sarcoma Neuroblastoma Wilms E. Adrenal carcinoma 19-Which of the following conditions should be considered specifically for children with head growth deceleration, abnormal respiration, seizures, and hand wringing in whom Autism Spectrum Disorder is suspected? a. Lead poisoning b. Celiac disease c. Fragile X syndrome d. Rett Syndrome e. Krabbe disease 20-Growth hormone may be used in the treatment of short/small stature associated with all of the following conditions except:A. B. C. D. E. Turner syndrome. Prader Willi syndrome. End stage renal failure (before kidney transplantation). d. Intra uterine growth retardation. Achondroplasia. 4 21-A mother brings her 8-months old daughter to your clinic for evaluation of colic. While interviewing the parent, you observe the child repeatedly flex her upper and lower extremities all at once. The child is developmentally normal. On physical examination, you find four irregular, hypopigmented spots on her trunk. Neurological examination results are otherwise normal. Of the following the test MOST likely to lead to this child’s diagnosis is A. B. C. D. E. Karyotyping. Lumbar puncture. Magnetic resonance imaging of the brain. Skin punch biopsy for fibroblasts Urine organic acid assessment 22-The triad of ipsilateral facial port wine stain, eye abnormalities, and leptomeningeal and brain abnormalities is known as: a. b. c. d. the Crow sign. PHACE syndrome. Kassaback-Merritt syndrome. Sturge-Weber syndrome. e. Klippel- Trenaunay syndrome 23-Which of the following conditions constitutes the need for renal biopsy in a patient who has nephrotic syndrome? A. B. C. D. E. Age between 2 to 6 years at initial presentation. Lack of response after 2 weeks of prednisone therapy Low serum complement level at initial presentation. Relapse 1 year after initial diagnosis. Serum albumin level less than 2 g/dL (20 g/L). 24-Among the following, the most sensitive laboratory test to diagnose primary hypothyroidism is measurement of serum A. B. C. D. E. Free T4 Thyroglobulin Thyroid Antibodies Total T3. TSH 25-a 4 years old child with nephrotic syndrome. He presented to the emergency department with abdominal pain and fever. You suspect spontaneous bacterial peritonitis. Which of the following micro organisms is the most likely cause of this clinical condition? A. B. C. D. E. Aspergillus species. Candida albican. Klepsiella. Amebiasis. Pneumococci 5 26-- The absolute neutrophil count of a patient who complains of fever is 200 cells/ mm3. Which of the following micro organisms should be taken into consideration when you treat this patient? A. B. C. D. Pseudomonas aerogenosa. Staphylococcus aureus. Both. Neither 27-Hypo parathyroidism is reported in which of the following diseases? A. B. C. D. E. Ataxia talengectasia. Wisckott Alrdrich syndrome. Digeorge syndrome. Bruton's disease. ADA deficiency causing SCID. 28- Influenza virus vaccine is usually administered annually because of antigen shift and limited duration of protection. Which of the following groups should receive the vaccine? A. B. C. D. E. Infant with Still’s murmur Government employees Infants under 6 months of age Patients on long-term aspirin therapy Young patients with seizure disorder 29-Criteria for SIADH (syndrome of inappropriate anti diuretic hormone) include all of the following except: A. B. C. D. E. Hyponatremia a. Hypoosmolality Hypovolemia High urinary sodium Concentrated urine 30- Chronic lead poisoning causes all of the following except: A. B. C. D. E. Failure to thrive Constipation Glomerulonephritis Macrocytic anemia Encephalopathy 31-You suspect constitutional short stature in a 14-year-old boy who is 5 ft 3 in (160 cm) tall. Among the following, the clinical finding that is most supportive of this diagnosis is: A. B. C. D. E. Bone age of 12 years Diagnosis of cystic fibrosis Elevated alkaline phosphatase activity Genital SMR of 4. Low thyroid-stimulating hormone level 6 32-Of the following major Jones criteria for rheumatic fever, the one that is sufficient to make the diagnosis when encountered alone is: A. B. C. D. E. Polyarthritis Sydenham chorea Erythema marginatum Carditis Huntington’s chorea 33-The best screening test for immunodeficiency in a patient after her second episode of meningococcal meningitis is: A. B. C. D. E. 34. Quantitative