Download PGIMER (Post Graduate Institute of Medical Education & Research, Chandigarh) 2020 May Pedia Solved Question Paper
include?
a)Neurologicaldamage
b)Subcutaneousfatnecrosis
c)Pulmonaryhypertension
d)Hyperglycemia
e)Hlpercalcemia
CorrectAnswer-A:B:C
Ans.is'a'i.e.,Neurologicaldamage,'b'i.e.Subcutaneousfat
necrosis&'c'i.e.,Pulmonaryhypertension
Clinicalfeaturesofhypoxicischemicencephalopathy'
oEncephalopathyprogressovertime?
1. Birthto12hoursDecreasedlevelofconsciousness,poortone,
decreasedspontaneousmovement,periodicbreathingor
apnoea,seizures.
2. 12-24hours-4Moreseizures,Apnoeicspells,jitteriness,weakness.
3. After24hoursHypotonia,consciousness,poorfeeding,
brainstemsigns(oculomotor)andpupillarydisturbances.
Hypotoniaisgeneralized,involvesbothlimbsandtrunkandall
musclessimultaneously.
2.Crouzonsyndromeconsistsof:
a)Maxillaryhypoplasia
b)Syndactyly
c)Hydrocephaly
d)Macrocephaly
e)Mandibularprognathism
CorrectAnswer-A:C:E
Ans.is'a'i.e.,Maxillaryhypoplasia,'c'i.e.Hydrocephaly&`e'i.e.
Mandibularprognathism
Crouzonsyndromeisageneticdisordercharacterizedbythe
prematurefusionofcertainskullbones(craniosynostosis).
Thisearlyfusionpreventstheskullfromgrowingnormallyand
affectstheshapeoftheheadandface.
ManyfeaturesofCrouzonsyndromeresultfromthepremature
fusionoftheskullbones:
Wide-set,bulgingeyesandvisionproblemscausedbyshalloweye
sockets
Strabismus
Midfacialhypoplasia
Beakednose:Upperairwayobstructiondevelopssecondarytoseptal
deviation,midnasalabnormalities,choanalabnormalities,and
nasopharyngealnarrowing
Underdevelopedupperjaw(Maxillaryhypoplasia)
Dentalproblemsandhearingloss,whichissometimesaccompanied
bynarrowearcanals.
Openinginthelipandtheroofofthemouth
Mandibularprognathism
3.Followingaresignsofgoodattachment
duringbreastfeeding?
a)Baby'smouthiswideopen
b)Baby'slowerlipisinverted
c)Upperareolaismorevisiblethanlower
d)Baby'schintouchingthebreast
e)Painduringsucking
CorrectAnswer-A:C:D
Ans.is'a'i.e.,Baby'smouthiswideopen,'c'i.e.,Upperareolais
morevisiblethanlower&'d'i.e.,Baby'schintouchingthebreast
Signsofgoodattachmentare
1. Baby'smouthiswideopen,
2. Mostofthenippleandareolainthemouth,onlyupperareolar
visible,notthelowerone,
3. Baby'schintouchesthebreastand
4. Baby'slowerlipiseverted
4.Allaremetaboliccausesofliverdisease
except
a)Histiocytosis
b)Hemochromatosis
c)Gaucher'sdisease
d)Wilsondisease
e)Galactosemia
CorrectAnswer-A
Ans.is'a'i.e.,Histiocytosis
Metabolicliverdiseasescanbeclassifiedinto3categories:
Manifestationsduetohepatocellularnecrosis:
Galactosemia,hereditaryfructoseintolerance,tyrosinemiatype
I,Wilsondisease,Hemochromatosisandal-antitrypsindeficiency.
Cholestaticjaundice:Al-antitrypsindeficiency,Byler'sdisease,
cysticfibrosis,Niemann-Pickdiseaseandsomedisordersof
peroxisomebiogenesis.
Hepatomegaly(disorderswithliverdamage&storagediseases):
Glycogenstoragediseases,cholesterylesterstoragediseaseand,
whenassociatedwithsplenomegaly,lysosomalstoragediseases
(eg:-Gaucherdisease).
5.Trueaboutconstitutionalgrowthdelay-
a)Baselinegrowthhormonedecreased
b)IGF-flevelsislowforchronologicalage
c)Growthdelayonlyoccursafter2-3yearsofage
d)Pubertyspurtisdelayed
e)Finalheightiswithinnormallimits
CorrectAnswer-B:D:E
Ans.(b)IGF-Ilevelsislowforchronologicalage,(d)Puberty
spurtisdelayed,(e)Finalheightiswithinnormallimits
ConstitutionalGrowthDelay
Thesechildrenarebornwithanormallengthandweightandgrow
normallyforfirst6-12monthsoflife.
Theirgrowththenshowsadecelerationsothattheheightand
weightfallbelowthe3rdcentile.
By3yrofage,normalheightvelocitylsresumedandtheycontinue
togrowjustbelowandparalleltothe3rdcentilewithanormalheight
velocity.
Theonsetofpubertyandadolescentgrowthspurtlsalsodelayedin
thesechildrenbutfinalheightiswithinnormallimits.
Boneageislowerthanchronologicalageandcorrespondstothe
heightage.
Historyofdelayedpubertyanddelayedheightspurtisusually
presentinoneorbothparents.
IGF-1levelstendtobelowerchronologicalagebutwithinthenormal
rangeforboneage
Pubertalgrowthspurtisdelayed.
Growthhormoneresponsestoprovocativetestingtendtobelower
thaninchildren
6.Featureofpallidbreathholdingspellin
comparisontocyanoticbreathholding
spell:
a)Morecommonthancyanoticbreathholdingspell
b)Elicitedbypainfulstimulus
c)Bradycardiaisprominent
d)Atropineisgiveninrefractorycases
e)None
CorrectAnswer-B:C:D
Ans.(b)Elicitedbypainfulstimulus.(c)Bradycardiais
prominent,(d)Atropineisgiveninrefractorycases
Breathholdingspells
Breathholdingspellisaparoxysmaleventoccuringin0.1%-5%of
healthychildrenfromtheageof6monthsto6years.
Thenameforthisbehaviourmaybemisnomerinthatitconnotes
prolongedinspiration.Infact,breath-holdingoccursduringexpiration
andisreflexive(notvolitional)innature.
Therearetwomajortypesofbreathholdingspells?
1.Cyanoticform(morecommon):
Temporarydisappearanceoradecreaseinintensityofthesystolic
murmurisusualasflowacrosstherightventricularoutflowtract
diminishes.
Paroxysmalhypercyanoticattacks(hypoxic,"blue,"or"tet"spells)
areaparticularproblemduringthe1st2yearsoflife.
Theinfantbecomeshyperpneaandrestless,cyanosisincreases,
gaspingrespirationsensue,andsyncopemayfollow.
Mostfrequentlyinmorningoninitiallyawakeningorafterepisodesof
vigorouscrying
2.Pallidform:
Triggeredbysuddenfrightorpainorfallingwithaminorinjurytothe
head
Childmaygaspandgiveabriefcry
Childbecomespale,losesconsciousnessandbecomeslimp
Childmaybecomesweatyandmaystiffenandhaveafewbody
jerksorlosebladdercontrol.
Treatment:
Asubgroupofinfantswithbreathholdingspellshaveirondeficiency
anemia.Irontherapymaytreatnotonlytheanemia,butalsothe
breath-holdingspells.
Pallidinfantilesyncopemayrespondtoatropinesulfate,whichis
usedonanongoingbasisifspellsarefrequent,orintermittentlyif
spellsaresituationallypredictable(suchaswithvenepuncture).
7.Whichofthefollowingfindingisnormalin
infant?
a)Papilledemaisrareinraisedintracranialpressure
b)Floppyinfant
c)Strokingpatellartendonofonesideleadstocontractionon
oppositeside
d)Elbowcrossmidlineifpassivelydonebyexaminer
e)Parachutereflex
CorrectAnswer-A:C:E
Ans.(a)Papilledemaisrareinraised,(c)Strokingpatellar
tendonofonesideleadstocontractiononoppositeside(e)
Parachutereflex
Inincreasedintracranialtension(ICT),thereisseparationofthe
cranialsutures,widefontanelsandincreasedheadcircumference.
TheMacewen'sorcrackpotsignindicatesraisedintracranial
pressureaftersuturesandfontanelhaveclosed.
Papilledemaisunusualininfantunlesstheincreaseinintracranial
pressureisveryrapid.
Botulismcausesacuteflaccidparalysisfloppyinfantwhichisnot
anormalfinding.
Thekneejerkinaninfantmayproduceacrossedadductor
response(tappingthepatellartendoninonelegcausescontraction
intheoppositeextremity),which,ifpresent,doesnotbecome
abnormaluntil6-7moofage.
