7/21/2019 Pedia Trik http://slidepdf.com/reader/full/pedia-trik-56d9af40bf63a 1/26 Pediatrik 1) (OBQ12.58) Which of the following most accuratel descri!es the cause of osteo"etrosis# $e%iew &o"ic Q' **18 1. ecreased e+"ression of t"e ' collagen ,- (/2*0) 2. ecreased mineraliation of osteoid matri+ - (1*/2*0) ,. 3oss4of4function of 6 al"ha "rotein gene - (1**/2*0) *. 3oss4of4function of car!onic anhdrase '' gene 82- (2,0/2*0) 5. 7utation of $, *- (0/2*0) P$99$$9 $96PO:69 ▶ * Osteo"etrosis is characteried ! defecti%e osteoclastic resor"tion of immature !one caused ! a loss4of4function mutation in the car!onic anhdrase '' gene. ;ar!onic anhdrase '' is an enme that con%erts car!on dio+ide and water into <= and !icar!onate. &he "rotons created are then trans"orted across the ruffled !order of osteoclasts> leading to acidification and demineraliation of !one matri+. &here are ? and ?$ forms of osteo"etrosis> with the ?$ form usuall !eing fatal in the first few ears of life. 6cwart et al. "resent a case re"ort of an infant with osteo"etrosis and no famil histor. iagnosis was made ! generalied increased radiological !one densit and ! a !one !io"s showing "ersistence of calcified cartilage. 7easurement of his erthrocte car!onic anhdrase acti%it re%ealed a deficienc of ;? ''. 'llustration ? shows ;? '' con%erting water and car!on dio+ide to !icar!onate and "rotons within an osteoclast. &he "rotons are then shuttled across the ruffled !order where !one is resor!ed. 'ncorrect ?nswers ?nswer 1 Osteogenesis im"erfecta is associated with a decrease in t"e ' collagen (;O3 1?1 and ;O3 1?2 genes) ?nswer 2 $ickets is associated with a defect in mineraliation of osteoid matri+ caused ! inade@uate calcium and "hos"hate ?nswer , i!rous ds"lasia is associated with a 6 al"ha "rotein mutation ?nswer 5 ?chondro"lasia is associated with a mutation of $, (fi!ro!last growth factor rece"tor ,> on chromosome *P)
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1) (OBQ12.58) Which of the following most accuratel descri!es the cause of osteo"etrosis#
$e%iew &o"ic
Q' **18
1. ecreased e+"ression of t"e ' collagen
,- (/2*0)
2. ecreased mineraliation of osteoid matri+
- (1*/2*0)
,. 3oss4of4function of 6 al"ha "rotein gene
- (1**/2*0)
*. 3oss4of4function of car!onic anhdrase '' gene
82- (2,0/2*0)
5. 7utation of $,
*- (0/2*0)
P$99$$9 $96PO:69 ▶ *
Osteo"etrosis is characteried ! defecti%e osteoclastic resor"tion of immature !one caused
! a loss4of4function mutation in the car!onic anhdrase '' gene.
;ar!onic anhdrase '' is an enme that con%erts car!on dio+ide and water into <= and
!icar!onate. &he "rotons created are then trans"orted across the ruffled !order of osteoclasts>
leading to acidification and demineraliation of !one matri+. &here are ? and ?$ forms of
osteo"etrosis> with the ?$ form usuall !eing fatal in the first few ears of life.
6cwart et al. "resent a case re"ort of an infant with osteo"etrosis and no famil histor.
iagnosis was made ! generalied increased radiological !one densit and ! a !one !io"s
showing "ersistence of calcified cartilage. 7easurement of his erthrocte car!onic
anhdrase acti%it re%ealed a deficienc of ;? ''.
'llustration ? shows ;? '' con%erting water and car!on dio+ide to !icar!onate and "rotonswithin an osteoclast. &he "rotons are then shuttled across the ruffled !order where !one is
resor!ed.