immunoglobulins CH50 T cell subsets IgG subclasses Nitroblue tetrazolium (NBT) test A FT NB infant has complex cyanotic congenital heart disease. Peripheral Blood Smear reveals: Marked anisocytosis, poikiloctosis & spherocytosis ≥ 25% RBCs have autophagic vacuoles, Howell-Jolly bodies or both. Which of the following is the most likely diagnosis? A. B. C. D. E. septicemia DIC Asplenia Drug induced hemolysis ABO hemolytic disease 35.Anemia of prematurity is best explained by which of the following? A. B. C. D. E. Inadequate iron stores in the preterm infant Production by the liver of a complex erythropoietin that is inactive Rapid destruction of fetal Red Blood Cells Circulating antagonist to erythropoietin Inadequate erythropoietin production by the kidney 36-Which of the following statements best describes the adjustments that should be made in the DTP immunization schedule of preterm infants? A. No adjustment should be made B. Immunization schedule should be adjusted according to corrected age C. Immunization should start when the infant reaches 2 kg of body weight D. Immunization schedule should be adjusted according to corrected age, unless disease prevalence in the community indicates otherwise. E. Immunization should start at 2 months or discharge from the hospital (whichever comes first) 7 37-In Down syndrome the following are TRUE EXCEPT: A. B. C. D. E. An increased incidence of ALL. In translocation the total number of chromosomes is always 47. Mosaiscism accounts for 1-2% of cases. The commonest form is non-disjunction. Duodenal Artesia is common in Down syndrome 38.All are true regarding Familial Mediterranean fever (FMF) EXCEPT A. Is an inherited disorder characterized by brief, acute, self-limited episodes of fever and polyserositis B. FMF appears to be transmitted as an autosomal dominant disease C. FMF occurs among ethnic groups of Mediterranean origin D. Attacks of FMF can be prevented by prophylactic colchicine therapy E. One third to one half of untreated patients with FMF develop amyloidosis that may progress to nephrotic syndrome and renal failure over a period of months to several years 39-A 6-yr-old girl presents with breast enlargement and pubic hair development. Otherwise, she is asymptomatic. Her serum LH level is elevated. The most likely diagnosis is: A. B. C. D. E. Central precocious puberty Hypothyroidism b. Exposure to exogenous estrogen An estrogen-secreting tumor An adrenal tumor 40.Hypoketotic hypoglycemia occurs in all of the following disorders except: A. B. C. D. Hyperinsulinism Growth hormone deficiency Fatty acid oxidation defects Ketogenesis defects E. Maple syrup urine disease 41-You are evaluating a 5 years old male for excessive obesity. He was extremely hypotonic in the first 2 years of his life, and then the muscle tone became gradually normal. Later he developed uncontrollable appetite. He has global developmental delay. His testis and penis are small. The scrotum is atrophic. The most likely diagnosis is: A. B. C. D. E. Fragile X syndrome. Down syndrome. Trisomy 13. Prader- Willi syndrome. Klinefelter syndrome 8 42-Of the following, the MOST common fetal anomaly associated with maternal parvovirus B 19 Infection during pregnancy is: A. B. C. D. E. Neural tube defect Dwarfism Nonimmune fetal hydrops Cataract Cardiomyopathy 43-Neonatal hyperammonemia can be the result of the following EXCEPT: A. B. C. D. E. Urea cycle defects Hypothyroidism Disorders of Branch chain amino acid metabolism Severe perinatal asphyxia Liver failure 44- A 12—year old boy presents with two weeks history of frontal headaches. He has had morning vomiting for the last three days. Upon further questioning you discover he is doing poorly in school and that his parents have recently divorced. His only medication has been aspirin for the headaches and tetracycline taken daily for the last six months for acne. Examination reveals facial acne and bi-lateral papilledema without focal neurological signs. Skull roentgenogram and computed tomography of the head (CT scan) are negative. The most likely diagnosis is: A. B. C. D. E. Craniopharyngioma Pseudo tumor cerebri Tension headache Ependymoma Migraine 45-The prenatal diagnosis of all the following may be accomplished by chorionic villous sampling EXCEPT: A. B. C. D. E. Neural tube