Whentheupperextremityofanormalterminfantispulledgently
acrossthechest,theelbownormallydoesnotquitereachthe
midsternum(scarfsign).Theelbowofahypotoniainfantextends
beyondthemidlinewithease"
Theparachutereflexisdemonstratedbysuspendingthechildbythe
trunkandbysuddenlyproducingforwardflexionasifthechildwere
tofall.Thechildspontaneouslyextendstheupperextremitiesasa
protectivemechanism.Theparachutereflexappearsbeforethe
onsetofwalking.
8.Trueaboutparacetamoltoxicityin
children:
a)HypernatremiaiscommonsideeffectofN-acetylcysteine
b)Liverfailuremayoccurafter3-4days
c)N-acetylcysteineisverylesseffectiveifgivenafter24hourof
paracetamolingestion
d)N-acetylcysteinebegivenorallyorIV
e)Renaldamagealsomayoccur
CorrectAnswer-B:C:D:E
Ans.b.Liverfailuremayoccurafter3-4days;c.N-
acetylcysteineisverylesseffectiveifgivenafter24hourof
paracetamolingestion;d.N-acetylcysteinebegivenorallyorIV;
e.Renaldamagealsomayoccur
Acetaminophenintoxicationlsacommoncauseofacuteliverfailure
inadolescentsandadults.
Acetaminophentoxicityresultsfromtheformationofahighly
reactiveintermediatemetabolite,N-acetyl-p-benzoquenoneimine
(NAPQI).
Theacutetoxicdoseofacetaminophenisgenerallyconsideredto
be>200mg/kglnchildrenyoungerthan12yrofage,asingle
ingestionof>7.5gisconsideredaminimumtoxicdosein
adolescentsandadults.
Adolescentshaveahigherincidenceoftoxicplasmaconcentration
afteringestionthandochildren,andtheirexposuresareoften
associatedwithintentionaloverdose.
AnIVpreparationofNACLsalsoavailable
N-acetylcysteinecancausenausea&vomitinganddiarrhoeaor
constipation.
Rarely,itcancauserashes,fever,headache,drowsiness,lowblood
pressureandliverproblems.
9.Whichvaccineisrecommendedat2years
ofageaccordingtolatestIAPguidelines:
a)MMR
b)Pneumococcalconjugate
c)VaricellaRubella
d)BoosterofTyphoidConjugateVaccine
e)IPVbooster
CorrectAnswer-D
Ans.(d)BoosterofTyphoidConjugateVaccine
Recommendedageatwhichthevaccinesshouldbereceived
andtypeofvaccine:
AGE
VACCINE
AtBirth
HepatitisB
DTaP-Diphtheria,Tetanus,Acellular
Pertussis
IVP-InactivatedPoliovaccine
2months
HepatitisB
Pneumococcalvaccine
HIB-HaemophilusinfluenzaTypeB
Rotavirusvaccine
DTaP
IVP
4months
Pneumococcalvaccine
HIB
Rotavirusvaccine
DTaP
IVP
HepatitisB
6months
Pneumococcalvaccine
HIB
Influenzavaccine**
Rotavirusvaccine
MMR-Measles,Mumps,Rubella
12months
Pneumococcalvaccine
HepatitisA
DTaP
15months
HIB
Varicella
18months
HepatitisA
2years
BoosterofTyphoidConjugateVaccine
DTaP
MMR
4to6yearsofage
IVP
Varicella
Tdap
11yearsofageto
Meningococcalvaccine
adult
HPV(humanpapillomavaccine)
10.Maximumrisktofoetusoccurswhen
maternalinfectionwithrubellaoccurs
duringwhichofthefollowingtime:
a)6-12week
b)12-l8week
c)14-20week
d)20-24week
e)32-36week
CorrectAnswer-A
Ans.(a)6-12week
Ingeneral,theearlierinpregnancyinfectionoccurs,thegreaterthe
damagetothefetus.Maximumdamagetothefetusoccurswhen
infectionisacquiredinthefirsttrimesterofpregnancy.
Duringacuterubellainpregnancy,therateofcongenitalinfectionis
over90%inthe12firstweeksofpregnancy,approximately60%in
weeks13to17,25%inweeks18to24andthenincreasesagain
duringthelastmonthofpregnancy
11.Trueaboutnewborns:
a)Apgarscoreprovideanimmediateestimateofthephysical
conditionofthebaby
b)APGARscoringisdoneatlmin
c)APGARscoringat5minhasnoprognosticvalue
d)NormalrespiratoryrateisRRis30-60breaths/min
e)Normalheartrate>100beats/min
CorrectAnswer-A:B:D:E
Ans.a.Apgarscoreprovideanimmediateestimateofthe
physicalconditionofthebaby;b.APGARscoringisdoneatl
min;d.NormalrespiratoryrateisRRis30-60breaths/min;e.
Normalheartrate>100beats/min
APGAR:
Ababy'sfirsttest
Quickassessmentofthenewborn'soverallwell-being
Givenone-minuteafterbirthandfiveminutesafterbirth
Rates5vitalareas
APGARat1min,indicatorsforneonatalresuscitation
LatertimesAPGARscore(after5minutes)indicatesaboutlong
termneurologicaldamage(notneonatalmortality)
Signs
0
1
2
Heartbeatsper
Absent
Slow(<100)
>100
minute
Respiratoryeffort
Absent
Slow,irregular
Good,crying
Someflexionof
Muscletone
Limp
Activemotion
extremities
No
Reflexirritability
Cryorcough
No
Reflexirritability
response Grimace
Cryorcough
Blueor
Bodypink,extremities Completely
Color
pale
blue
pink
12.Trueaboutcephalohematoma:
a)Maximumatbirththenregress
b)Occursduetoforcepinjurytoperiosteum
c)Edematousswellingofsofttissue
d)Localizedcollectionofbloodbelowperiosteum
e)Mayextendacrossthemidlineandacrosssuturelines
CorrectAnswer-B:D
Ans.(b)Occursduetoforcepinjurytoperiosteum,(d)
Localizedcollectionofbloodbelowperiosteum
Cephalohematoma:
Itiscausedbyinjurytotheperiosteumoftheskullduringlaborand
delivery.
Thisleadstodevelopmentofhemorrhageoveroneorbothparietal
boneswithpalpableedgesappreciatedasthebloodreachesthe
limitsoftheperiosteum.
Itisacollectionofbloodlnb/wthepericraniumand.theflat
bone(subperiosteal)oftheskullusuallyunilateraland,overa
parietalbone
13.Highriskinfantare:
a)Birthorder>3
b)Twinning
c)Birthweight<3kg
d)Failuretogainweightfor3consecutivemonths
e)Artificialfeeding
CorrectAnswer-B:C:E
Ans.(b)Twinning,(d)Failuretogainweightfor3consecutive
months,(e)Artificialfeeding
Highriskinfantrisk
Birthweightlessthan2.5kg
Twins
Birthorder5ormore
Artificialfeeding
Weightbelow70%ofexpectedweight(i.eIIandIIIdegreeof
malnutrition)
Failuretogainweightduringthreesuccessivemonths
ChildrenwithPEM,diarrhoeaWorkingmother/oneparent
14.Trueaboutbreastmilkjaundiceis/are:
a)Appearsafterweek
b)Typicallybilirubinlevelisaround10-20ng/d1
c)Phototherapyisuseful
d)Managedconservatively
e)Diaperstainingispresentingfeature
CorrectAnswer-A:B:C:D
Ans.(a)Appearsafter1week,(b)Typicallybilirubinlevelis
around10-20ng/d1(c)Phototherapyisuseful,(d)Managed
conservatively
BREASTMILKJAUNDICE:
Occurslaterinnewbornperiod,withbilirubinlevelpeakingin6thto
14thdays.
First,atbirth,thegutissterile,andnormalgutfloratakestimeto
establish.
Breastmilkcontains:
GlucuronidaseIncreasedeconjugationandenterohepatic
recirculationofbilirubin.
Highepidermalgrowthfactor(EGF)IncreaseBilirubinuptakein
thegut(enterohepaticcirculation)
Second,breast-milkofsomewomencontains3-alpha-20-beta
pregnanediol.
Itinhibitsuridinediphosphoglucuronicacid(UDPGA)glucuronyl
transferaseresponsibleforconjugationandsubsequentexcretionof
bilirubin.
Inthenewbornliver,activityofglucuronyltransferaseisonlyat0.1-
1%ofadultlevels,soconjugationofbilirubinisalreadyreduced.
Third,lipoproteinlipaseinbreastmilkproducesincreasedFFAthat
inhibithepaticglucuronyltransferase,whichdecreasesconjugation
ofbilirubin.
