svshari grt finalqa

7/28/2019 Svshari Grt Finalqa

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PRELIMINARY WRITTEN EXAM FOR IAPSCON 2011 QUIZ FOR POST-GRADUATES

Name of the post-graduate :

S.No Question Answer

1 Maypole deformity

2 All are True about RTK ( Rhabdoid Tumour of Kidney) except:a. Highly malignant Renal Tumour of Infants & Young children.b. Rhabdomyoblast is the cell of origin.c. Concurrent Brain tumours are frequent.d. Deletion of INI - 1 gene on 22q.

b

3 Syndromic variety of biliary hpoplasia carries a good prognosistrue/ false

false

4 All are True about CMN except : ( Congenital Mesoblastic Nephroma)a. Most Common Renal tumour in infants.b. Mixed CMN is the most common Histological subtype.c. Classic CMN resembles Infantile Fibromatosis.d. Cellular CMN resembles Congenital Fibrosarcoma.

b

This Type III(b) atresia (apple peel, Christmas tree) isalso called as :.


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5 Opsomyoclonus in neuroblastoma carries a good prognosis. True / false true

6 Most common extra-gonadal site for GERM CELL TUMORS is :

.

Sacro-coccygeal

region

7 What is the treatment for trichotilophagy ? counselling

8 Predominantly presacral SCT presenting in late infancy carries greaterrisk of malignancy - True / False ?

True

9 Defici ency of which enzyme causes hype rtension in CAH? 11beta Hydroxylase

10 Introduction of Platinum based chemo for malignant sacro -coccygeal germ

cell tumors has promoted the survival rate to

> 80%

11 Mention one of the risks of alpha 2A Interferon therapy for InfantileHaemo-Endothelioma liver :

Rebound lesions.Spastic diplegia.

12 The floor of Penile Urethra is fully formed by. weeks of intra-uterine life.

11 weeks

13 Radiotherapy effectively controls inoperable Infantile Haemo-Endothelioma Liver. True / False ?

False

14 During Left Upper Lobectomy, Superior Segmental Artery is ligated &divided on the Anterior surface of the Left Pulmonary Hilum. True/False

False

15 Paediatric Surgical Speciality was first established in India in theyea r

1965


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16 Unilateral coronal synostosis leads to ...... Plagiocephaly

17 During the third trimester, urine constitutes what percentage ofamniotic fluid?

(a) 50 % (b) 100 % (c) 70 % (d ) 90 % (e) 10 %

(d) By the thirdtrimester, urine

productionequals 3040 ml/hand comprises up to90 % of amnioticfluid volume.

18 Which of the following ultrasound probeswould be most appropriate toimage the kidney of a large 14-year-old boy?(a) 3 mHz (b) 5 mHz (c) 7.5 mHz (d) 10 mHz (e) Any of the aboveprobes would be appropriate

Answer: (a) The lowerthe mHz of theprobe, the bettertissue penetrance isobtained. In order to

image the kidneys ina large 14-yearoldchild, the 3 Mhzprobe offers the besttissue penetranceand would be thebest choice.

19 All the following are related to Beckwith Weideman Syndrome except :A ExomphalosB Visceromegaly

C HepatoblastomaD Hyperglycemia

D.Hyperglycemia

20 An 18-month-old child is seen in the office for ultrasound. During theexam, an overlying rib obscures the child ' s left kidney and she becomesincreasinglyagitated and uncooperative. Which of the followingpositions is optimal to obtain an image of this kidney?(a) Supine with the probe placed in laterally along the axillary line

(d) The proneposition is the bestposition to image akidney obscured byribs. In an


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(b) Left lateral decubitus position(c) Prone position(d) Have the caregiver hold the child and image the kidney from theprone position

(e) Supine with the probe anteriorly in an intercostal space.

uncooperativeyounger child, thecomfortof being held by the

caregiver will oftencalm the child downand allow a betterexam.

21 Rex shunt is done between .. MesentericoportalRex-shunt as atreatment forextrahepaticportal veinthrombosis

22 A two-month-old child is seen in the office for evaluation of severeunilateral hydronephrosis. You perform an ultrasound . Which of thefollowing findings would be suggestive of a severely obstructed pelvicureteric junction (PUJ)rather than a multicystic dysplastic kidney?(a) An echogenic contralateral kidney(b) Contiguity of the dilated renal pelvis and dilated calyces(c) Involution of the kidney on serial follow-up exams(d) Elongated kidney without reniform shape with multiple cysts(e) All of the above

(b) Thedifferentiationbetween a severelyobstructed kidneyand a multicysticdysplastic kidney(MCDK) can bechallenging. The keydifferential feature isthe contiguity of the

dilated renal pelviswith the dilatedcalyces that is notseen in a MCDK andthe lack of involutionof the severelyobstructed kidney onserial exams.