defects Thalassemia Sickle cell anemia Hurler disease Glycogen storage disease type II 46.All of the following are manifestations of Infantile Autism except a. The autistic child is withdrawn and often spends hours in solitary play b. Non-developed or poorly-developed verbal and non-verbal communication skills. c. Tantrum-like rages may accompany disruptions of routine daily activities d. Eye contact is prolonged and persistent e. Stereotypic body movements 9 47-One of the following is true concerning Guillain Barré Syndrome (GBS):A. Motor conduction velocities are within normal ranges B. Urinary incontinence or retention is against the diagnosis of GBS C. Bulbar involvement is a rare occurrence D. DTRs reflexes are exaggerated E. CSF studies are essential for diagnosis. Normal CSF findings in the 1st days do not rule out GBS. 48-Each of the following anti-epileptic drugs is matched correctly with one of its side effects except a. Diazepam // sedation b. Phenobarbital // irritability c. Carbamazepine // skin lesions d. Valproic Acid // hepatic dysfunction e. Phenytoin // gum hypoplasia 49- Which of the following cells are usually absent in the circulation of a boy with Bruton's disease? a. T helper lymphocytes. b. B lymphocytes. c. Natural killer cells. d. Monocytes. e. T suppressor lymphocytes 50-A positive ANA test in a 7 years old child who has juvenile idiopathic arthritis (JIA) puts the child at a higher risk to develop which of the following complications? A. B. C. D. Anterior uvietis. Glomerulonephritis. Gastritis. Splenomegally. E. Liver failure 51-Cystic hygroma and lymphatic channel malformations are associated with which of the following karyotypes? a. 47,XX,+18 b. 69,XXX c. 46,XX d. 45, X 10 52- Common clinical findings in children with phenylketonuria include all of the following except: a. Mental retardation. b. Musty odor. c. Seizures. d. Macular cherry-red spots. e. Recurrent vomiting 53-management of the newborn with large cephalhematoma is: a. surgical drainage b. needle aspiration c. observation d. head CT scan e. EEG 54-The patient in the given picture has fever daily for the last 6 days, he is irritable and the focus of fever was not localized. He has cervical lymphadenopathy and balanitis. The treatment of choice in this patient at this point is A. B. C. D. E. Intravenous pulse dose of methylpredniselone. Intra venous high dose of vitamin A. Intravenous immune globulins and high dose aspirin. Supportive only. Methotrexate. 55- A 4 years old child has persistent (even when not febrile) neutrophil count of 30 K/ mm3. Pseudomonas speciecs was isolated from her skin lesion. She has poor wound healing and recurrent oral ulcerations. The most likely diagnosis is A. B. C. D. E. Common variable immune deficiency. Severe combined immune deficiency. Leukocyte adhesion defect type I. Simple eczema. Dermatographism 11 56-All of the following are features of asthma except a. Recurrent wheezing. b. Reversibility of pulmonary function tests especially FEV1. c. Finger clubbing. d. Familial tendency. e. Night time symptoms indicate severity of the disease 57-A 15-year-old girl presents to your office with a 3-month history of facial rash, headaches, swollen knees, and pallor. Urine dipstick analysis shows the presence of protein and blood. You are highly suspicious that she has SLE. Her parents are seeking as much information as possible as you proceed with her evaluation to confirm the diagnosis. The organ system involvement in SLE that is most likely to cause the most serious morbidity and mortality is: A. B. C. D. E. Cardiac. Central nervous system Gastrointestinal Hematologic. Renal. 58-An obstetrician is closely monitoring a 25-year-old pregnant woman who has SLE. Her pregnancy, even with the history of SLE, has been stable. You are consulted to advise the woman about the risks for her newborn. You advise her that her infant is most likely to develop neonatal lupus erythematosus if the mother has: A. B. C. D. E. a.A low C4 concentration b.Anti-Ro antibodies c.Anti-Sm (Smith) antibodies. d.Proteinuria. e.Thrombocytopenia. 