'ncorrect ?nswers
?nswer 1 Osteogenesis im"erfecta is associated with a decrease in t"e ' collagen (;O3
1?1 and ;O3 1?2 genes)
?nswer 2 $ickets is associated with a defect in mineraliation of osteoid matri+ caused !
inade@uate calcium and "hos"hate
?nswer , i!rous ds"lasia is associated with a 6 al"ha "rotein mutation
?nswer 5 ?chondro"lasia is associated with a mutation of $, (fi!ro!last growth factor
;ham!ers et al re%iewed the charts of 2 known sickle cell "atients. ourteen "atients had
an e"isode of osteomelitis or se"tic arthritis. $adiogra"hs and !one scans were not hel"ful
in differentiating infection from an acute !one infarct. 6almonella was the most fre@uent
organism cultured from the osteomelitis cases. &he authors recommend !one as"iration or
!io"s in an sickle cell "atient with e+tremit "ain> swelling> and a fe%er greater than ,8.2degrees ;.
3) (OBQ5.1*) ? 1* ear4old girl has chronic foot "ain which has failed to res"ond to
"re%ious surgical coalition resection and soft tissue inter"osition. ? radiogra"h of her foot is
shown in igure ?. ? ;& scan demonstrates a talocalcaneal coalition with almost com"lete
in%ol%ement of the su!talar Aoint. What is the treatment of choice# $e%iew &o"ic
'D$96 ?
Q' 12
1. re%ision coalition resection and e+tensor digitorum !re%is inter"osition
12- (82/58)
2. re%ision coalition resection and fat inter"osition
C- (**/58)
,. ti!iotalocalcaneal arthrodesis
5- (,/58)
*. talona%icular arthrodesis
*- (2C/58)
5. tri"le arthrodesis
C1- (*C/58)
P$99$$9 $96PO:69 ▶ 5
or sm"tomatic coalition !efore degenerati%e changes ha%e occurred> resection is the usual
treatmentE howe%er> this is not indicated if the "atient has failed "re%ious coalition resection
surger> and has greater than 5- in%ol%ement of the "osterior facet of the su!talar Aoint.
&ri"le arthrodesis in%ol%es fusion of the su!talar> calcaneocu!oid> and talona%icular Aoints
and is the most effecti%e "rocedure for fi+ed hindfoot and forefoot deformities. 6u!talarfusion can !e "erformed in select cases with no significant hindfoot deformit. &his
"rocedure is contraindicated in oung children (1412 ears) !ecause of the limitation it "uts
on foot growth. Wilde et al found fair or "oor results in all ten feet with "reo"erati%e ;&
scans showing an area of relati%e coalition to !e F5- and heel %algus F 1 degrees.
6cranton re"orted consistentl successful resections of talocalcaneal coalitions if the coalition
was less than one4half of the surface area of the talocalcaneal Aoint and there was no
degenerati%e arthritic changes in the talona%icular Aoint.
*) (OBQ*.C*) ?ll of the following are clinical features of neurofi!romatosis t"e ' (:4')
9G;9P&# $e%iew &o"ic
Q' 11C0
1. freckling in the a+illae
*- (1/2C2)
2. o"tic glioma
11- (,/2C2)
,. 2 or more 3isch nodules
,- (8/2C2)
*. tali"es e@uinus
81- (22/2C2)
5. cafe au lait s"ots
1- (,/2C2)
P$99$$9 $96PO:69 ▶ *
:41 is an autosomal dominant disorder> with a""ro+imatel 5- of cases due to a new
mutation. 't is the most common genetic disorder caused ! a new mutation of a single gene.