TREATMENT:
Phototherapy:
Anynewbornwithatotalserumbilirubingreaterthan359mol/l(21
mg/dL)shouldreceivephototherapy
15.RiskfactorforNeuraltubedefectis/are:
a)Diabeticmother
b)MTHFRmutation
c)Antiepilepticdrugintake
d)Methotrexateintake
e)All
CorrectAnswer-E
Ans.E.AllA,DiabeticmotherB,MTHFR
mutationC,AntiepilepticdrugintakeD,Methotrexateintake
ETIOLOGY:
Teratogens-(hyperthermia,sulphas,antihistaminic,nutrition
deficienciesandanticonvulsantsuse)
Moststronglytied=carbamazepine,valproicacid(folateantagon)
Folatedeficiency
16.Whichofthefollowingis/aretrueabout
atrialseptaldefect(ASD):
a)Ostiumprimumismostcommontype
b)Surgeryusuallydonebefore3year
c)Secondheartsound-Wideandfixed
d)Softdelayeddiastolicrumbleatleftlowerleftsternalborder
e)None
CorrectAnswer-B:C:D
Ans.(b)Surgeryusuallydonebefore3year,(c)Secondheart
sound-Wideandfixed,(d)Softdelayeddiastolicrumble
ClinicalmanifestationsofASD
PatientswithASDaregenerallyasymptomatic.
Mildeffortintoleranceandrespiratorytractinfectionmayoccur.
CHFisrare.
Physicalexamination
Parasternalimpulse
Systolicthrillat2ndleftinterspace.
AccentuationofS,duetoloudtricuspidcomponent.
WidesplitandfixedS2.
Ejectionsystolicmurmuratthesecondandthirdleftinterspaces.
Delayeddiastolicmurmuratthelowerleftsternalborder.
ASDwithmitralstenosisLutembachersyndrome.
Chestx-rayinASD
Mildtomoderatecardiomegalyarterysegment.
Prominentpulmonary
Rightatrialandrightventricularenlargement.
Relativelysmallaorticshadow
Plethoriclungfields.
17.Achildispresentingfeaturesofrickets
includingchangesonbonesandhas
hypophosphatemia.Whichofthe
followingistrue:
a)ItiscommonlycausedbyXlinkedrecessivedisorder
b)Normalzoneofprovisionalcalcificationadjacenttothe
metaphysisispresent
c)Thereisdefectofmineralizationofmatrix
d)CRFmaybethecause
e)Renaltubuledysfunctionleadstohypophosphatemia
CorrectAnswer-C:E
Ans.(c)Thereisdefectofmineralizationofmatrix,(e)Renal
tubuledysfunctionleadstohypophosphatemia
X-linked.hypophosphatemicrickets(XLH)inheritedindominant
manner(notrecessive)
Radiologicalchangesarecharacteristicallyseenatmetaphysis.
Thefirstchangeislossofnormalzoneofprovisionalcalcification
adjacenttothemetaphysis.
Rickets,adiseaseofgrowingbone,occursinchildrenonlybefore
fusionoftheepiphyses,andisduetounmineralizedmatrixatthe
growthplates.
RADIOGRAPHICFINDINGS:
Thickeningandwideningofepiphysis
Cuppingandfrayingofmetaphysis
Irregularmetaphysealmargins
Flaringofanteriorendsofribs
Ricketicrosary
Bowingofdiaphysis
18.Corticosteroidsareusefulinwhichofthe
followingpaediatricsdisease:
a)TBmeningitis
b)EndobronchialTB
c)Spinaventosa
d)Acutetuberculouspericardialeffusion
e)Severemiliarytuberculosis
CorrectAnswer-A:D:E
Ans.(A)TBmeningitis;b.EndobronchialTB;(D)Acute
tuberculouspericardialeffusion;(E)Severemiliary
tuberculosis
Corticosteroidsuseinpaediatrics:
Theseareusefulinthetreatmentofsomechildrenwithtuberculosis
disease.
Corticosteroidsdecreasemortalityratesandlong-termneurologic
sequelaeinsomePatientswithtuberculousmeningitisbyreducing
vasculitis,inflammation,and,ultimately,intracranialpressure.
Shortcoursesofcorticosteroids:Endobronchialtuberculosisthat
causesrespiratorydistress,localizedemphysema,orsegmental
pulmonarylesions.
Relievesymptomsandconstrictionassociatedwithacute
tuberculouspericardialeffusion.
Dramaticimprovementinsymptomsoftuberculouspleuraleffusion
andshiftofthemediastinum.
Severemiliarytuberculosishavedramaticimprovementwith
corticosteroidtherapyiftheinflammatoryreactionissoseverethat
alveolocapillaryblockispresent.
19.Eosinophiliainchildrenis/arepresentin:
a)Allergicrhinitis
b)Echinococcosis
c)Rheumatoidarthritis
d)Infectiousmononucleosis
e)Wiskott-Aldrichsyndrome
CorrectAnswer-A:B:C:E
Ans.(A)Allergicrhinitis;(B)Echinococcosis;(C)Rheumatoid
arthritis;(E)Wiskott-Aldrichsyndrome
Commoncausesofeosinophilia:
Acute:
Allergicdisorder:Asthma,atopicdermatitis,urticaria.drug
hypersensitivity,pemphigoid
Parasiticinfestation:Toxocara,ascaris,amebiasis,strongyloidiasis,
filarial,toxoplasmosis,trichinosia,schistosomiasis,malaria,scabies
Fungalinfections:Bronchopulmonaryaspergillosis,coccidiomycosis
Malignancy:Hodgkinlymphoma,Tcelllymphoma,acute
myelogenousleukaemia,myeloproliferativesyndrome
Hypereosinophilicsyndrome
Chronic:
Allergicdisorders:Pemphigus,dermatitisherpetiformis
Autoimmunedisorders:Inflammatoryboweldisease,rheumatoid
arthritis,Myeloproliferativesyndrome,hypereosinophilicsyndrome
,Loefflersyndrome.
Immunodeficiencysyndromes:HyperIgE,WiskottAldrichsyndrome;
Omennsyndrome;graftversushostreaction
Miscellaneous:Thrombocytopeniawithabsentradii;renalallograft
rejection;Addisondisease
20.Syndromenotassociatedwithchildhood
leukaemia:
a)Edwardsyndrome
b)Fanconisyndrome
c)Diamond-Blackanaemia
d)Patausyndrome
e)Kostmannsyndrome
CorrectAnswer-A:D
Ans.(A)Edwardsyndrome;(D)Patausyndrome
RiskfactorsforChildhoodLeukemia
Downsyndrome
Shwachman-Diamondsyndrome
Kostmannsyndrome
Li-Fraumenisyndrome
Fanconisyndrome
Bloomsyndrome.
Klinefeltersyndrome
Turnersyndrome
NeurofibromatosistypeI
Ataxia-telangiectasia
Severecombinedimmunedeficiency
Paroxysmalnocturnalhaemoglobinuria
21.AchildhasvitaminDdeficiencyrickets.
Whichofthefollowingis/arecorrect
regardingbiochemicalchanges:
a)Alkalinephosphatase
b)serumcalcium
c)parathyroidhormone
d)phosphate
e)25-(OH)D3
CorrectAnswer-A:C
Ans.(A)Alkalinephosphatase;(C)parathyroidhormone
BIOCHEMICALREACTIONS:
Serumcalcium:normalorlow
Serumphosphate:low
Alkalinephosphatase:high
Hypophosphatasiashowslowlevelofalk.phosphatase
PTH:High
Hypophophatemia
22.Trueaboutfetalalcoholsyndrome:
a)Increasedbirthweight
b)Normalfinemotordevelopment
c)Normalsocialskilldevelopment
d)Facialabnormalities
e)Intelligencesubnormal
CorrectAnswer-D:E
Ans.(D)Facialabnormalities;(E)Intelligencesubnormal
Fetalalcoholsyndrome
Highlevelofalcoholingestioninpregnancycancausedamageto
fetus,knownasfetalalcoholsyndrome.
Theharmfuleffectsmaybeduetoalcoholitselforduetooneofits
breakdownproducts.Someevidencesuggeststhatalcohol
mayimpairplacentaltransferofessentialaminoacidsandzinc,both
necessaryforproteinsynthesis,whichmayaccountforIUGR.
Characteristicsoffetalalcoholsyndromeinclude:?
IUGR(notlargeproportionatebody)
Microcephaly
Congenitalheartdefects(ASD,VSD)
Mentalretardation
FacialabnormalitiesShortpalpebralfissures,epicanthalfolds,
maxillaryhypoplasia,micrognathia,lowsetears,smoothphiltrum,
thinsmoothupperlip.