59-You are asked to see a 21-day-old boy who has jaundice. Physical examination findings are normal except for yellow discoloration of the skin and sclera. Serum bilirubin is 10 mg/dL (171 mcmol/L), with the direct component of 5 mg/dL (85.5 mcmol/L). Serum alpha-1antitrypsin concentrations are normal. Ultrasonography of the abdomen shows a nondilated biliary tract. Which of the following is the most appropriate next step A. B. C. D. E. Computed tomography of the abdomen. Doppler velocities measurement of hepatic artery and venous blood flow. Endoscopic retrograde cholangiopancreatography. Hepatobiliary nuclear medicine scan Magnetic resonance imaging of the abdomen 12 60-At times, the pediatrician must discern between the clinical manifestations of bleeding seen in patients who have hemophilia and those seen in a child who has thrombocytopenia. The type of clinical manifestations of bleeding seen in a patient who has classic hemophilia compared with a child who has thrombocytopenia is most likely to be A. B. C. D. E. Central nervous system Epistaxis. Gingival. Hematuria. Large joints 61-A 32-year-old female who is grava 2 para 1 reports that her first infant had severe bruising and nosebleeds and died of a central nervous system hemorrhage at 5 days of age. You suspect that this first newborn died of neonatal alloimmune thrombocytopenia. Her second infant, for whom you are now caring, has mucous membrane bleeding and petechiae, and a laboratory evaluation demonstrates a platelet count of 5x103/mcL (5x109/L) at 48 hours of age. The treatment that could help control this infant’s bleeding as well as diagnose the cause of the thrombocytopenia is: A. B. C. D. E. Anti-D immunoglobulin Corticosteroids Exchange transfusion Maternal donor platelet transfusion Random donor platelet transfusion 62-The mother of a 2-year-old boy expresses concern during a health supervision visit that her child has had increased problems with snoring and periods of breathing difficulties during his sleep. You suspect the boy has obstructive sleep apnea (OSA) due to adenotonsillar hypertrophy. Of the following, the most serious complication if this condition is not recognized and treated is: A. B. C. D. E. Cor pulmonale Craniofacial abnormalities Daytime narcolepsy Failure to thrive Severe behavioral problems 63-A 16-month-old boy is brought to your clinic for an evaluation. His mother reports that he has had more than 10 episodes of otitis media and one hospitalization for pneumonia. Her only other complaint is that he bruises easily. On physical examination, you note multiple bruises and patches of eczema over his arms and legs. Of the following, the most likely diagnosis is: A. B. C. D. E. Ataxia-telangectasia Chronic granulomatous disease Combined variable immunodeficiency Wiskott-Aldrich syndrome .X-linked agammaglobulinemia 13 64-Name the syndrome associated with LGA, macroglossia, hypoglycemia, hepatoslenomegaly and omphalocele: a- Prader willi syndrome b- Beckwith Weidemann syndrome c- Sotos syndrome d- Silver Russell syndrome 65- The usually benign .self limited reddening of one side of the infant with a sharp line of demarcation At the midline possibly related to autonomic factors is called: A. B. C. D. Allagillie syndrome Harlequin color change Horner syndrome Red man syndrome 66- What is generally the length of time considered an apneic period of clinical importance? a- Greater than 6 seconds b- Greater than 30 seconds c- Greater than 20 seconds d-Greater than 45 seconds 67- Platelets trapping, consumptive coagulapathy associated with congenital hemangioma is called: A. B. C. D. Kasabach –merrit syndrome TAR syndrome HUS syndrome Strawberry hemangioma syndrome 68-What is the cause of roseola infantile? a- mycoplasma pneumonia b- human herpisvirus 6 c- parvovirus 19 d- parainfluenza A 69- Somogyi phenomenon is: a- early morning hyperglycemia associated with rebound from severe nocturnal hypoglycemia b- early morning hypoglycemia associated with hyperinsulinemia from nocturnal hyperglycemia c- early morning hyperglycemia due to lack of insulin and rising cortisol d- early morning hypoglycemia due to high evening insulin dose 70- The following may be associated with Down syndrome: a- Hirschsprung disease b- Hypothyroidism c- Increased risk of leukemia d- Celiac disease e- all of the above 14 71- Pansinusitis, bronhiectasis, male infertility and situs inversus is a condition called: a- Eisienmenger syndrome b- immotile cilia syndrome or Kartagener syndrome c- Rubenstein