&he entit is @uite common> affecting 1 in *> and is one of the most common dominantl
inherited gene disorders in humans. ?ccording to the :'< ;onsensus e%elo"ment
;onference 6tatement (108C) the diagnostic criteria for :41 are met in an indi%idual if twoor more of the following are found 1) 6i+ or more cafH4au4lait macules o%er 5 mm in greatest
diameter in "re"u!ertal indi%iduals and o%er 15 mm in greatest diameter in "ost"u!ertal
indi%iduals. 2) &wo or more neurofi!romas of an t"e or one "le+iform neurofi!roma. ,)
reckling in the a+illar or inguinal region. *) O"tic glioma. 5) &wo or more 3isch nodules
(iris hamartomas). )
? distincti%e osseous lesion such as s"henoid ds"lasia or thinning of long !one corte+ with
or without "seudarthrosis. C) ? first4degree relati%e ("arent> si!ling> or offs"ring) with :41 ! the a!o%e criteria. ?ccording to the ;rawford article> common ortho"aedic manifestations
are Is"inal deformit> congenital ti!ial ds"lasia (congenital anterolateral ti!ia !owing and
"seudarthrosis)> and disorders of e+cessi%e !one and soft4tissue growthI.
5 ) (OBQ5.120) igure ? shows a lateral radiogra"h of an 04month olds dorsifle+ed foot.
What is the first line treatment for this condition# $e%iew &o"ic
'D$96 ?
Q' 115
1. O!ser%ation
8- (5*/*)
2. 6erial casting
*C- (,11/*)
,. 7ani"ulation under anesthesia followed ! a single casting
1- (8/*)
*. 6urgical re4alignment
2- (1/*)
5. 6erial mani"ulation and casting followed ! surgical release and talona%icular
reduction with "inning
*1- (2C5/*)
P$99$$9 $96PO:69 ▶ 2
&he radiogra"hs show hindfoot "arallelism !etween the talus and calcaneus which is
characteristic of congenital tali"es e@uino%arus> also known as clu!foot. &his @uestion
em"hasies the im"ortance of the talocalcaneal angle. rom a testing "ers"ecti%e> it is
im"ortant to !e a!le to differentiate clu!foot from congenital %ertical talus (;J&) on
radiogra"hs. Jertical talus will not show the "arallelism !etween the talus and calcaneus seen
with clu!foot and the na%icular (which is not ossified in infants) will not !e aligned with the
talus e%en on attem"ts to reduce with "lantarfle+ion. 7ears angle and the talocalcaneal
angle are shown for clu!foot and %ertical talus in 'llustration ? and B res"ecti%el.
$oe> et al. stated that all radiogra"hs of the foot should simulate weight !earing. &he two
most commonl used measurements are the lateral and ?P talocalcaneal angles> which
dis"la "arallelism.
3aa%eg et al re%iewed the long term treatment of congenital clu!foot and found thatfunctional results were satisfactor in !oth a""earance and function in 88.5- of 1* clu!feet.
6) (OBQ1.1) Which of the following must !e done whene%er a non4am!ulator infant
"resents to the 9$ with a dia"hseal long !one fracture# $e%iew &o"ic
Q' ,80
1. 'mmediate consultation with child "rotecti%e ser%ices and "ossi!le admission to the
hos"ital
00- (1C8/180)
2. Order serum %itamin le%els
- (,/180)
,. Order serum calcium and "hos"horus le%els
- (/180)
*. 7$' of the cer%ical s"ine
- (/180)
5. Perform genetic testing for ;O314?1 and ;O314?2
- (5/180)
P$99$$9 $96PO:69 ▶ 1
9ach of the answers could !e "erformed in this scenario as "art of a diagnostic e%aluation.
<owe%er> missing a case of child a!use could result in further a!use of the child or e%en
death> making this the most im"ortant issue to address.
Banaskiewic et al "erformed a retros"ecti%e re%iew o%er a fi%e ear "eriod on children K 1
ear of age who "resented to the 9$ with a fracture. &he found that 28- of the time> a!use
was underestimated at the time of e%aluation. &he recommend admitting all "atients in thisage grou" with an fracture and consulting child "rotection ser%ices.
