Tuesday, March 25, 2008

20 - Paediatrics Mcqs - 152 to 165

152 - 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

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

153 - 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

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

154 - 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

(Ref: Nelson’s Textbook of Pediatrics 17th Ed, Ch. 148, P-809)

155 - 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


156 - 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

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

157 - 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; prothrombin 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 leukemia. Which of the following is the most likely?
A. Myeloblastic leukemia without maturation
B. Myeloblastic leukemia with maturation
C. Promyelocytic leukemia
D. Myelomonscytic leukemia

*The type of AML, which is most commonly associated with DIC is AML M3.
(Ref: Manual of Pediatric Hematology and Oncology, 4th Ed/P-306, 443)

158 - The defective migration of neural crest cells results in:
A. Congenital megacolon
B. Albinism
C. Adrenogenital hypoplasia
D. Dentinogenesis imperfecta

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

159 - 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


160 - 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


161 - Cushing’s Triad includes all except:
A. Hypertension
B. Bradycardia
C. Hypothermia
D. Irregular respiration

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

162 - All of the following drugs are used for managing status epilepticus except:
A. Phenytoin
B. Diazepam
C. Thiopentone sodium
D. Carbamazepine

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

163 - 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

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

164 - Which organ is the primary site of hematopoiesis in the fetus before midpregnancy?
A. Bone
B. Liver
C. Spleen
D. Lung

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

165 - All of the following are the complications in the new born of a diabetic mother except:
A. Hyperbilirubinemia
B. Hyperglycemia
C. Hypocalcemia
D. Hypomagnesemia

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

McQs in Paediatrics (Pocket MCQs)
Paediatric McQs for Postgraduate Examinations: Practice Examinations
McQs in Paediatrics: With a Tactical Guide on How to Approach Clinical Examinations (PMCQ)

19 - Paediatrics Mcqs - 141 to 151

141 - 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)

142 - 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)

143 - 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)
*Here is a short notes on Juvenile Myoclonic Epilepsy (Janz syndrome)


144 - 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)

145 - 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)

146 - 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)

147 - 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)

148 - 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)

149 - 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

150 - 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.

151 - 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)

18 - Pediatric Pulmonology mcqs - Question 1


1. A 10 year old boy has a 1 year history of cough that is worse at night and with exercise.Which one of the following tests is most likely to assist you to make a diagnosis:

A-Barium swallow.
B-Bronchoscopy.
C-Chest radiograph.
D-Spirometry.
E-Sweat chloride test.

the answer is d . as the diagnosis is in favour of bronchial asthma the answer is d.




Essential Pediatric McQs
MCQs in Pediatrics 2nd edition
MCQs in Pediatrics ; Over 600 Questions with Explanatory Notes

Monday, March 10, 2008

17 - Congenital Dislocation of Hip ( CDH or DDH)


*Synonyms: CDH, Developmental dysplasia of the hip, DDH, dysplasia of the hip

*Developmental dysplasia of the hip (DDH) was formerly referred to as congenital dislocation of hip. DDH is now the preferred term to reflect that DDH is an ongoing developmental process, which is variable in presentation and not always detectable at birth. Developmental dysplasia of the hip refers to a range of developmental hip disorders, from a hip that is mildly dysplastic, concentrically located, and stable, to one that is severely dysplastic and dislocated.

*There is a suggestion that the national screening programme in the UK, that has operated since 1969, has not resulted in any less operations for late disease. Even the use of ultrasound has failed to improve the situation. It may be that the use of more experienced clinicians in the screening programme, rather than very junior doctors, produces better results.


*Epidemiology
-The prevalence of developmental dysplasia of the hip varies with age and method of assessment. In unscreened populations the median prevalence of persistent and clinically diagnosed hip dysplasia is estimated to be 1·3 per 1,000 births.
-The prevalence of neonatal clinical hip instability detected through the Ortolani and Barlow manoeuvres is higher, ranging from 1·6 to 28·5 per 1,000.1
-The left hip is dislocated more often than the right. This is thought to be due to the common position of the baby's left hip against the mother's sacrum, restricting movement.
-It is more common in cultures that use swaddling of babies, forcing the hips into extension and adduction.
-The pictures below show SWADDLING.






*Risk factors:
-There are racial differences. In the USA it is found that native Americans and Laplanders have a high incidence whilst Chinese and African Americans have a low incidence.
-Positive family history of the condition increases the risk in a manner suggestive of multifactorial inheritance.
-About 80 to 85% are female.
-About 60% of cases are first-born, compared with about 40% of all babies.
-Breech presentation increases the risk of DDH. The extended breech position in which knees are extended is a higher risk than with knees flexed. Elective caesarean section reduces the risk compared with vaginal breech delivery but it remains higher than after a cephalic presentation.
-Restriction of movement as with oligohydramnios increases the risk.
-It is commoner with neuromuscular disorders, such as cerebral palsy, meningomyelocele and arthrogryposis.