Minorjointanomalies
Hyperkineticmovements
23.Feature(s)ofcongenitalrubellasyndrome
include:
a)Cataract
b)ASD
c)Deafness
d)Patentductusarteriosus
e)Alltheabove
CorrectAnswer-A:C:D
Ans.(A)Cataract;(C)Deafness;(D)Patentductusarteriosus
Congenitalrubellasyndrome(CRS)canoccurinadevelopingfetus
ofapregnantwomanwhohascontractedrubella,usuallyinthefirst
trimester.
Triadshows:PDA,cataractanddeafnes
Ifinfectionoccurs0?28daysbeforeconception,theinfanthasa
43%riskofbeingaffected.
Infectionin2ndtrimester?maybedeafnessonly.
>6wks?nomajorabnormalities
Diagnosis:Isolationofvirusincellculturesofthroatsamples,urine
orothersecretions.
DetectionofIgMinsingleserumsampleshortlyafterbirth.
PersistenceofRubellaIgGantibodiesserumbeyond1yearor
risingantibodytitreanytimeduringinfancyinanunvaccinatedchild
24.A4.2kgsbabyborntouncontrolled
diabeticmother.Thefollowingcondition
willnotoccurinthebaby:
a)Hypercalcemia
b)Hypoglycemia
c)Hyperbilirubinemia
d)Polycythaemia
e)Cardiovasculardefects
CorrectAnswer-A
Ans.(A)Hypercalcemia
NeonatalComplication:
Hypoglycaemia
Respiratorydistresssyndrome
Hyperbilirubinemia
Hypocalcemia
Hypomagnesemia.
Polycythemia
Cardiomyopathy
25.Trueaboutcaputsuccedaneum-
a)Oedematousswellingofscalp
b)Marginsareclearlydefined
c)Swellingresolvespontaneouslyafterfewdaysofbirth
d)Swellingremainsforthreemonths
e)Maximumsizeatbirth
CorrectAnswer-A:C:E
Ans.(A)Oedematousswellingofscalp;(C)Swellingresolve
spontaneouslyafterfewdaysofbirth;(E)Maximumsizeat
birth
Caputsuccedaneumisadiffuse,sometimesecchymoticedematous
swellingofthesofttissuesofthescalpinvolvingtheareaPresenting
duringvertexdelivery.
Itmayextendacrossthemidlineandacrosssuturelines.
Theedemadisappearswithinthelstfewdaysoflife.
Moldingoftheheadandoverridingoftheparietalbonesare
frequentlyassociatedandbecomemoreevidentaftercaputhas
receded;theydisappearduringthelstweeksoflife
Analogousswelling,discoloration,anddistortionofthefaceareseen
infacePresentations.
26.Achildhasfeverwithrednessofcheek.
Thecausativeorganismforthiscondition
is:
a)Herpesvirus
b)ParvovirusB-19
c)Adenovirus
d)Rubella
e)Roseola
CorrectAnswer-B
Ans.(B)ParvovirusB-19
Exanthemainfectiosum:
Thecharacteristicrashfirstappearsaserythematousflushingonthe
faceinaslappedcheekappearance(redcheek).
ThemostcommonmanifestationofparvovirusB19iserythema
infectiosum,alsoknownasfifthdisease,whichisabenign,self-
limitedexanthematousillnessofchildhood.
Itwasthe5thinaclassificationschemeofcommonchildhood
exanthems.
Thepreceding4exanthemsweremeasles,scarletfever,rubella,
andFilatov-Dukesdisease(anatypicalscarletfever),withroseola
infantumasthe"sixthdisease
27.Ingestionofwhichofthefollowingmimics
scurvyinchild:
a)Magnesium
b)Potassiumiodide
c)Arsenic
d)Iron
e)Copper
CorrectAnswer-A
Ans.a.Magnesium
Hypermagnesemiaismostprobableanswerbecauseitalsocause
paralysis
Hypermagnesemiainhibitsacetylcholinereleaseatthe
neuromuscularjunction,producinghypotonia,hyporeflexia,and
weakness;paralysisoccursathighconcentrations.The
neuromuscular
28.Inwhichcondition(s),urethralopeningis
neverattipofglanspenis:
a)Epispadias
b)Hypospadias
c)Urethralstricture
d)Bladderexstrophy
e)Alloftheabove
CorrectAnswer-A:B:D
Ans.a.Epispadias;b.Hypospadias;d.Bladderexstrophy
Hypospadias
Acongenitaldisorderoftheurethrawheretheurinaryopeningisnot
attheusuallocationontheheadofthepenis.Itisthesecond-most
commonbirthabnormalityofthemalereproductivesystem,affecting
aboutoneofevery250malesatbirth.
EPISPADIAS
Anepispadiasisararetypeofcongenitalmalformationinwhichthe
urethraopensonthedorsum(theupperaspect)ofthepenis.Itis
oftenpartoftheconditiontermedEpispadias-exstrophyofthe
bladder.Epispadiasisamildformofbladderexstrophy,andin
severecases,exstrophyandepispadiascoexist.
29.Whichofthefollowingtrueaboutnew-
born&children:
a)Erythropoietinlevelismoreinpretermthanterm
b)Erythropoietingiventopretermonlyinsomespecialcases
c)ErythropoietingiventoPretermcancausecomplication
d)a&b
e)Noneoftheabove
CorrectAnswer-B:C
Ans.(B)Erythropoietingiventopretermonlyinsomespecial
cases;(C)ErythropoietingiventoPretermcancause
complication
Mostinfantswithbirthweightof<1KgRBCtransfusions.Akey
reasonwhythenadirhaemoglobinvaluesofprematureinfantsare
lowerthanthoseofterminfantsistheformergroup'srelatively
diminishedplasmaEPOlevelinresponsetoanaemia.
PreterminfantsexhibitasluggishEPOresponsetofalling
haematocritvalues.
LowplasmaEPOlevelproviderationaletheuseofrecombinant
EPOinthetreatmentofanemiaofprematurity.
ProperdosesofEPOandironeffectivelystimulateneonatal
erythropoiesis.However,theefficacyofEPOtherapytosubstantially
diminishtheneedfarRBCtransfusionhasnotbeen
convincinglydemonstrate,particularlyfarsick,extremelypremature
neonates,andrecombinantEPOhasnotbeenwidelyacceptedas
treatmentforanemiaofprematurity.
lnrarecases,somepreparationsofEPOhavebeenassociatedwith
thedevelopmentofanti-EPOantibodiesthatresultinsevereanemia.
30.Macrocephalyisseenin?
a)Soto'ssyndrome
b)GorlinSyndrome
c)Achondroplasia
d)Maternaldiabetes
e)Struge-Webersyndrome
CorrectAnswer-A:B:C:E
Ans.is'a'i.e.,Soto'ssyndrome;'b'i.e.,GorlinSyndrome;'c'
i.e.,Achondroplasia;&'e'i.e.,Struge-Webersyndrome
Syndromes:
Fragile-Xsyndrome
Neuro-cutaneoussyndromes
Tuberoussclerosis
Sturge-Weber
IncreasedCSF:
Hydrocephalus,
Choroidplexuspapilloma.
Bonedisease:
Achondroplasia
Osteogenesisimperfecta
OsteoPetrosis.
Others:
AVmalformation
Intracranialhaemorrhage
Thalassemiamajor
Hypervitaminosis-A
Leadpoisoning
Pseudotumorcerebri
Galactosemia
Canavan'sleukodystrophy.
Overgrowthsyndromes:
Sotosyndrome(Cerebralgigantism)
Weaversyndrome
Simpson-Golabi-Behmelsyndrome(bulldogsyndrome)
Macrocephaly-Capillarymalformation(M-CMTC)syndrome.
Neuro-cardiofacial-cutaneoussyndromes:
Noonansyndrome
Costellosyndrome
Gorlinsyndrome(BasalCellNevussyndrome)
Cardio-facio-cutaneoussyndrome.
Fragile-xsyndrome&leukodystrophies(Alexanderdisease,
Canavandisease).
31.LargeSkullsizeofnewbornisseenin?
a)Fetalalcoholsyndrome
b)Gestationaldiabetes
c)Turner'ssyndrome
d)Canavan'sleukodystrophy
e)Neurofibromatosis
CorrectAnswer-D:E
Ans.is'd'i.e.,Canavan'sleukodystrophy;&'e'i.e.,
Neurofibromatosis
Canavandisease:
Autosomal-recessiveneurologicaldisorderassociatedwith
macrocephalyandspongiformdegenerationofbrain.
