Q-101. Hypoplasia of limbs & scarring is caused due to a) Varicella b) Herpes simplex c) Rubella d) Toxoplasma Answer: Varicella Explanation: Congenital Varicella Syndrome: Limb hypoplasia Cutaneous scars Microcephaly Cortical atrophy Chorio-retinitis Cataracts Q-102. In a child with respiratory distress, failure to thrive. His sweat chloride leve ls were estimated (35 meq/ L and 41 meq/ L). What is next best test to do cystic fibrosis for diagnosis? a) Nasal trans-membrane potential difference b) DNA analysis ?F-508 mutation c) CT chest d) 72 hour fecal fat estimation Answer: Nasal trans-membrane potential difference Explanation: The gene for cystic fibrosis is on the long arm of human chromosome 7 and codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. CFTR can regulate multiple ion channels and cellular processes including: Chloride channels Potassium channels Gap junction channels Bicarbonates ions The most common mutation, called delta F508 is a deletion of three base pairs at position 508 in the gene. The gene mutation leads to defects or deficiency in CFTR causing cell to produce abnormally thick mucus. Clinical findings in newborn: Meconium ileus Failure to thrive Recurrent respiratory distress Elevated immuno-reactive Trypsinogen (Pancreatic enzyme precursor in blood) Sweat chloride > 60 m mmol/L Important point: Initial airways colonization: Staph aureus Most common organism and rapid declination in pulmonary function: Pseudomonas aeruginosa A normal sweat chloride doesn’t exclude the diagnosis. Measurement of nasal trans-membrane potential difference can be useful adjunct in diagnosis of the cystic fibrosis. Q-103. A study under Australian collaborative trial on steroids use in neonates was done. Which of the following is true? a) No difference between placebo & corticosteroid b) Corticosteroid to children causes behavioral worsening c) Corticosteroid to children causes reduction in head circumference d) Corticosteroid to children causes neuro sensitivity degradation
Answer: Corticosteroid to children causes behavioral worsening Explanation: Q-104. International prognostic index for lymphomas includes the following prognostic factors except a) Stage of disease b) Number of extra-lymphatic sites involved c) LDH d) Hemoglobin and albumin Answer: Hemoglobin and albumin Explanation: International prognostic index for lymphomas: The patient’s age The stage of the lymphoma Number of extra-lymphatic sites involved Performance status (PS) Serum LDH Q-105. A child is admitted on 7 days of life with severe respiratory distress and shock. He was discharged 2 days back healthy. What could be the probable diagnosis? a) VSD Large b) Hypoplastic left heart syndrome c) Ebstein anomaly d) AP window defect Answer: Hypoplastic left heart syndrome Explanation: Childs with hypo-plastic left heart syndrome might not have trouble for the first few days of life while the patent ductus arteriosus and the patent foramen ovale (the normal openings in the heart) are open, but quickly develop signs after these openings are closed, including: Problems breathing Pounding heart Weak pulse Ashen or bluish skin color Q-106. Ductus dependent blood flow is required for all of these congenital heart disease except a) Persistent truncus arteriosus b) Hypoplastic left heart syndrome c) Pulmonary stenosis d) TGA with intact ventricular septum Answer: Persistent truncus arteriosus Ductal independence: TAPVR Truncus arteriosus Anomalous origin of the left main coronary artery from the pulmonary artery Q-107. All of the following statements regarding jaundice in a newborn are true except a) Physiological jaundice usually peaks after 48 hours b) Breast milk jaundice usually peaks after day 7 c) High levels of conjugated bilirubin may cause Kernicterus d) All of the above are true Answer: High levels of conjugated bilirubin may cause Kernicterus
Explanation: It is assumed that if the amount of lipid soluble un-conjugated bilirubin exceeds the a vailable bilirubin sites on albumin, free bilirubin will be available to enter neurons and damage them. Clinical jaundice appears after 24 hours of age and peaks after 48 hours. Clinical jaundice is resolved by 1 week in full terms infants and by two weeks in the preterm infants. Breast milk jaundice typically peaks during the second weeks and resolves only very slowly lasting up to three months. Q-108. A child has a vocabulary of 4-6 words however the main mode of communication and social interaction continues to be non-verbal. What is the most likely developmental age of the child? a) 12 Months b) 15 Months c) 18 Months d) 24 Months Answer: 15 Months Explanation: By 15 months of the age, the average child point to major body parts and use 4-6 words spontaneously and correctly. The child also enjoys polysyllabic jargoning. Most communication of wants and ideas continues to be non-verbal. Q-109. The most common bacterial cause for diarrhea in children in India is: a) Entero-toxigenic E. coli (ETEC) b) Entero-pathogenic E. coli (EPEC) c) Entero-hemorrhagic E. coli (EHEC) d) Vibrio cholerae Answer: Entero-toxigenic E. coli (ETEC) Explanation: In India, Rotavirus and Entero-toxigenic E. coli (ETEC) account for nearly half the total diarrhoea episode among the children. Q-110. A child is brought to the pediatric OPD with fever of 24 hours duration. History reveals 3 episodes of chest infection and passage of foul smelling bulky stools. The most probable diagnosis is a) Cystic fibrosis b) Maple syrup disease c) Bilirubin conjugation defect d) Criggler Najjar syndrome Answer: Cystic fibrosis Explanation: The gene for cystic fibrosis is on the long arm of human chromosome 7 and codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. CFTR can regulate multiple ion channels and cellular processes including: Chloride channels Potassium channels Gap junction channels Bicarbonates ions The most common mutation, called delta F508 is a deletion of three base pairs at position 508 in the gene. The gene mutation leads to defects or deficiency in CFTR causing cell to produce abnormally thick mucus. Clinical findings in newborn: Meconium ileus Failure to thrive Recurrent respiratory distress Elevated immuno-reactive Trypsinogen (Pancreatic enzyme precursor in blood) Sweat chloride > 60 m mmol/L
Clinical findings in children Recurrent chest infections Pancreatic exocrine deficiency mainly manifests as Steatorrhoea which is characterized by passage of bulky, foul smelling, pale and greasy stool due to mal-absorption and increased fecal fat con tent. Important point: Initial airways colonization: Staph aureus Most common organism and rapid declination in pulmonary function: Pseudomonas aeruginosa A normal sweat chloride doesn’t exclude the diagnosis. Measurement of nasal trans-membrane potential difference can be useful adjunct in diagnosis of the cystic fibrosis.