Taybi syndrome d-combined immune deficiency syndrome 72- Precocious puberty is defined as puberty beginning before: a- boys 9-10 years, girls 8 years b- boys 8 years, girls 11 years c- boys 12 years, girls 12 years d- boys 14 years, girls 13 years 73-A 12-year-old boy who is at the 90th percentile for weight complains of slight pain in the right thigh and knee for about a month. His complaints are made worse by physical activity and he has a mild limp. He has no history of recent infections or trauma. Physical examination reveals a slight decrease in internal rotation of the right hip. There is mild rightsided metaphyseal osteopenia on radiograph. Of the following, which would be the MOST likely diagnosis in this boy? A) Transient synovitis B) Septic arthritis C) Osteomyelitis D) Slipped capital femoral epiphysis E) Legg-Calve-Perthes disease 74- An infant is born at 28-weeks gestation with severe Respiratory Distress Syndrome (RDS). The infant requires assisted ventilation and surfactant replacement therapy. Secondary to poor oxygenation, the positive end-expiratory pressure (PEEP) is increased from 4-6 cm H20. Shortly thereafter, you are called to the bedside for an acute decrease in oxygenation and an increase in the partial pressure of carbon dioxide (PaC02). Which of the following therapeutic interventions would be MOST indicated at this time? A) Transillumination of the chest B) Increase the ventilator peak inspiratory pressure (PIP) C) Decrease the PEEP D) Begin oscillatory high-frequency ventilation E) Administer a bolus of normal saline at 10 cc/kg 15 75- A 9 years old child with follicular tonsillitis developed diffuse erythematous maculopapulr rash after staring treatment with oral amoxicillin, the child most likely has had: CMV infection influenza type A Parainfluenza virus EBV infection Simple cold 76- 2 months old girl presents to you with fever, skin rash, hepatosplenomegaly and an enlarged left axillary lymph node. You confirm suspected disseminated BCG vaccine infection. Select the incorrect statement: Treatment is not indicated. A. B. C. D. Type 1 cytokines deficiency is a possible explanation. Severe combined immune deficiency is still possible. Chronic granulamatous disease is also a possible diagnosis. Hyper IgM syndrome is likely as well. 77-Among the following clinical findings, the one most suggestive of a diagnosis other than rotavirus infection is: A. B. C. D. E. Fever grossly bloody stools Low serum bicarbonate concentrations Non-bilious vomiting Reducing substance-positive stool 78- How long is a child infectious with chickenpox after the lesions erupt? A. B. C. D. 7-10 days 14 days 3 weeks 3 days 79- In malabsorption all of the following are correctly matching with the corresponding disease Except: A. B. C. D. E. Acanthocytes - Abetalipoproteinemia Pellagra-like rash - Hartnup disease Lymphopenia - Shwachman-Diamond syndrome Chronic sinopulmonary disease - Cystic fibrosis Responds to oral zinc sulfate -Acrodermatitis enteropathica 16 80-A 10-year-old has a Glasgow Coma Scale score of 4 and develops irregular respirations after head trauma. The next important step in the care of this patient is to A. B. C. D. perform endotracheal intubation. administer 20 mL/kg of lactated Ringer solution administer naloxone administer mannitol E. obtain a head CT scan 81-All are correct associations with the corresponding mineral deficiency EXCEPT: Zinc – Dwarfism. Chloride – Alkalosis. Selenium - Cardiomyopathy. Iodine – Caries. E. Phosphorus – Rickets. A. B. C. D. 82-Examination of the cerebrospinal fluid of a 2-year-old child supports the diagnosis of bacterial meningitis. The child is moderately dehydrated. The BEST approach to prescribing fluid therapy for this child would be to A. restrict fluid to prevent the syndrome of inappropriate antidiuretic hormone (SIADH) B. treat the dehydration immediately C. consider the possibility of the SIADH only if the child’s urine sodium concentration is greater than 60 mEq/L D. treat the dehydration after antibiotic therapy has been provided for 24 hours 83-A 10-month-old boy has lethargy, pinpoint pupils, and excessive salivation His mother reports that he has vomited twice within the past hour and has had one liquid stool. Two days earlier, the home was sprayed with an unknown