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MALIGNANT INFANTILE OSTEOPETROSIS ( A CASE REPORT ) Liveana Sugono, Dian Puspita Sari, Julius Anzar, RM. aisal!

Depart"ent o# C$il% &ealt$, Depart"ent o# Ra%iolog'! a(ult' o# Me%i(ine, Sri)i*a'a +niversit', Dr. Mo$a""a% &oesin &ospital, Pale",ang A,stra(t Background Malignant in#antile osteopetrosis (M-OP) is an autoso"al re(essive osteopetrosis )$i($ is a rare $ere%itar' ,one %isease ($ara(terize% ,' %ense, ,rittle, s(leroti(, ra%iopa.ue ,ones. M-OP generall' results in neurologi(al an% $e"atologi(al a,nor"alities. T$e in(i%en(e is /0122.222 o# ($il%ren. M-OP is %iagnose% in earl' in#an(' an% $as a #atal prognosis. Objective To report a rare (ase o# M-OP. Ca e A 13'ears ol% ,o' )as a%"itte% to our $ospital )it$ pale an% #ailure to t$rive. &e $a% "a(ro(ep$al', #rontal ,ossing, a,nor"al (ranio#a(ial appearan(e, %evelop"ental %ela' an% $epatospleno"egal'. La,orator' investigations reveale%4 &,4 1.5 g0%l, 67C4 /5.8220"" 5, PLT4 58.2220""5, Ca4 /2.9 "g0%l, P4 :.2 "g0%l, ALP4 5;: -+0L, PT& $or"one4 51 pg0"l (nor"al range4 ;3 :/). 7one "arro) aspiration )as planne% ,ut several atte"pts )ere unsu((ess#ul. Osteopetrosis )as suspe(te% an% )$ole ,o%' < ra' ra%iograp$ies )ere o,taine%. &e $a% generalize% osteos(lerosis, =,one )it$in a ,one> appearan(e in t$e verte,ral ,o%ies an% ="as? li?e> appearan(e aroun% t$e or,ita. Sin(e $e $a% t'pi(al ra%iologi(al #in%ings supporting t$e p$'si(al e@a"ination an% la,orator' #in%ings, $e )as %iagnose% as M-OP. Conc!u ion M-OP $as to ,e (onsi%ere% in (ase o# a,nor"alities in (ranio#a(ial ,ones, toget$er )it$ organo"egal' an% ('topenias. Ra%iologi(al #in%ings are usuall' %iagnosti(. "e#$ord 4 Malignant in#antile osteopetrosis, generalize% osteos(lerosis.