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Furthermore,additional featuressuggestive of aMCDK include: an

echogenic elongatedkidney withoutreniform shape;multiplenoncommunicatingcysts; a contralateralechogenic kidney;and perhaps mostimportantly,involution of the

MCDK over time.23 Which coagulation factor deficiency may be associated with Wilms tumor

?VWf, vWFactorFactor VII

24 A ultrasound is performed on routine follow-up on a 14-day-old neonatefound to have bilateral prenatal sonographically evident renalpelvis(SERP ). Examination of the bladder ultrasound demonstratesechogenic material within the bladder lumen. Which of the followingis most likely responsible for this finding?(a) Bladder calculi (b) Phosphate crystals (c) Uric acid crystals (d)

Ta mm-Horsfal l protei n (e) Hy percalcu ria

(d) The echogeniccharacteristic of aneonate' s bladder isusually due to the

Tamm-Horsfallprotein. The normal

urine inolder children isecholucent.

25 What is the expected bladder capacity of a 6-year-old child?(a) 140 ml (b) 180 ml (c) 240 ml (d) 280 ml (e) 340 ml

Answer: (c) Theformula to estimatethe bladder capacity


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is as follows: Bladdercapacity (ounces) =age (years) + 2. Toconvert ounces

to ml, one multiplesthe capacity by 30.

Thus, a 6-year-oldchild should have abladder capacity of240 ml (or 8 ounces).

26 All the following are POOR PROGNOSTIC SIGNS in ANORECTALMALFORMATIONSexcept:

a. Abnormal sacrumb. Flat perineumc. Cloacas with common channel > 3 cms.d. Prominent midline groove

d) Prominentmidlinegroove

27 The radioactive tracerproviding the most effective relative measure ofGFR in children more than 5 year of age is:

(a) 99m Tc-diethylenetriaminepentaacetic acid (DTPA) (b) 99m Tc-mercaptoacetyl triglycine (MAG3) (c) 131 I- or 123 I-orthoiodohippurate(OIH) (d) 99m Tc-dimercaptosuccinic acid (DMSA) (e) 131 I radioiodine

Answer: (a) 99m TcDTPA has > 95 %excretionby glomerularfiltration andbecomes a reliablemethod of measuring

relative function oneach side andmeasuring GFR inchildren morethan 5 years of age.DTPA is taken up bythekidney through


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glomerular filtrationand isnot secreted orreabsorbed by the

renal tubules.Once it reaches thekidney, about 20 %isaccumulated and theremainder flowsaway.

28 All of the following are findings of bladder exstrophy by screening fetalultrasoundexcept :

(a) Lower abdominal bulge (b) Low set umbilicus (c) Nonvisualization ofthe bladder (d) Hydronephrosis (e) Small penis

Answer: (d) Studieshave identified five

common prenatalfinding onultrasound tosuggest bladderexstrophy: inabilityto visualizebladder on multipleultrasounds, lowerabdominal bulge,small penis with

anteriorly placedscrotum, low setumbilicus, andabnormal wideningof the iliac crests.Althoughhydronephrosis canbe found in addition


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to bladder exstrophy,it is not indicative ofthe diagnosis.

29 Prune belly syndrome is classically defined by all of the following

abnormalities except:(a) Deficiency of the abdominal wall musculature (b) Hypospadias (c)Bilateral cryptorchidism (d) Dilated, dysmorphic urinary tract

(b) Hypospadias is

not a classifinding associatedwith prune bellysyndrome.

30 Based on Woodard's classification, the majority of patients with prunebelly syndromereside in which category?(a) I (b) II (c) IIa (d) III (e) IV

(d) Woodarddescribed threemajorcategories forchildren with prunebelly syndrome,

with Category III, thelast category, tobe the mildest.Category III patientsareaffected by theexternal abdominalfeatures andundescended testes,but neither

pulmonary norrenal function issignificantlyimpaired.

