AIIMS 2006(from AIPPG website)

LInk to original source-AIPPG.

 

Conjugated hyperbilirubinemia is seen in:

A. Gilbert’s syndrome

B. Griggler Najjar syndrome

C. Breast milk jaundice

D. Dubin Johnson syndrome

Ans. (D) Dubin Johnson syndrome

(Ref: Nelson’s Textbook of Pediatrics 17th Ed, Ch. 338, P-1321)

A 15-year-old female presented to the emergency department with history of recurrent epistaxis, hematuria and hematochezia. There was a history of profuse bleeding from the umbilicus stump at birth. Previous investigations revealed normal prothrombin time, activated partial thromboplastin time, thrombin time and fibrinogen levels. Her platelet counts as well as platelet function tests were normal but urea c1ot Jt. positive. Which one of the following clotting factor is most likely to be deficient?

A. Factor X

B. Factor XI

C. Factor XII

D. Factor XIII

Ans. (D) Factor XIII

(Ref: Nelson Pediatrics 17th Ed/P-1661)

Which one of the following is the characteristic feature of juvenile myoclonic epilepsy?

A. Myoclonic seizures frequently occur in morning

B. Complete remission is common

C. Response to anticonvulsants is poor

D. Associated absence seizures are present in majority of patients

Ans. (A) Myoclonic seizures frequently occur in the morning

(Ref: Harrison’s Principles of Internal Medicine 16th Ed. 2005—Part XV-Neurologic Disorders;Sec. 2-Diseases of the Central Nervous System;Ch. 348-Seizures and Epilepsy)

Plethoric lung fields are seen in all of the following conditions, except:

A. Atrial septal defect (ASD)

B. TAPVC (Total Anomalous Pulmonary venous connection)

C. Ebstein’s anomaly

D. Ventricular septal defect

Ans. (C) Ebstein’s anomaly

(Ref: Review of Radiology 3rd Ed/p-42-43)

Which of the following is an example of disorders of sex chromosomes?

A. Marfan’s syndrome

B. Testicular feminization syndrome

C. Klinefelter’ s syndrome

D. Down’s syndrome

Ans. (C) Klinefelter’ s syndrome

(Ref: Robbins and Cotrans’s Pathologic Basis of Disease 7th Ed/P-145)

Which of the following haemoglobin (Hb) estimation will be diagnostically helpful in a case of beta thalassemia trait?

A. Hb-F B. Hb1C

C. Hb-A2 D. Hb-H

Ans. (C) Hb-A2

(Ref: Nelsons Pediatrics 17th Ed/P-1633)

Which of the following circulating antibodies has the best sensitivity and specificity for the diagnosis of celiac disease?

A. Anti-endomysial antibody

B. Anti-tissue transglutaminase antibody

C. Anti-gliadin antibody

D. Anti-reticulin antibody

Ans. (A) Anti-endomysial antibody

(Ref: Nelsons Pediatrics 17th Ed/P-1265)

A couple has two children affected with tuberous sclerosis. On detailed clinical and laboratory evaluation (including molecular studies) both parents are normal. Which one of the following explains the two affected children in this family?

A. Non penetrance

B. Uniparental diasomy

C. Genomic imprinting

D. Germline mosaicism

Ans. (D) Germline Mosaicism

(Ref: Journal of Child Neurology/Vol. 19, No. 9, Sept. 2004)

Cardiomyopathy may be seen in all of the following except:

A. Duchenne muscular dystrophy

B. Friedreich’s ataxia

C. Type II glycogen storage disease

D. Alkaptonuria

Ans. (D) Alkaptonuria

Enzyme replacement therapy is available for which of the following disorders?

A. Gaucher disease

B. Niemann Pick disease

C. Mucolipidosis

D. Metachromatic leukodystrophy

Ans. (A) Gaucher’s disease (repeat)

In a child with acute liver failure, the most important prognostic factor for death is:

A. Increasing transaminases

B. Increasing bilirubin

C. Increasing prothrombin time

D. Gram negative sepsis

Ans. (C) Prothrombin time

(Ref: Diseases of the liver and the biliary system 11th Ed, Ch. 8-Acute Liver Failure, P-118)

Which of the following does not establish a diagnosis of congenital CMV infection in a neonate?

A. Urine culture of CMV

B. IgG CMV antibodies in blood

C. Intra-nuclear inclusion bodies in hepatocytes

D. CMV viral DNA in blood by polymerase chain reaction

Ans (B) IgG CMV antibodies in blood

(Ref: Cloherty’s Manual of Neonatal Care 5th Ed/P-257)

All of the following are true of β thalassemia major, except:

A. Splenomegaly

B. Target cells on peripheral smear

C. Microcytic hypochromic anemia

D. Increased osmotic fragility

Ans. (D) Increased osmotic fragility

(Ref: Manual of Pediatric Hematology and Oncology, 4th Ed/P-184)

Transient synovitis (toxic synovitis) of the hip is characterized by all of the following, except:

A. May follow upper respiratory infection

B. ESR and white blood cell counts are usually normal

C. Ultrasound of the joint reveals widening of the joint space

D. The hip is typically held in adduction and internal rotation

Ans. (D) The hip is typically held in adduction and internal rotation.