8) (OBQ0.1*1) ? ,4ear and 4month old child fell while "laing with friends 2 hours ago
and has a%oided !earing weight on the right leg since that time. &he child is afe!rile and
e+am re%eals tenderness along the distal ti!ial shaft with no significant swelling. $adiogra"hs
are shown in igure ? and B. What is the most a""ro"riate treatment# $e%iew &o"ic
'D$96 ? B
Q' 205*
1. 7$' of the ti!ia
1- (8/1,18)
2. ?s"iration of the ti!ia
- (,/1,18)
,. $eferral to child ser%ices
,- (,,/1,18)
*. 3ong leg cast a""lication
0- (12*/1,18)
5. 6erum %itamin > calcium> and "hos"hate le%els
1- (C/1,18)
P$99$$9 $96PO:69 ▶ *
&he clinical "resentation and radiogra"hs are consistent with a toddlers fracture of the ti!ia
and the ne+t ste" in management includes long leg cast a""lication.
?s descri!ed ! 7ellick et al this scenario is classic for the toddlers fractureE a toddler4aged
child with a%oidance of weight!earing after a fall. &he radiogra"hs demonstrate a non4
dis"laced s"iral fracture of the distal ti!ial shaft. &here are no signs/sm"toms of infection> so7$' and as"iration are not a""ro"riate. 7eta!olic work4u" is not warranted for this common
fracture and found no correlation !etween surgical outcome and inAur mechanism>
dis"lacement> inter%al from inAur to surger> dislocation> fi+ation method> or duration of
immo!iliation. ?ll "atients in this stud had good to e+cellent outcomes.
arsetti et al com"ared "ediatric "atients with medial e"icondle fractures with dis"lacement
F 5mm who recei%ed a long arm cast without reduction> O$'> and e+cision. &he found nodifference in long term results !etween "atients treated with O$' and those who recei%ed
non4o" treatment> while those treated with e+cision had "oor long term results.
13 ) (OBQ8.15) 'n "atients older than 124months of age with de%elo"mental ds"lasia ofthe hi"> all of the following "hsical e+am findings are likel "resent 9G;9P&# $e%iew
&o"ic
Q' 5,
1. 3imited hi" a!duction
,- (2/C80)
2. Positi%e Ortolani maneu%er
8,- (5C/C80)
,. ?!normal leg lengths on aleai testing
2- (18/C80)
*. &rendelen!urg gait
C- (5C/C80)
5. Pel%ic o!li@uit
5- (,/C80)
P$99$$9 $96PO:69 ▶ 2
&here are man e+am maneu%ers which are used in the diagnosis of de%elo"mental ds"lasia
of the hi" (<). 9+am findings differ !ased on the age of the "atient.
Jitale et al em"hasie that "hsical e+am findings associated with < in a child older than
124months can !e different than those seen in the new!orn. 6"ecificall> limited hi"
a!duction> a "ositi%e aleai test> a "ositi%e &rendelen!urg gait> and asmmetr of hi"
a!duction are all useful e+ams tests that are likel to !e "ositi%e. &he Barlow and Ortolani
maneu%ers are of limited use in older children (F months) !ecause the soft tissues a!out the
hi" tighten.
'llustration J demonstrate how the Barlow and Ortolani test are "erformed.
Waters studied the natural histor of !rachial "le+us "als and the effects of treatment
including microsurgical re"air> tendon transfer> and derotational osteotom.
6mith et al e%aluated 1C "atients to determine "rognosticators for reco%er of function in
!rachial "le+o"ath. &he found that "rolonged neurological reco%er or a greater le%el ofinitial inAur were each associated with worse long4term shoulder function.