pesticide to eliminate fleas You suspect either organophosphate or carbamate poisoning. A TRUE statement about the similarities and differences between organophosphate and carbamate poisoning is: A. B. C. D. Both classes of drugs inhibit acetylcholinesterase Carbamates cross the blood-brain barrier more readily than do Organophosphates Measurement of erythrocyte cholinesteraSe activity is more useful for detecting Carbamates than organophosphates E. Administration of pralidoxime is contraindicated in the treatment of organophosphate poisoning 17 84-A TRUE statement about acute pancreatitis in children is: A. Abdominal pain is a rare symptom B. An elevated serum amylase level is the most specific indicator of pancreatic infIammation, C. Obstructive lesions may be diagnosed by ( ERCP ) retrograde cholangiopancreatography D. Ultrasonography is not helpful in diagnosis E. Immediate surgical intervention is the treatment of choice 85-The parents of a 3-year-old boy are concerned because he stutters. A TRUE statement about stuttering is: A. B. C. D. It is indistinguishable from nonfluency of speech It occurs with equal frequency among children and adolescents Anxiety can be a contributing factor in its occurrence It rarely is influenced by parental speech habits 86-Of the following possible explanations for the occurrence of falsenegative results of rapid antigen detection tests for streptococcus, the LEAST likely is A. variability in technique B. low numbers of organisms in infected patients C. low levels of antigen among asymptomatic controls who have positive cultures D. weak antigenicity of the organism 87-A 6 week-old male infant is admitted to hospital with 10-15% dehydration. The serum sodium is 160 mmol/l and the serum potassium is 3.3 mmol/l. THE CORRECT ANSWER IS : A. Congenital adrenal hyperplasia is a likely diagnosis. B. Loss of skin Turgor is usually present. C. The dehydration should be corrected over the next 2-4 hours. D. Bloody diarrhea suggests a bacterial cause. E. Initial fluid replacement should be with D/W 4.3%-0.18N/S. 18 88- Computed tomography of the head in an 8-year-old child with heacache and vomiting reveals hydrocephalus without evidence of tumor. You consider the possibility of a tumor in the brain stem. True statements about intracranial tumors in children include each of the following EXCEPT: A. Less than 15% of intracranial tumors are located in the brain stem B. Hydrocephalus is a common presenting finding of brainstem tumors C. MRI is more helpful than computed tomography in diagnosis. D. Early detection of brainstem tumors does not substantially affect prognosis 89-A 16-year-old boy received radiation therapy for acute lymphoblastic leukemia when he was 4 to 6 years old. His mother is concerned about late effects of the treatment. A TRUE statement about the late effects of antileukemic treatment is: A. Risk of a second malignancy is greater than 25% B. Osteosarcoma is the most common second malignancy C. Neurotoxicity from current protocols involving limited cranial irradiation generally is benign D. Reduced secretion of growth hormone occurs in less than 10% of patients 90-A 2-year-old boy presents with rales, pallor, chronic failure to thrive, recurrent thrush, diarrhea, and oxygen saturation of 84% in room air. Echocardiography demonstrates an enlarged left ventricle with diminished systolic function. Of the following, the blood test MOST likely to establish the diagnosis in this child is: A. B. C. D. E. antibody testing for Epstein-Barr virus antibody testing for human immunodeficiency virus antibody testing for human parvovirus serum carnitine level serum selenium level 91-A 1-day-old term infant develops bilious vomiting and poor feeding. You recall that the differential diagnosis of vomiting is age-related. Of the following, the condition that is MOST likely to cause bilious vomiting in this infant is: A. B. C. D. E. gastric stress ulcer gastroesophageal reflux gastrointestinal food allergy intussusception midgut volvulus 19 92-A 12-year-old girl has had recurrent episodes of scleral icterus, particularly following viral illnesses. She is otherwise well and is taking no medications. Laboratory studies reveal an indirect bilirubin of 58.1 mcmol/L (3.4 mg/dL) and direct bilirubin of 5.13 mcmol/L (0.3 