31 What is the muscle that forms the Passavants Ridge ? Palatopharyngeus


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32 All are true about Zinc Deficiency in Short Bowel Syndrome except:(1) 17mg/L loss in Ileostomy Fluid (2) Acrodermatitis Enteropathica(3) Serum Acid Phosphatase is a surrogate marker

3marker isSerumAlkPO4ase

33 Which is the ImmunoHist ochemical marker for Interstitial Cell of Cajal ? c-Kit

34 Which is the mos t comm on salivary malig nancy in child ren ? MucoEpidermoidCa

35 The most important/immediate factor which affects prognosisin thenewborn with prune bel ly syndrome is :(a) Renal involvement (b) Pulmonary involvement (c) Cardiac involvement(d) Orthopedic abnormalities (e) Gastrointestinal abnormalities.

Answer: (a) Prognosisof a child with prunebelly syndrome isdirectly related torenalfunction and thedegree of renal

dysplasia. Ithas been proposedthat the renaldysplasiaseen in prune bellysyndrome is due tocombinationof a ureteric bud andmetanephricdefect.

36 The most comm on anterior urethralabnormality associated withpatients with prune bel ly syndrome is :(a) Megalourethra (b) Hypospadias (c) Microurethra (d) Urethralduplication (e) Nonobstructing bulbar urethral stricture

Answer: (a) Theanterior urethralabnormalitiesassociated withprune bellysyndrome range fromurethral atresia to


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fusiformmegalourethra. Bothscaphoid andfusiform

megalourethra maybe associated withprunebelly syndrome.

37 The most frequent musculoskeletal/orthopedic abnormalityin patientswith prune be lly syndrome is:(a) Talipes equinovarus (b) Polydactyly (c) Congential dislocation of thehips (d) Syndactyly (e) Scoliosis

Answer: (c)Intrauterinecompression andoligohydramnios maycause lateral dimplesof

the elbows andknees at the milderend of thespectrum, and, mostcommonly,congenitaldislocation of thehips at the severeend.Scoliosis, talipes

equinovarus,polydactyly andsyndactyly are rareanomalies associatedwithprune bellysyndrome.


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38 In regards to ambiguous genitalia which of the following is true ?(a) The most common cause is mixed gonadal dysgenesis (b) The mostcommon enzyme deficiency in congenital adrenal hyperplasia (CAH) is11B-hydroxylase

(c) CAH may be life-threatening secondary to low serum potassium andhigh serum sodium levels (d) 17-hydroxyprogesterone levels will beelevated when CAH is present (e) CAH is the most likely diagnosis whenthe karyotype is 46XX and uterus is not visualized on ultrasound.

Answer: (d) The mostcommon cause ofambi guous genitaliais CAH. The most

commonenzyme deficiencyassociated with CAHis 21-hydoxylasedeficiency. Thesecond mostcommon deficiency is11-hydroxylase.Lastly,3beta-hydroxysteroid

dehydrogenase canalsocause CAH but isextremely rare. CAH,ifunrecognized anduntreated, is lifethreateningin the salt-wastersecondary to poor

feeding,dehydration, andhyperkalemia leadingtoarrhythmias andshock. When thecause is21-hydroxylase,


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blood tests for 17-hydroxyprogesteroneand progesterone willbe elevated.

These tests shouldbe ordered after day2 oflife because theymay be falselyelevated earlyin life from the stressof delivery. 45XXCAH patients willstill have Mllerian

ducalstructures, such as auterus, visible onpelvicultrasound.

39 The most common solid malignancy in the neonate is:(a ) Neuroblastoma (b) Wilms ' tumor (c) Renal cell carcinoma (d)

Congenital mesoblastic nephroma

Answer: (d)Neuroblastoma is themost commonsolid malignancy inthe neonate. Wilms '

tumors are the mostcommon renaltumors inchildren but rarelyoccur in the neonate.Renalcell carcinoma israre in young


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children. However,in children olderthan 10 years of age,presenting with a

solid renal mass, 50% willbe of renal cellorigin. Congenitalmesoblasticnephroma is themost common solidrenalmass in the neonate.Many are detected

prenatallyor in the first monthafter birth. Thecellular variant iscapable ofmetastasis andonce this occurs thechild will requirechemotherapy.

The classic variant is

not capableof metastasis andnephrectomy alone isthetreatment of choice.

40 Anorectal malformations are associated with a urologic abnormality in 2060 % ofpatients. One of these, neuropathic bladder, should be studied early in the evaluationprocess with urodynamics. What is the expected etiology of a neuropathic bladder in

Answer: (a) Whenvertebral anomaliesare


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this population?(a)Tethered cord

(b) Spina bifida(c) Lipomeningocele

(d) Syrinx(e) Myelomeningocele

present, a tetheredspinal cord is themostcommon etiology of a

neuropathic bladderinthis population.Although the otherprocessesmay occur, it is notan anticipatedfinding inthis population.