17 ) (OBQ.0) ? 1,4month4old !o has shortening and deformit of the right leg. ?
clinical "hotogra"h and radiogra"h are "ro%ided in igures ? and B. Which of the following
findings are indicati%e of a good outcome with a ti!iotalar 6me am"utation# $e%iew
&o"ic
'D$96 ? B
Q' 21
1. ,5 degree fle+ion contracture
2- (8/5,2)
2. ?!sence of ti!iofi!ular snostosis
12- (5/5,2)
,. Bilateral in%ol%ement
- (2/5,2)
*. ull functional @uadrice"s strength
85- (*5/5,2)
5. amil histor of ti!ial hemimelia
1- (*/5,2)
P$99$$9 $96PO:69 ▶ *
&he clinical scenario and images demonstrate ti!ial hemimelia. ?n am"utation !elow the
knee has greater likelihood of success if there is a functional @uadrice"s and no fle+ion
contracture of the knee. Otherwise> a knee disarticulation ma !e necessar.
Lalamchi et al re%iew 2* cases of ti!ial hemimelia to esta!lish a classification scheme. &"e '
is total a!sence of the ti!ia. &"e '' is distal a!sence of the ti!ia. &"e ''' is distal deficienc
with ti!iofi!ular diastasis. ?ssessment of the @uadrice"s function and degree of fle+ion
contracture is crucial to following the a""ro"riate treatment o"tion.
Buckle et al re%iewed 85 "ediatric trauma "atients who sustained 05, fractures and
dislocations to determine the relationshi" !etween skeletal inAur and trauma score> inAur
se%erit score> length of hos"ital sta> hos"ital charges> and mortalit. &he femur was the
most commonl fractured !one> and o%erall mortalit was ,- in the grou" studied. &he
trauma "atients with associated sca"ula or "el%ic fractures had an 11- mortalit rate>
whereas those "atients with s"ine fractures had a 1- mortalit rate. emur and ti!ia
fractures had a mortalit rate of 2- and 1- res"ecti%el. &he authors concluded that central
musculoskeletal inAuries (s"ine> cla%icle/sca"ula> and "el%is) were associated with the longest
hos"ital stas and intensi%e care unit admissions> as well as the highest 'nAur 6e%erit6cores> hos"ital charges> and mortalit rates. &e"as et al re%iewed the records of *>*
children with traumatic head inAuries> and com"ared them to head inAuries re"orted from an
adult trauma registr. &he found that o%erall children ha%e a lower mortalit than adults> and
that ;:6 inAur is the "redominant and most common cause of "ediatric traumatic death.
20 ) (OBQ12.,0) ? 284ear4old ?frican4?merican male with a histor of 6ickle ;ell
isease com"lains of "rogressi%e left hi" "ain for the "ast two ears. <e denies an causati%e
inAuries. <is images are shown in igures ? and B. Which of the following mechanisms is
most likel res"onsi!le for his sm"toms# $e%iew &o"ic
'D$96 ? B
Q' *,00
1. Blood disorder due to a!normal hemoglo!in 6 alleles
01- (218/2,0)
2. Progressi%e sli""age of "hsis though the h"ertro"hic one
1- (25/2,0)
,. Osteomelitis most likel due to 6almonella s"ecies
5- (112/2,0)
*. ?ccumulation of glcosaminoglcan !reakdown "roducts
2- (*5/2,0)
5. ;O35?1 or ;O35?2 mutation
1- (10/2,0)
P$99$$9 $96PO:69 ▶ 1
&he clinical "resentation and images are most consistent with left hi" osteonecrosis as a result
of coagulation and %ascular occlusion caused ! sickle cell anemia.
sha"edI and una!le to "ass through %essels. &his results in %ascular occlusion that ma ha%e a
%ariet of clinical conse@uences de"ending on the !od "art affected.
<ernigou et al. re%iew the natural histor of sm"tomatic osteonecrosis in adults with sicklecell disease. Once sm"tomatic > osteonecrosis of the hi" in sickle cell disease has a high
likelihood of "rogressing and leading to femoral head colla"se. eterioration can !e ra"id
and in most "atients o"erati%e inter%ention is necessar to "re%ent further colla"se or
alle%iate intracta!le "ain.