Thereiseitheralackofdevelopmentorrapidregressionof
psychomotorfunction,lossofsightandopticatrophy,lethargy,
difficultyinsucking,irritability,reducedmotoractivity,hypotonia
followedbyspasticityofthelimbswithcorticospinalsigns,andan
enlargedhead(macrocephaly).
32.ClinicalfeaturesofB-cellALLinchildren
include:
a)Fever
b)Intrauterinedeath
c)Shock
d)Rigormortis
e)Mediastinalenlargement
CorrectAnswer-A:B:C:D
Ans.is'a'i.e.,Fever;'b'i.e.,Intrauterinedeath'c'i.e.,Shock
&'d'i.e.,Rigormortis
ClinicalmanifestationsofALL:
Symptomsrelatedtodepressionofnormalmarrowfunction.:
Anemia
Neutropenia
Fatigue,pallor
Infection,intermittentlever
Thrombocytopenia
Bleeding,petechiae,ecchymoses,epistaxis.
Bonepainandtenderness
Generalizedlymphadenopathy,splenomegalyandhepatomegaly
Symptomsrelatedtocompressionoflargemediastinalvesselsor
airway
CNSmanifestation:
Headache,vomiting,nervepalsies.
SeriousinfectionsmaycauseSepticshockandlifethreatening
bleeding.
Duetoneutropenia,theremaybeinfectiousdiarrhea.
33.Trueaboutheartsounds/murmurinatrial
septaldefect-
a)WidesplitS,
b)Earlydiastolicmurmur
c)Loudshuntmurmur
d)DelayedP2
e)AttenuationofS1
CorrectAnswer-A:D
Ans.is'a'i.e.,WidesplitS2&'d'i.e.,DelayedP2
ClinicalmanifestationsofASD
PatientswithASDaregenerallyasymptomatic.
Mildeffortintoleranceandrespiratorytractinfectionmayoccur.
CHFisrare.
Physicalexamination
Parasternalimpulse
Systolicthrillat2ndleftinterspace.
AccentuationofS,duetoloudtricuspidcomponent.
WidesplitandfixedS2.
Ejectionsystolicmurmuratthesecondandthirdleftinterspaces.
Delayeddiastolicmurmuratthelowerleftsternalborder.
ASDwithmitralstenosisLutembachersyndrome.
Chestx-rayinASD
Mildtomoderatecardiomegalyarterysegment.
Prominentpulmonary
Rightatrialandrightventricularenlargement.
Relativelysmallaorticshadow
Plethoriclungfields.
34.Trueaboutmurmur(s)inPatentDuctus
arteriosus-
a)Delayeddiastolicmurmur
b)Continuousmurmur
c)ReversesplittingofS2
d)Ejectionsystolicmurmur
e)Middiastolicmurmur
CorrectAnswer-A:B:C:D
Ans.is'a'i.e.,Delayeddiastolicmurmur;'b'i.e.,Continuous
murmur;'c'i.e.,ReversesplittingofS2;&'d'i.e.,Ejection
systolicmurmur
Pressuregradientbetweenaortaandpulmonaryarteryismaintained
throughoutthecardiaccycle(duringsystoleand
diastole)Continuousmurmur,i.e.,murmurstartsinsystoleafter
S1,andreachesapeakatS2.Itthendiminishesandaudibleonlya
partofdiastole.
Largerbloodvolumepassesthroughpulmonarycirculation(blood
fromrightsideofheartplussomebloodfromaorta)Pulmonary
plethorawhichmaycausepulmonaryhypertension.
Increasedflowafterpassingthroughlungreachestheleftatriumand
causesvolumeoverloadLeftatrialdilatationandhypertrophy.
Increasedbloodvolumepassesfromleftatriumtoleftventricle
throughmitralvalve,i.e.,increasedflowthroughmitral
valveAccentuationofS1anddelayeddiastolicmurmur.
Leftventriclereceiveslargeramountofbloodthatresultsinvolume
overloadLeftventricleenlargement.
Extravolumepassesthroughaorticareacausedelayedclosureof
aorticvalvewhichmaycloseevenafterpulmonaryvalve(normal
pulmonaryvalvescloseafteraorticvalves).Paradoxicalsplittingof
S2,i.e.,A,occursafterP.
Largeleftventricularvolumeejectedintotheaortaresultsin
dilatationoftheascendingaortaAorticejectionclick.
LargevolumeofbloodpassesthroughnormalaorticvalveAortic
ejectionsystolicmurmur
35.DifferenceinMurmursofPDAandASD
is/are-
a)DelayedP2inPDA
b)WidesplitofS2inPDA
c)AccentuationofS1inASD
d)ContinuousmurmurinPDA
e)DelayeddiastolicmurmurinASD
CorrectAnswer-D
Ans.is'd'i.e.,ContinuousmurmurinPDA
DelayedP2andwidesplitS2arefeatureofASD(notPDA).
ThereiscontinuousmurmurinPDA.
36.RadiologicalfeaturesofTOFis/are-
a)Cardiomegaly
b)Bootshapedheart
c)Rightsidedaorticarch
d)PulmonaryPlethora
e)Coeuransabot
CorrectAnswer-B:C:E
Ans.is'b'i.e.,Bootshapedheart;'c'i.e.,Rightsidedaortic
arch;&'e'i.e.,Coeuransabot
RadiologicalfeaturesofTOF
Bootshapedheart(Coeuransabot)
Normalheartsize
Oligaemiclungfields
Rightaorticarch(in25%)
37.TrueaboutRETTSyndrome?
a)Macrocephaly
b)Cardiacarrhythmia
c)Seizures
d)Mentalretardation
e)Autisticbehaviour
CorrectAnswer-B:C:D:E
Ans.is'b'i.e.,Cardiacarrhythmia,'c'i.e.,Seizures,'d'i.e.,Mental
retardation&'e'i.e.,Autisticbehaviour
Rett'sSyndrome
Thisisthecharacteristicfeatures,thattheybegintoloosetheir
acquiredskills,e.g.,cognitiveandheadgrowthisnormalduring
earlyperiodafterwhichthereisanarrestofgrowth.
Acquiredmicrocephaly
Mostchildrendeveloppeculiarsighingrespirationswithintermittent
periodsofapneathatmaybeassociatedwithcyanosisBreath
holdingspells.
AutisticbehaviourImpairedsocialinteraction,languageand
communication.
Generalizedtonic-clonicconvulsionsoccurinthemajority.
Feedingdisorderandpoorweightgain
38.Congenitaladrenalhyperplasiaisdueto
deficiencyofenzyme?
a)3?-Hydrorysteroiddehydrogenasedeficiency
b)5?reductase
c)l7?-Hydrorylasedeficienry
d)2l-Hydroxylasedeficiency
e)Aromatase
CorrectAnswer-A:C:D
Ans.(a)3?-Hydrorysteroiddehydrogenasedeficiency;(C)l7?-
Hydrorylasedeficienry(d)2l-Hydroxylasedeficiency
Congenitaladrenalhyperplasia(CAH)
GroupofARdisorder
MCadrenaldisorderinchildhood
Mostcommon21-hydroxylasedeficiency
In21a-hyroxylesedeficiency
Thereisdeficiencyofmineralocorticoids&glucocorticoid.
Thisleadstohypoglycemia,hyponatremia
39.CauseoflowerGIbleedinchildrenofage
>2yearofage
a)TB
b)Meckel'sdiverticulum
c)Aspirin
d)Esophagealvarices
e)Itisbleedingfromasitedistaltoligamentoftreitz
CorrectAnswer-A:B:C:E
Ans.is'a'i.e.,TB;"b'i.e.,Meckel'sdiverticulum'c'i.e.,Aspirin;
'e'i.e.,Itisbleedingfromasitedistaltoligamentoftreitz
LowerGIbleedingisdefinedasbleedingfromasitedistalto
ligamentoftreitz.