41 In the newborn nursery on routine examination 5 hours after a prolonged

vaginal delivery, an erythematous, firm and tender right hemiscrotum isdiscovered. On reviewing the delivery examination notes, bo th testeswere thought to be present at birth and the scrotum was nor mal. Thenext step should be:(a) Allow discharge home (b) Observation in the nursery for 24 hours (c)Immediate exploration for suspected testicular torsion (d) Attempt todetorse at the bedside and then obtain an ultrasound (e) Obtain a KUBradiograph

Answer: (c) Neonatal

torsion may presentin the earlypostdelivery timeperiod. It ishallmarkedby a change in thescrotal exam ascompared toimmediate delivery.In this setting,

i.e. < 6 hours,immediateexploration iswarranted. Thepathogenesis is likelyto beextravaginal torsion.Bilateral inguinal


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incisionsare most commonlyused because of theincreased incidence

of a patent process.Also,in rare cases, atesticular tumor isresponsiblefor the torsion of thetesticle.

42 All the following statements regarding breast feeding are true except one

a. Start feeding early on day 1 even in small and sick babiesb. Formula milk is the best food for low birthc. weight neonatesd. initial exclusive breast feeding should be complemented by

complementary foods by six months of agee. None of the above

b)Formula milk isthe best food for lowbirth

weight neonates

43 Most prevalent nutritional deficiency is infants and childrena. Xerophthalmiab. iron deficiency anemiac. Megaloblastic anemiad. Dimorphic anemia

b)iron deficiencyanemia

44 During the evaluation of gross hematuria for a 1-week-old prematureinfant born to a diabetic mother, a renal ultrasound reveals an edematouskidney with minimal but present arterial wave form. A renal scan revealsan enlarged kidney with decreased blood flow and function. The mostlikely diagnosis is:(a) Adrenal hemorrhage (b) Renal artery thrombosis (c) Renal traumasecondary to prolonged vaginal delivery (d) Congenital mesoblastic

Answer: (e) Althoughall of these mayresult in grosshematuria in theneonate,given the abovehistory, renal vein


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nephroma (e) Rena l vein thrombos is. thrombosisis the most likelyetiology. Infants ofdiabetic

mothers may beinitially dehydratedandresult in renal veinthrombosis. A renalultrasoundof renal arterythrombosis typicallydoes notdemonstrate an

edematous kidneyand the renal scanwill reveal nofunctionand no blood flow.

The management ofrenalvein thrombosis mayconsist of hydrationand/

or anticoagulantsdepending on theclinical

45 The diagnosis of Congenital esophageal stenosis (CES) isconfirmed by

a. histopathologic pictureb. narrowing of the esophagus in barium swallow

a.histopathologicpicture, whichmayshow


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47 Opsomyoclonus in neuroblastoma carries a good prognosis. True /false

True

48 All the following statements regarding Microgastria are true except one

a) secondary to defective mesodermal development around the 4thto 5th weeks of intrauterine life

c)Megaesophagusis a commonassociation withmicrogastria and

c. narrowing of the esophagus in esophagoscopyd. Manometric studies fibromusculardisease (FMD) or

tracheobronchialremnants (TBR).

46

Studies frequently employed in the initial evaluation of a child with ahistory of febrile UTIs include all except:(a) Intravenous pyelography (b)Renal and bladder ultrasound (c)Contrast voiding cystourethrog ram in boys (d)Direct radionuclidecystography in girls (e)DMSA renal scintigraphy

Answer: (a)Intravenouspyelography has norole in the evaluationof the child with UTI.Other imagingprotocols, includingthe standardcombination ofcystography and

sonography,or the recent top-down approachthat obtains earlyDMSA renal scans asthefirst-line evaluationtool, are moreappropriatefor evaluating.


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b) presents with recurrent vomiting, respiratory infection andfailure to thrive.

c) Microesophagus is a common associationd) can be managed with Hunt-Lawrence pouch

isthought to besecondary toreflux or to the

esophagustaking over thestomachsreservoircapacity

49 Choledochocele belongs to which type in Todani classification?

50 Kartagener Syndromes is characterized by all the following except :a) dextrocardiab) male infertilityc)

situs inversusd) bronchial adenoma

d.bronchialadenoma

svshari grt finalqa

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