7ont et al. "erformed a literature re%iew on the natural histor of untreated asm"tomatic
osteonecrosis of the femoral head. &heir findings su""orted that asm"tomatic osteonecrosis
had a high "re%alence of "rogression to sm"tomatic femoral head colla"se. 6mall> mediall
located lesions had a low rate of "rogression> while medium to large sied osteonecrotic
lesions did "rogress in a su!stantial num!er of "atients. &he recommended consideration of
Aoint4"reser%ing surgical treatment in asm"tomatic "atients with a medium4sied or large>laterall located lesion.
igure ? shows an ?P "el%is with left hi" osteonecrosis. igure B shows a &2 coronal 7$'
with left hi" osteonecrosis. 'llustration ? shows an e+am"le of a hemoglo!in molecule which
has !ecome Isickle sha"ed>I and as a result is una!le to "ass through %essels efficientl.
'ncorrect ?nswers
?nswer 2 Progressi%e sli""age of the "hsis though the h"ertro"hic one descri!es sli""ed
ca"ital femoral e"i"hsis.
?nswer , &hese radiogra"hs are most consistent with osteonecrosis of the femoral head.
&here is an increased incidence of 6almonella osteomelitis in "atient with 6ickle ;ell
disease> !ut 6ta"hlococcus aureus is still the most common organism.
?nswer * ?ccumulation of glcosaminoglcan !reakdown "roducts descri!es lsosomal
disorders.
?nswer 5 ;O35?1 or ;O35?2 mutation descri!es the mutation of 9hlers anlos
21 ) (OBQ0.28) &he "arents of a wheelchair4!ound 84ear4old !o with cere!ral "als
"resent with difficult during dia"er changes and with hgiene care. <is "hsical e+am
demonstrates 5R of hi" a!duction on the left hi" and 15R on the right. ?n ?P "el%is radiogra"h
is shown in figure ?. What is the most a""ro"riate treatment# $e%iew &o"ic
'D$96 ?
Q' ,21
1. Bilateral !oto+ inAections and "hsical thera"
1- (5/*C)
2. :ighttime Pa%lik harness
2- (8/*C)
,. Bilateral a!ductor release and %algus femoral osteotomies
12- (5/*C)
*. Bilateral adductor release> %arus femoral osteotomies and aceta!ulo"lasties
5- (2*/*C)
5. O!ser%ation with re"eat radiogra"h in months
5- (10/*C)
P$99$$9 $96PO:69 ▶ *
;hildren with s"astic cere!ral "als de%elo" hi" su!lu+ation as the result of long term muscle
im!alance. 6u!lu+ation can "rogress to hi" dislocation with resulting difficulties in seating>hgiene or "ersonal care> and/or hi" "ain. &he "atient has !ilateral dislocated/near dislocated
lnn and 7iller "ro%ide a thorough re%iew of hi" disorders in "atients with cere!ral "als
including etiolog> "hsical e+am and treatment guidelines. $ecommendations included hi"
reconstruction for children o%er * ears of age with se%ere su!lu+ation or dislocation if
se%ere degenerati%e changes are a!sent. <i" reconstruction consists of a one4stage soft4tissuelengthening with %arus derotational femoral osteotom and "ossi!le aceta!ulo"last.
6"iegel and lnn also ha%e "ro%ided a com"rehensi%e re%iew of hi" ds"lasia in "atients
with cere!ral "als. &he article discusses earl inter%ention to "re%ent com"lications
associated with su!se@uent hi" dislocations. 6urgical inter%ention in "atients older than *
ears with hi" dislocations was once again hi" reconstruction with soft4tissue lengthening>
femoral osteotomies and aceta!ulo"lasties in "atients in "atients with marked aceta!ular