In>2years:
Infectiouscolitis
Inflammatoryboweldisease
Tuberculosis
Pseudomembranecolitis
Cowmilkproteinallergy
Uncommon:Amebiasis,cytomegalovirus,neutropeniccolitis
Fissure,Arteriovenousmalformation
Polyposissyndrome
Solitaryrectalulcersyndrome
Meckel'sdiverticulum
Rectalvaricesorcolopathy
NSAIDS
Haemorrhoids;Coagulopathy
Henochschonleinpurpura
40.Whichofthefollowingis/aretrueabout
developmentalmilestonesof2yearsold
child-
a)Canwalkup&downstairswithalternatingfeet
b)Walksupanddownstairs,onestepatatime
c)Ridestricycle
d)Knowsageandsex
e)WeightquadruplesofbirthweightAchildishavingWilson
disease
CorrectAnswer-B:E
Ans.is'b'i.e.,Walksupanddownstairs,onestepatatime;'e'
i.e.,WeightquadruplesofbirthweightAchildishavingWilson
disease
GROSSMOTORMILESTONES:
Age
Milestone
3months Neckholding
5months Rollsover
6months Sittingsupported
8months Sittingwithoutsupport
9months Standswithsupport
12months Standswithoutsupport,Walksbutfalls
15months Walksalone,Creepsupstairs
18months Runs,exploresdrawers
2years
Walksupstairs(babysteps),Jumps
3years
Walksupstairs(alternatefeet),ridestricycle
4years
Hopsononefoot,walksdownstairs(alternatefeet)
FINEMOTORMILESTONES:
Age
Milestone
4months Bidextrousreach
6months Unidextrousreach
9months Immaturepincergrasp
12months Maturepincergrasp
15months Imitatesscribbling,towerof2blocks
18months Scribbles,towerof3blocks
2years
Towerof6blocks,vertical&circularstroke
3years
Towerof9blocks,copiescircle
4years
Copiescross,bridgewithblocks
5years
Copiestriangle
41.Achildispresentedwithmediastinal
mass,swellingofface,dyspnea&stridor,
Nextlineofmanagementis/are
a)Administrationofoxygenwithventimask
b)Tracheostomy
c)Biopsyofmassandfineneedleaspirationcytology
d)Mediastinalradiation
e)Rasburicase
CorrectAnswer-A:B:C
Ans.is.a,i,e.,Administrationofoxygenwithventimask;'b'i.e.,
Tracheostomy`c'Biopsyofmassandfineneedleaspiration
cytology
Thisisacaseofsuperiorvena-cavasyndromeorsuperior
mediastinalcompressionsyndrome.
Itisamedicalemergencyandrequiresimmediatediagnostic
evaluationandtherapy.
Nextlineofmanagementinthegivenpatientincludes:-
Inclinedpositionifpossible
Oxygenwithventimask
Tracheostomy
Biopsyandaspirationcytology
Furosemide
42.Whichofthefollowingstatement(s)is/are
notcorrect-
a)Kayser-fleischer(KF)ringmaybepresentineye
b)Serumceruloplasminis<20mg/dl
c)Hepaticcopperis>250?g/gdryweightofliver
d)Insymptomaticpatients,the24hrurinarycopperexcretionis
lessthan40?g/day
e)Liverbiopsyisofvaluefordeterminingtheextentandseverity
ofliverdisease
CorrectAnswer-D
Ans.(d)Insymptomaticpatients,the24hrurinarycopper
excretionislessthan40?g/day
Wilson'sdisease(Hepatolenticulardegeneration)
DIAGNOSIS:
Thegoldstandardfordiagnosisisliverbiopsywithquantitative
copperassay->concentrationofcopperinaliverbiopsysample>
200?g/gdryweight.
Othertestsare?
1. Serumceruloplasminlevel->low(20mg/dl)
2. KFrings
3. Urinecopperexcretion->increased(>100?g/day)
4. DNAHaplotypeanalysis.
43.Complication(s)ofH.typeoftracheo-
esophagealfistulais/are
a)Dysphagia
b)Aspirationpneumonia
c)Hematemesis
d)Paroxysmsofcoughingorcyanosiswithfeeding
e)Deathusuallyoccurininfancy
CorrectAnswer-B:D
Ans.b)Aspirationpneumonia;d)Paroxysmsofcoughingor
cyanosiswithfeeding
H-Type{Type-E)TEF:
Itaccountsfor4-5%ofallcongenitalTEF'
Commonclinicalfeaturesare:
Recurrentrespiratorysymptom
Paroxysmsofcoughingandcyanosisduringfeeding'
Aspirationduringfeedingwithcyanosis
Abdominaldistension.
Dysphagiaisnotpresentbecauseofpatencyofesophagus
Theremaybeassociatedanomalies
VACTERL(vertebral,anorectal,cardiac,tracheal,esophageal,
renal,radial'limb)syndrome
44.Trueaboutbenignidiopathicneonatal
seizures
a)Calledas5ddayfits
b)Seizureoftenoccurlaterinlife
c)Statusepilepticusmayoccur
d)Familyhistoryisusuallypresent
e)Morecommoninpreterm
CorrectAnswer-A:C
Ans.is'a'i.e.,Calledas5thdayfits;'c'i.e.,Statusepilepticus
mayoccur
BenignNeonatalSeizures(5thdayfits)
Increasinglyrecognizedsyndromecharacterizedbyseizuresinthe
neonatalorinfantileperiod.
2forms:Familialandnonfamilial.
Quitesevere,andstatusepilepticusiscommon.
Nonfamilialformischaracterizedby:
Idiopathic,self-Limitedseizureinpreviouslynormalneonates.
Mostcommonlyoccuratday5
Familialseizures:
Mostfrequentlyhavetheironsetduringthefirstweekoflife,but
onsetmayoccuraslateasearlyinfancy.
Theseseizuresmayrecurforseveralmonthsbeforeresolving.
Thefamilyhistoryrevealsbenignneonatalseizuresinotherfamily
members.
Prognosisisfavourableinbothsyndrome,butseizuresmay
occasionallyoccurlaterinlifeinthefamilialform.
45.Allaretrueaboutwilmstumorexcept-
a)Painlessabdominalmass
b)Mostlyasymptomatic
c)Swellingisballotable
d)StageI-tumourconfinedtokidney
e)CompletelyexcisionistreatmentofchoiceinstageI&II
CorrectAnswer-E
Ans.(e)CompletelyexcisionistreatmentofchoiceinstageI&
II
PresentationofWilm'stumor:
Asymptomaticabdominalmass(mostcommon)
Abdominelswelling(rcnallump)inwilm'stumorisballottable.
Abdominalpain(30%)
Hypertension(25%)
Heamaturia(10-25%)
Fever(20%)
Anorexiaandvomiting
Stages Features
StageI Tumorislimitedtokidenyandcompletelyexcised
Tumorbeyondkidney&completelyexcised.Regional
StageII extensionconfinedtoflank
Residualnon-hematogenoustumorconfinedtothe
abdomen.Lymphnodeinvolvementofhilus,periaortic
chains,orbeyond;diffuseperitonealimplantsoftumor,
StageIII tumorextendsbeyondsurgicalmarginsmicroscopicallyor
macroscopically;tumornotcompletelyremovablebecause
oflocalinfiltrationintovitalstructures
oflocalinfiltrationintovitalstructures
Stage
DepositsbeyondstageIII(e.g.,lung,liver,bone,brain)
IV
StageV Bilateralrenalinvolvementatdiagnosis.
Treatment-
1.Fortumourconfinedtorenalcapsule-
RadicalnephrectomyfollowedbychemotherapywithantinomycinD
andVincristine
2.Fortumourbeyondrenalcapsule
Nephrectomyfollowedbylocalradiotherapyandchemotherapy
TheidealtimingofradiotherapyforWilmsTumouraftersurgeryis
within10days.
3.BilateralWilm'stumour-
Radicalnephrectomyonlargersideoftumourandpartial
nephrectomyonsmallersideoftumour.
46.A5-month-oldinfanthasmassive
hepatomegaly.Whichofthefollowing
condition(s)presentswithmassive
hepatomegaly
a)TypeIglycogenstoragedisorder
b)Biliaryatresia
c)Gaucher'sdisease
d)Biliarycirrhosis
e)Noneoftheabove
CorrectAnswer-A:D
Ans.is'a'i.e.,TypeIglycogenstoragedisorder;'d'i.e.,Biliary
cirrhosis
Causesofmassivehepatomegaly:
Chroniccongestivehepatomegalyeg..,
VSDwithheartfailure(chronic).
Cardiomyopathywithcongestiveheartfailure.
Constrictivepericarditis.
Chronicextrahepaticcholestasise.g.,congenitalbiliaryatresia.
Malaria,kala-azarforlongduration.
Glycogenstoragedisease
Congenitalhepaticfibrosis
Amoebicliverabscess.
Hepatomaorsecondarymalignantdeposits.
Cystsofliver.
Biliarycirrhosis.