*Examination :
-The Ortolani and Barlow tests are the most common clinical tests for newborn babies.
-In the Ortolani test, the examiner applies forward pressure to each femoral head in turn, in an attempt to move a posteriorly dislocated femoral head forwards into the acetabulum. Palpable movement suggests that the hip is dislocated or subluxed, but reducible.
-In the Barlow test, backward pressure is applied to the head of each femur in turn, and a subluxable hip is suspected on the basis of palpable partial or complete displacement.
-A clunk feels like turning a light switch on or off. A click is much lighter and probably of no significance.
-Some units insist that the neonatal test should be performed only by an experienced person, usually an orthopaedic surgeon or a paediatrician, and not repeated by others less skilled for fear that numerous tests, perhaps with a little more force than necessary, may encourage instability of the joints.
-An early sign is limitation of hip abduction.

*Children between 1 and 2 months
-True dislocation has occurred and the Ortolani test is used.
-The thigh is flexed and abducted and the femoral head is lifted anteriorly into the acetabulum. Reduction will be felt as a clunk rather than heard as a click. This procedure is impossible after 2 months because soft tissue contractures develop.

*3 to 6 months
-The physical signs are rather different and so are the requirements of examination.
-If the hip is dislocated it is in a fixed position.
-The Galeazzi sign:
o The child is examined lying supine with the hips and knees flexed.
o A positive sign is that one leg appears shorter than the other.
o This is usually due to dislocation of the hip but any discrepancy of limb length will produce a positive sign.
-Other physical signs for late dislocation include asymmetry of the gluteal thigh or labral skin folds, decreased abduction on the affected side, standing or walking with external rotation of the affected leg, and leg length inequality.

*Bilateral dislocation of the hip:
-Can be quite difficult to diagnose, especially after the neonatal period.
-There is often a waddling gait with hyperlordosis.
-The Galeazzi sign for hip shortening is often absent as are asymmetrical thigh and skin folds, or asymmetrically decreased abduction.
-Careful examination is needed with a high level of suspicion.

*Investigations :
-Dynamic ultrasound is used to assess hip stability and acetabular development in infants.
-It is the technique of choice and has even been suggested as a universal screening procedure.
-In older children, x-rays of the pelvis are required.
-Arthrography, CT and MRI may also be needed.

*Management
-Bracing is first-line treatment in children younger than six months. Surgery is an option for children in whom nonoperative treatment has failed and in children diagnosed after six months of age.
-It is important to diagnose developmental dysplasia of the hip early to improve treatment results and to decrease the risk of complications.
-However early detection and treatment does not entirely avoid the need for subsequent surgery, and surgery is needed by up to 5% of infants treated with abduction splinting.
-The indications for the various procedures and the most effective management interventions remain controversial.

*At birth
-It is usually sufficient to maintain the unstable hip in a position of flexion and abduction for one or two months.
-This keeps the femoral head in the correct position and allows time for the ligamentous structures to tighten as well as bones to grow normally.
-The Pavlik harness, Frejka splint and other orthoses are kept in place until the hip is clinically stable with normal ultrasound or X-ray measurements. Controversially, double or triple nappies are often used in newborns for 2 or 3 weeks until splints and harnesses will fit properly.

*Between 1 and 6 months :
-A true dislocation may have occurred, so the aim of treatment is to reduce the femoral head into the acetabulum.
-The Pavlik harness is used to put the hip in the normal position by flexing it at least 90° and preferably 100 to 110°, with relatively full but gentle abduction of 50 to 70°.
-Spontaneous reduction of the femoral head usually occurs within 3 or 4 weeks and the harness is continued until X-ray measurements are normal. This is usually 3 months for dislocation and 2 months for subluxation.
-If this does not occur, closed surgical reduction is attempted with 1 to 3 weeks of preliminary skin traction to place the femoral head opposite the acetabulum, percutaneous adductor tenotomy, closed reduction and a hip spica cast.

*Between 6 months and 2 years :
-Surgical closed reduction may work but, if there is significant instability, an open reduction may be necessary.
-Open reduction is required for children above 2 years of age. A hip spica cast is worn for 6 weeks with a gradual return to full activities.
-Pelvic osteotomies and femoral shortening may be required.

*Complications :
-Surgery can result in a number of complications including re-dislocation, stiffness, blood loss and avascular necrosis of the capital femoral epiphysis (which occurs in 5 to 15% cases).
-Avascular necrosis of the capital femoral epiphysis is caused by compression of cartilage when reducing the femoral head under pressure, which may result in occlusion of intra-articular and extraosseous epiphyseal blood vessels causing infarction.
-Although it revascularises, there may be abnormal growth and development.
-Extreme abduction, especially combined with extension and internal rotation, results in a higher rate of avascular necrosis.



Essential Pediatric McQs
MCQs in Pediatrics 2nd edition
MCQs in Pediatrics ; Over 600 Questions with Explanatory Notes


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