mg/dL). Serum transaminase concentrations, prothrombin time, partial thromboplastin time, and serum ammonia levels all are within normal limits. Of the following, the MOST likely etiology of this girl's hyperbilirubinemia is: A. B. C. D. E. chronic active hepatitis Dubin-Johnson syndrome Gilbert syndrome hepatitis A infection infectious mononucleosis 93-A 3-year-old child was found drinking from a bottle of liquid drain cleaner. Upon evaluation in the emergency department, he appears quite irritable and has difficulty swallowing. Examination of his oral cavity reveals no evidence of burns or ulcerations. Of the following, the MOST appropriate management is: A. B. C. D. E. computed tomography of the abdomen computed tomography of the chest and mediastinum discharge to home on a liquid diet for 48 to 72 hours esophagoscopy within 48 hours of ingestion indirect laryngoscopy within 12 hours of ingestion 94-You are evaluating a 2-month-old infant for microscopic hematuria. He has a history of respiratory distress syndrome and bronchopulmonary dysplasia and currently is receiving caffeine and furosemide. Urinalysis reveals: specific gravity, 1.010; pH, 6.5; 15 to 20 red blood cells; no protein; and 0 to 2 white blood cells. Electrolyte levels are normal Of the following, the MOST likely cause of the hematuria is: A. benign familial hematuria B. hypercalciuria C. multicystic kidney dysplasia D. tumor E. urinary tract infection 20 95-A 4-year-old girl who has presumed minimal change nephrotic syndrome presents at your office because of a recent rapid weight gain. The mother reports that the urine protein determinations have been 4+ for 2 weeks. Physical examination reveals generalized edema, blood pressure of 115/74 mm Hg, and heart rate of 85 beats/ min. Findings on the remainder of the examination are normal. The urine protein is 4+. You prescribe oral prednisone. Two days later the mother reports that the girl's urine is dark, she has not voided for 12 hours, and she has abdominal pain Of the following, the most likely cause of this girl's hematuria and decreased urine output is A. B. C. D. E. acute glomerulonephritis hemorrhagic cystitis pyelonephritis renal vein thrombosis tubulointerstitial nephritis due to steroid therapy 96-Of the following, the class of drugs MOST likely to cause bladder outlet obstruction is: A. B. C. D. E. antiarrhythmics anticholinergics antihistamines antipyretics Antitussive 97-A 10-year-old boy presents for his annual preparticipation sports physical examination. His father has renal failure. On physical examination, the boy's blood pressure is 145/100 mm Hg and pulse is 90 beats/min. Urinalysis reveals 10 to 20 red blood cells per high-power field, and serum creatinine level is 61.9 mcmol/L (0.7 mg/dL). Renal ultrasonography shows enlarged kidneys, with three renal cysts in each. Of the following, the MOST likely diagnosis in this child is: A. B. C. D. E. autosomal dominant polycystic kidney disease autosomal recessive polycystic kidney disease juvenile nephrophthisis medullary sponge kidney multicystic dysplastic kidney disease 98-A 20-month-old child is brought to the emergency department because of fever and irritability and refusal to move his right lower extremity. Physical examination reveals a swollen and tender right knee that resists passive motion. The most important test to confirm the impression of septic arthritis is A. B. C. D. Examination of joint fluid X-ray of the knee Erythrocyte sedimentation rate (ESR) Complete blood count (CBC) and differential E. Blood culture 21 99-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? A. B. C. D. E. Trisomy 21 Trisomy 18 Trisomy 13 Fragile X syndrome William syndrome 100-A fully immunized 2-year-old presents to the emergency room with several days of low-grade fever, barking cough, and noisy breathing. Over the last few hours he has developed a fever to 40°C (104°F) and looks toxic. He has inspiratory and expiratory stridor. The family has not noticed drooling, and he seems to be drinking without pain. Direct laryngoscopy reveals a normal epiglottis. The management of this disease process includes A. B. C. D. E. Intubation and intravenous antibiotics Inhaled epinephrine and oral steroids Inhaled steroids Observation only Oral antibiotics and outpatient follow-up 22