47.Syndrome(s)relatedtopaediatricbrain
tumors-
a)Tuberoussclerosis
b)Neurofibromatosis-2
c)Cokaynesyndrome
d)Fanconisyndrome
e)Turcotsyndrome
CorrectAnswer-A:B:E
Ans.is'a'i.e.,Tuberoussclerosis'b'i.e.,Neurofibromatosis-
2;'e'i.e.,Turcotsyndrome
FamilialSyndromesAssociatedwithPaediatricBrainTumors:
NeurofibromatosistypeI
Neurofibromatosistype2
vonHippel-Lindausyndrome
Tuberoussclerosis
Bilateralretinoblastoma
Li-Fraumenisyndrome
Cowdensyndrome
Turcotsyndrome
Gorlinsyndrome
Nevoidbasalcellcarcinoma
48.Commoncauseofdistalsmallintestinal
obstructionininfants/child:
a)Meconiumileus
b)Jejunalatresia
c)Ilealatresia
d)Meckel'sdiverticulum
e)Duodenalatresia
CorrectAnswer-A:B:C:D
Ans.a.Meconiumileu;sb.Jejunalatresia;c.Ilealatresia;d.
Meckel'sdiverticulum
Theprimaryetiologiesofcongenitalsmallbowelobstruction
involves:
Abnormalitiesinanatomicdevelopment(jejunoilealstenosis&
atresia)
Mucussecretion(meconiumileus)
Bowelwallinnervation(long-segmentHirschsprungdisease)
MeckeldiverticulumisthemostcommoncongenitalanomalyofGI
tract
49.Congenitalhyperbilirubinemiais/areseen
in:
a)Prematurity
b)Hypoalbuminaemicstate
c)Hepatitis
d)Sepsis
e)Polycythemia
CorrectAnswer-A:C:D:E
Ans.a.Prematurity;c.Hepatitis;d.Sepsis;e.Polycythemia
Albuminlessthan3.0mg/dlisriskforhyperbilirubinemia
neurotoxicity
Twoothergroupsofdisordersareassociatedwith
hyperbilirubinemia:
(1)Unconjugatedhyperbilirubinemiaseenin,
Breastmilkjaundice
Bloodgroupincompatibility
Lucey-Driscollsyndrome
Congenitalhypothyroidism
Upperintestinalobstruction
Gilbertdisease
Crigler-Najjarsyndrome
Hereditaryspherocytosis
Non-spherocytichemolyticanemia
Drug-inducedhyperbilirubinemia
(2)Conjugatedhyperbilirubinemiapresentin,
Dubin-Johnsonsyndrome
Rotorsyndrome
Biliaryatresia
Neonatalhepatitis
50.ChildcanSwapsobjectfromonehandto
otherin:
a)2-3month
b)5-6month
c)6-12Months
d)12-18Months
e)20-24Months
CorrectAnswer-B
Ans.b.5-6month
Childcantransferobjectsfromonehandtootherby6-7month
Transferobjectshaniltohanilin5'5months
51.VitaminKdependentcoagulationfactor(s)
inchildrenis/are:
a)Factor2
b)Factor7
c)Factor8
d)Factor9
e)Factorl0
CorrectAnswer-A:D:E
Ans.a.Factor2;b.Factor7;d.Factor9;e.Factorl0
Vit.Kcarboxylatesglutamicacidsoftranslationproductsofvitamin
K-dependentproteins,toproduce?-carboxyglutamate
FactorII,VII,IX&XareprocoagulantProenzymeswhereasproteins
C&Sareanticoagulantproenzymes
52.Whichofthefollowingcausesneonatal
jaundice:
a)Sicklecellanaemia
b)?-Thalassemia
c)Meningitis
d)G6PDdeficiency
e)Rhincompatibility
CorrectAnswer-B:D:E
Ans.b.?-Thalassemia;d.G6PDdeficiency;e.Rh
incompatibility
ETIOLOGY:
NonConjugated:Haemolytic:
Intrinsiccauses:
Membraneconditions
Spherocytosis(50%)
Hereditaryelliptocytosis
Systemicconditions
Sepsis
Arteriovenousmalformation
Enzymeconditions
G6PDdeficiency
Pyruvatekinasedeficiency
Globinsynthesisdefect
Sicklecelldisease
Alpha-thalassemia,e.g.HbHdisease
Extrinsiccauses:
Alloimmunity
Hemolyticdiseaseofthenewborn(ABO)
Rhdisease
Hemolyticdiseaseofthenewborn(anti-Kell)
Hemolyticdiseaseofthenewborn(anti-Rhc)
Otherbloodtypemismatches
Non-hemolyticcauses:
Breastmilkjaundice
Cephalohematoma
Polycythemia
Urinarytractinfection
Sepsis
Hypothyroidism
Gilbert'ssyndrome
Crigler-Najjarsyndrome
HighGIobstruction
Conjugated:Hepaticcauses:
Infections
Sepsis
HepatitisA
HepatitisB
TORCHinfectionsverticallytransmittedinfections
T?Toxoplasmosis/Toxoplasmagondii
O?Otherinfections
R?Rubella
C?Cytomegalovirus
H?Herpessimplexvirus-2orneonatalherpessimplex
Metabolic
Galactosemia
Alpha-1-antitrypsindeficiency
Cysticfibrosis
Dubin-JohnsonSyndrome
Rotorsyndrome
Drugs
Totalparenteralnutrition
Idiopathic
Post-hepatic:
Biliaryatresiaorbileductobstruction
Alagillesyndrome
Choledochalcyst
53.Whichofthefollowingmilestoneis
developedbychildb/w6to9month:
a)Canpointsomethingwithindexfinger
b)Swapsomeobjectfromonepalmtoanother
c)Canholdobjectwiththumb&indexfinger
d)Canvoluntarydropobject
e)Canextendarm
CorrectAnswer-B:C:D
Ans.b.Swapsomeobjectfromonepalmtoanother;c.Can
holdobjectwiththumb&indexfinger;d.Canvoluntarydrop
object
6MONTH:
GrossMotor:
Sitsunsupported.
Putsfeetinmouthinsupineposition
Visual-Motor/Problem-Solving:
Unilateralreach.
Usesrakinggrasp
Language:
Babbles
Social/Adoptive
Recognizesstrangers
7MONTH:
GrossMotor:
Creeps
Language:
Orientstobell((localizedindirectly)
8MONTH:
GrossMotor:
Comestosit.
Crawls
Visual-Motor/Problem-Solving:
Inspectsobjects
Language:
"Dada"indiscriminately
Social/Adoptive
Fingerfeeds
9MONTH:
GrossMotor:
Pivotswhensitting.
Pullstostand
Cruises
Visual-Motor/Problem-Solving:
Usespincergrasp
Probeswithforefinger
Holdsbottle,throwsobjects
Language:
"Mama"indiscriminately
Gestures,wavesbye-bye
Inhibitsto"no"
Social/Adoptive
Startstoexploreenvironment
Playsgesturegames(eg,pat-a-cake)
54.Trueaboutosteumprimumdefect:
a)ItisfoundinASD
b)MorecommonlyassociatedwithASDthanVSD
c)MaybeassociatedwithDownsyndrome
d)OsteumprimumASDismorecommonthanosteumsecondum
ASD
e)Alltheabove
CorrectAnswer-A:B:C
Ans.a.ItisfoundinASDb.Morecommonlyassociatedwith
ASDthanVSDc.MaybeassociatedwithDownsyndrome
Themostcommontypeofatrialseptaldefectistheostium
secundumtype.
ChildrenwithDownsyndrome,however,arefrequentlyafflicted
withtheostiumprimumtypeofatrialseptaldefects,whichmaybe
accompaniedbytricuspidandmitralvalvemalformations.
Morecomplexatrioventricularseptaldefectsmayalsooccurinthis
disorder.
Childrenexhibitingtheselesionsshouldbespecificallyevaluatedfor
chromosomalabnormalities.
Clinically,thelesionsproduceleft-to-rightshuntswithlatecyanosis
(aftertherightventriclehypertrophiesinresponsetodevelopinglung
diseasefromtheincreasedbloodflowinthepulmonarysystem).
NeithercysticfibrosisnorGaucherdiseaseisspecifically
associatedwithcardiovasculardefects.
DissectingaorticaneurysmisassociatedwithMarfansyndrome.
55.Vesico-uretericreflexiscommonly
diagnosedby:
a)Micturatingcystography
b)Radioisotoperenography
c)IVU
d)CTscan
e)All
CorrectAnswer-A:B
Ans.a.Micturatingcystography;b.Radioisotoperenography
TherecommendedradiographicevaluationforVURincludesa
VCUG,renal-bladderultrasonographyandnuclearrenalscan
(DMSA).
PerformVCUGandrenal-bladderultrasonographyinanychildwith
documentedUTIbeforeage5years,anychildwithpyelonephritis,
andanymalechildwithasymptomaticUTI.
Arenal-bladderultrasonographymaybeusedtoscreenolder
childrenwithUTI.Ifultrasonographicfindingsareabnormal,conduct
furtherworkupstudieswithVCUGtoruleoutVUR.
