Chronic GranulomatousDiseasewith : A CaseReport Thichosporonosis Disseminated SamonmaNKanngurn,M.D * PaisarParichariktnond,M.D.+ VisanuPootong,M.D.+* Pakit Vichyanond,M.D.++*
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DUn!{Jru INTRODUCTION choniccnidonriousdnede(ccD) i! rn errrcmelydrc iihdired immunodeficicncydisdile
No hnbry of had injury v6
prcdm'jmofredrcPhik'Jkp,}l,oc6a'e6 isrhcnoncomnonbeb al itrfErionbut,{va3ir thc dc, smmd funsar iiredioi i rd/'tdd. parieih, is r m.jor caurcorn.rbidiry 3id rh$e T'.],qaD,',,"s!gseiifs|romm|y
of a. crcalbdrLk,riicphosphMsc,r313u/L. A
wercp{lomcd. He hrd nomal hunoFl rnd @ll rbiornel inroblue'eftzoliun kn (0%)vnh io
in a young bdy wirh ccD trid tichosporotrosis
rh4 rinc, rhi pdj.ni rcqundd.evoBrnodicd yisih
CASE REPORT
i[, !i$ a rfr pmrof ot39.2oc,Thcptrhsra@ es
A r*o yar and$d
nomh old Thii boy
pEsenrn b sirinj Hospnalvirh fevcr,h.p!rcpl.' mmeg.ly,picumoniamd dieh6. At tbd rmc, he alsolad r siaEepisode. Luftbd puicdE Evctrled oily nish prcteinin theCSFmd slish y lor susar meninsris H. ws ftded for prc.trdcdrub€rculous
fiidiiss wciciorcd.Thc *hire bl@dccll cdrdr
rccordinsro dibiderial srsiliviry teding,The
rob. ricmic with hemdocriiof r9%.Boie nmov pureredccll rplair. Ai uLrmsouidoi rhclbdomci
undormm*rclocicil inve*isationfor orhr c,u*s or h.plrGpl.mmegalyd rollowsvDRr, rnri HIV Hjs hd cMv Iite. all of whhh wercnegative. irlcdionsor childhoodmotuhy wA nored.He
prdroosdd {irh idft.Nitrg truhbmor D.dimd.
andrcqunddm.cbanicirvcnrirarioiwirhhEh Fioz. ff$'Jdild?6d.ysofhospiblizdiondcspicvigo
p,.bolqi. d'nd.arioi Pdtuotu fteautopsyes p.lfomed I d'v anerde{h rnd cvdled a hypodenicboyot 9,1s0Fam oi bodywight *nn miB aDeni, BilsEmllv3up.dichl wd ptrent Bo$ pleumlcavirics lymphtd.nop&n\y *.igh.n 30ostrd230c@q Bpecrivelt cut surfe. show.dnultiplDrbsc*sesor2 cr in harimal di,mch (Fisre l). MiciGcopic fiidins shov.-d Nmdus sm0llandlts. e*nuloEs *ih Nmercls sirn. c.Us,mdt ot vhich areor rorei$ bodvrvpe Somegnnllona .otuidcd sm,ll sPhe hvphre bv psudohtphsandy.an rdr ol runsiprcved (Figuc qp Arr 2). ?t r,'@., dsue cDuurcto bc lymph nodesrsketrtron p0ia*rche'1,hilor.
Fiqlz t. Both ltrdss rcv.il.d meked cois.sdoi
rlso showedmulripl.erhnloms wil\ theo*mjsm 1],e livr *.is[ed 950gEmsandnaltcdly cnla4.d .oi'aiiine multipl. c$eatins iodul.s (FisuE r). sd lords otrbidng thcorslii.m (Figurc4).The spled*6 |'gcd,w.jghilgl4ognn:.G6s'nd micd.opj. fiidings *.F tidils a in lneliv* Thc incritre sho*.d diedcd cmuloms in bothsiosa ,nd subnuco$(F'sre 5).Tle cuxurehln $c ruic lesioi yicldedno grcw|nof AFBrdd orhd brd.na Th. patholosical findiigs A describedabove
riqlz
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rtrd mnltimuclarbd c.ftal cs.tliot (lt&Exl0) siad cdls.
tufl!{{u
tiq!2 2r. Hish powr or (A) show.r plconoohlc
loma.(cMsraoo)
Flsrz r setid.d $rtme or dc tivd ave.t.d multiplesm,tl.4.arinsnodul6.
DTSCUSSTON chftiic grrnuromdous di*m (ccD) js a rarcitrhe redimnhodgfrciscy disorddch'mb. rizd bysuredi onenff..rhlcnenins beredsl md rtrisal irloctionsrnd gmnnlonasii nuxiple orgais. I ' Nd rophils,motrGyres dd mkophiga from pdi.ds wirh cGD havc nomal phrgocyric eriviry but h.k rhesbiliryro conen molsut{ orys.i i'o hlsh.energy mchborid G?q) 6ar conriburesroni.rcbial da$.?r pennci.e or eiablc inrac.luh orsanism eicncEd. gFnulom&rous
fii,z
a Microscopicpicrurcor $. Iivft showii! (H&Er400) epirheroidcdh.
B eny,lh. pr$ of phagocyrosis cm bD dhsecrediuo (a) rdhsioi of pirriclesro rhe leukocyre menbrarc (b) pr.i.l. iic.*ion rtrd .earion or rhc phasocrtc va.uol. G) npbE ot lysorofrr snnots wnh r rcl{se or sraiuhr enzym* in'o $c phrclcyric vicuote (d) a bud or o'idiiv. mebboricadivny n,iit66 b), inmae orys.r consurpriotrand hydroe.n pcroiidc prcdodion.,Ph'socyb, fon parienbwirh ccD
F*d? t\,B rh. smll incsnea* of 473interionsrnd 16?sverciirar T'blc 1 Lo.alizdioiald ftequcn.v
pl.ud[s, lung Lms inrcdions(pncunonii brcnchiris,
E.i, nos.aod'hrcd i',rcd,ons(o'iln, somdiris.siiunis, pxrcriris,rhinius) unn,ry trsd idedjon (ur'cml sftmlom4 ieonls)
riked i!hfibicc (6096) maletorcnllchrioirrbouorT:rrft.dirsiosisis
inf.dion.orurib.oi,ar.hdb@nsrrowiroredi'.e
rcpon.dcas.b dqnosedarrhcasddrz ydm.ro casesofccDi , aid io.xscofccD wiurdrsmi
bd*en 1c?lald 1993is in MunichandGoniosdn
tuied crsniidion Dn$niDft d rnchGporonosis jnGdrons krnunconnonburitrdcsrnglytupdncd
rrequentand&.ious be@rialdid rmcil iituriois.
iifilhriois,'ftissrncmid mymrnison.nrefrrcbry
rorar33 pdiens wid r3 dcdhsd.oniig bcrorerhc mlki||gdbyrmphotriciiB,! rrbl intEdioi $d ,. Jarar(r
is rhal.adiig caus
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