(Ref: Nelson’s Textbook of Pediatrics 17th Ed, Ch. 148, P-809)
A 3-year-old boy presents with fever, dysuria and gross hematuria. Physical examination shows a prominent suprapubic area which is dull to percussion. Urinalysis reveals red blood cells but no proteinuria. Which of the following is the most likely diagnosis?

A. Acute glomerulonephritis

B. Urinary tract infection

C. Posterior urethral valves

D. Teratoma

Ans. (B) Urinary tract infection

Which of the following statements is true of primary grade IV-V vesicoureteric reflux in young children?

A. Renal scarring usually begins in the midpolar regions

B. Postnatal scarring may occur even in the absence of urinary tract infections

C. Long-term outcome is comparable in patients treated with either antibiotic prophylaxis or surgery

D. Oral amoxicillin is the choice antibiotic for prophylaxis

Ans. (B) Postnatal scarring may occur even in the absence of urinary tract infections.

(Ref: Nelson’s Textbook of Pediatrics 17th Ed—Ch. 531-Vesicoureteric Reflux, P-1791-1793)

15-year-old boy presented with one day history of bleeding gums, subconjunctival bleed and purpuric rash. Investigations revealed the following results:

Hb-6.4 gm/dL; TLC-26,500/mm3 Platelet-35,000/mm3; prathrombin time–20 sec with a control of 13 sec; partial thromboplastin time-50 sec; and Fibrinogen 10 mg/dL. Peripheral smear was suggestive of acute myeloblastic leukernice. Which of the following is the most likely?

A. Myeloblastic leukemia without maturation

B. Myeloblastic leukemia with maturation

C. Promyelocytic leukemia

D. Myelomonscytic leukemia

Ans. (C) Promyelocytic leukemia

(Ref: Manual of Pediatric Hematology and Oncology, 4th Ed/P-306, 443)

The defective migration of neural crest cells results in:

A. Congenital megacolon

B. Albinism

C. Adrenogenital hypoplasia

D. Dentinogenesis imperfecta

Ans. (A) Congenital megacolon

(Ref: Schwartz’s Principles of Surgery 8th Ed. 2005— Part II- Specific Considerations; Ch. 38-Pediatric Surgery)

A premature infant is born with a patent ductus arteriosus. Its closure can be stimulated by administration of:

A. Prostaglandin analogue

B. Estrogen

C. Anti-estrogen compounds

D. Prostaglandin inhibitors

Ans. (D) Prostaglandin inhibitors

The loading dose of Aminophylline is:

A. 50-75 ug/kg

B. 0.5-1.0 mg/kg

C. 2.0-3.5 mg/kg

D. 5-6 mg/kg

Ans. (D) 5-6 mg/kg

Cushing’s Triad includes all except:

A. Hypertension

B. Bradycardia

C. Hypothermia

D. Irregular respiration

Ans. (C) Hypothermia

(Ref: Current Pediatric Diagnosis and Treatment 17th Ed. 2005—Ch. 11-Emergencies and Injuries)

All of the following drugs are used for managing status epilepticus except:

A. Phenytoin

B. Diazepam

C. Thiopentone sodium

D. Carbamazepine

Ans. (D) Carbamazepine

(Ref: Current Pediatric Diagnosis and Treatment 17th Ed. 2005—Ch. 23-Neurologic and Muscular Disorders; Table 23.9-Status epilepticus treatment)

Administration of glucose solution is prescribed for all of the following situations except:

A. Neonates

B. Child of a diabetic mother

C. History of unconsciousness

D. History of hypoglycemia

Ans. (C) History of unconsciousness

(Ref: Rudolph’s Pediatrics 21st Ed. 2003—24. The Endocrine System; 24.9-Hypoglycemia)

Which organ is the primary site of hematopoiesis in the fetus before midpregnancy?

A. Bone

B. Liver

C. Spleen

D. Lung

Ans. (B) Liver

(Ref: Nelson’s Textbook of Pediatrics 17th Ed/
P-1599)

All of the following are the complications in the new born of a diabetic mother except:

A. Hyper bilirubinemia

B. Hyperglycemia

C. Hypocalcemia

D. Hypomagnesemia

Ans. (B) Hyperglycemia

(Ref: Cloherty’s Manual of Neonatal Care 5th Ed./P-13-1