Duringtheinitialworkupinapatientwithsuspectedreflux,perform
thestandardVCUG,whichprovidesclearanatomicdetailandallows
accurategradingoftherefluxdegree.Byfillingandemptyingthe
bladderseveraltimes(cycling)withthecatheterstillinthebladder,
asdescribedbyLebowitz,theyieldofidentifyingVURisclearly
enhanced.
Theconventionalcystographyprovidesmoreanatomicalaccuracy
thannuclearcystography;however,nuclearcystographyis
advantageous(usedwidelytomonitorVUR)becauseoflower
radiationexposureandincreasedsensitivity.
56.TrueaboutTannerstageII:
a)Penisincreasesinlength
b)Penisincreasesinwidth
c)Scantyhairatbaseofpenis
d)Darkeningofscrotum
e)Moregrowthoccurinboysthangirls
CorrectAnswer-A:C
Ans.a.Penisincreasesinlength;c.Scantyhairatbaseof
penis
DEVELOPMENT:
Genitals(male):
IllustrationoftheTannerscaleformales.
TannerI
testicularvolumelessthan1.5ml;smallpenis(prepubertal;typically
agenineandyounger)
TannerII
testicularvolumebetween1.6and6ml;skinonscrotumthins,
reddensandenlarges;penislengthunchanged(9?11)
TannerIII
testicularvolumebetween6and12ml;scrotumenlargesfurther;
penisbeginstolengthen(11?12.5)
TannerIV
testicularvolumebetween12and20ml;scrotumenlargesfurther
anddarkens;penisincreasesinlength(12.5?14)
TannerV
testicularvolumegreaterthan20ml;adultscrotumandpenis(14+)
Pubichair(bothmaleandfemale)
TannerI
nopubichairatall(prepubertal)(typicallyage10andyounger)
TannerII
smallamountoflong,downyhairwithslightpigmentationatthe
baseofthepenisandscrotum(males)oronthelabia
majora(females)(10?11.5)
TannerIII
hairbecomesmorecoarseandcurly,andbeginstoextendlaterally
(11.5?13)
TannerIV
adult-likehairquality,extendingacrosspubisbutsparingmedial
thighs(13?15)
TannerV
hairextendstomedialsurfaceofthethighs(15+
57.Pretermbabieshaveincreasedchance
of:
a)Heartdisease
b)Respiratorydistresssyndrome
c)Necrotisingcolitis
d)Meconiumaspirationsyndrome
e)All
CorrectAnswer-A:B:C
Ans.a.Heartdisease;b.Respiratorydistresssyndrome;c.
Necrotisingcolitis
PretermNeonate:
Respiratorysyndrome:Pulmonryoedema,Intra-alveolar
haemorrhage,Idiopathicrespiratorydistresssyndrome&
Bronchopulmonarydysplasia
Infection:Bronchopneumonia,meningitis&necrotizingenterocolitis.
Metabolic(hypoglycemia,hypocalcemia)
Retinopathyofprematurity
Asphxia
Cerebralhaemorrhage
Hypothermia
Fetalshock
HeartfailurePrecipitatedbyasphyxiawithrapiddevelopmentof
pulmonaryedema.TheremaybepatentDuctusarteriosus
Jaundice
Anaemia
58.Whichofthefollowingcanpresentswith
cyanosisatbirth:
a)TetralogyofFallot
b)TGA
c)PDA
d)VSD
e)Atrialseptaldefect(ASD)
CorrectAnswer-A:B
Ans.a.TetralogyofFallot;b.TGA
TOF:Cyanosismaybepresentfrombirthormakeitsappearance
someyearsafterbirth.
TGA:PatientsofcompleteTGAwithintactventricularseptumare
cyanoticatbirth.
PDA,ASD&VSDareacyanoticcondition
59.Whichofthefollowingis/aretrue
regardingdevelopmentofchildduring6
to12yr.ofage:
a)Weightincreasesbyl-2kg/yr
b)Headgrowattherateof2-3cm/yr
c)Growthoccurcontinuously
d)Growthoccurinspurts
e)Heightincreasesatrateof6-7cm/yr
CorrectAnswer-D:E
Ans.d.Growthoccurinspurts;e.Heightincreasesatrateof6-
7cm/yr
PhysicalDevelopmentDuringMiddleChildhood(6-11yrofage)
Growthduringtheperiodaverages3-3.5kg(7lb)and6-7cm(2.5
in)peryear.
Growthoccursdiscontinuously,in3-6irregularlytimedspurtseach
year,witheachgrowthspurtlasting,onaverage,8wk.
Theheadgrowsonly2-3cmincircumferencethroughouttheentire
period,reflectingaslowingofbraingrowth.
60.TrueaboutAspergersyndrome:
a)Morecommoningirl
b)Repetitiveactivitypattern
c)Subnormalintelligenceisconsistentfeature
d)Severelanguageimpairmentsischaracteristic
e)All
CorrectAnswer-B
Ans.b.Repetitiveactivitypattern
Aspergersyndrome:
Itisfourtimesmorelikelytooccurinmalesthaninfemalesand
usuallyisfirstdiagnosedinchildrenbetweentheagesof2and6.
Thecommoncharacteristicsincludeaverageoraboveaverage
intelligence"
Thereisnoclinicallysignificantgeneraldelayinspokenorreceptive
languageorcognitivedevelopment.Self'helpskills,adaptive
behaviour,andcuriosityabouttheenvironmentduringthefirst3
yearsshouldbeatalevelconsistentwithnormalintellectual
development
61.TrueaboutAutisticdisorder:
a)Qualitydecreaseinsocialinteraction
b)Allaffectedchildrenhavesubnormalintelligence
c)TreatmentshouldtargetedtowardSpeechdevelopment
d)Seenonlyafter3yrofage
e)Stereotypedpatternsofbehaviour
CorrectAnswer-A:C:E
Ans.a.Qualitydecreaseinsocialinteraction;c.Treatment
shouldtargetedtowardSpeechdevelopment;e.Stereotyped
patternsofbehaviour
Autisticdisordersarecharacterizedbythetriadofimpaired
socialinteraction,communicationandimagination.Theseare
associatedwithrigidrepetitivepatternofbehaviour.
Theonsetofautisticdisorderalwaysoccursbeforeage3,attwo
peakperiods.
Difficultyinconcentrationandcommunication,
Lesioninfrontalandtemporallobeandcerebellum,
Delayedspeechandlanguagedevelopment,
Problemsinformingsocialrelationshipsinearlychildhood,
Stereotypedmovements,
Poorspeech,
Lackofsocialinteraction,
62.TrueaboutHIVinfectionininfants:
a)Mainlyoccurthroughhorizontaltransmission
b)HIVDNAPCRpositiveat3monthconfirmsdiagnosis
c)PositiveantibodytestforIgGantibodyat3monthconfirm
infectedinfant
d)Passivetransferofmaternalbodygenerallypersistsfor12
month
e)All
CorrectAnswer-B:D
Ans.b.HIVDNAPCRpositiveat3monthconfirmsdiagnosis;
d.Passivetransferofmaternalbodygenerallypersistsfor12
month
AllinfantsborntoHIV-infectedmotherstoantibodypositiveatbirth
becauseofpassivetransferofmaternalHIVantibodyacrossthe
placentaduringgestation.
Mostuninfectedinfantslosematernalantibodybetween6and12
monthofageandareknownasseroreverters.
Becauseasmallproportionofuninfectedinfantscontinuestotest
HIVantibodypositiveforupto18monthofage,positiveIgG
antibodytests,includingtherapidtests,cannotbeusedtomakea
definitivediagnosisofHIVinfectionininfantsyoungerthanthisage.
Inanychild>18monthsofage,demonstrationofIgGantibodyto
HIVbyarepeatedlyreactiveenzymeimmunoassay(EIA)and
confirmatorytest(immunoblotorimmunofluorescenceassay)
establishesthediagnosisofHIVinfection.
Viraldiagnosticassays,suchasHMNAorRNAPC&HIVculture,or
HIVp24antigenimmune-dissociatedp24
By4-6monthsofage,theIIIVcultureand/orPCRidentifuall
infectedinfants.
63.Commonfeatureofmarfansyndrome&
Homocystinuria:
a)Armspan>Height
b)Ectopialentis
c)Hypermobilityofjoint
d)Mentalretardation
e)Arachnodactyly
CorrectAnswer-B:E
Ans.b.Ectopialentis;e.Arachnodactyly
Arachnodactyly:Thisfeaturecanoccuronitsown,withno
underlyinghealthproblems.However,itcanalsobeassociatedwith
certainmedicalconditions.ExamplesincludesMarfansyndrome,
Ehlers-Danlossyndrome,Loeys-Dietzsyndromeand
homocystinuria.
This post was last modified on 11 August 2021