18_fibrosi cistica (mucoviscidosi) optimized

7/25/2019 18_Fibrosi Cistica (Mucoviscidosi) Optimized

1/15

HNO_M\N LN\YNLF (EZLMXN\LNIM\N)

Lystnl ormsnsIfrnf _ussm, \jrjdf Vufttrulln

IJHNDNQNMDJ

@f ormsn lnstnlf (HL) `f pn hrjqujdtj ef`fttnf bjdjtnlf ij``f rfzzfonfdlf. Lm`pnslj b`n jpntj`n sjljrdjdtn lmd efdnhjstfznmdn l`ndnlgj prj-vf`jdtjejdtj `mlf`nzzftj f``fppfrftm rjspnrftmrnm, ndtjstndm, pfdlrjfs,vnj on`nfrn, imttn ijhjrjdtn.

NDLNIJDQF

@ndlnijdzf in 61?:33 dftn vnvn j f hrjqujdzf ij` pmrtftmrj sfdm in 61?:.

JQNMSFYMBJDJ\N J HN\NMSFYM@MBNF

udf ef`fttnf futmsmenlf rjljssnvf `f lun ndsmrbjdzf imvutf f``jeutfznmdn in ud bjdj in ?:3333 dul`jmtnin lgj sn trmvf su` lrmemsmef2 j lgj lminlf pjr ud _DF ejssfbbjrm in 8:33 dul`jmtnin lgj, fsuf vm`tf, prmiulj udf prmtjndf in 6973 fendmflnin lgnfeftf _jbm-`ftmrj ij``f Lmdiuttnvnt Yrfdsejeorfdf dj``f HL (Lystnl HnormsnsYrfdsejeorfdj Lmdiultfdlj _jbu`ftmr m LHY_). @f hudznmdj prnd-lnpf`j in qujstf prmtjndf quj``f in hmrefrj ud lfdf`j trfdsejeorfdfpjr m nmdj l`mrurm, rjbm`ftm if``FES lnl`nlm; n` sum ef`hudznmdfejdtmprmiulj, trf f`trm, ud ndlrjejdtm in nmdj l`mrurm dj` suimrj lgj lmstn-

tunslj `f ofsj onmlgnenlf ij` tjst ij` suimrj su` quf`j sn hmdif tuttmrf`f infbdmsn ij``f HL. @jstjdsnmdj ij` bjdj spnjbf `j`jvftm duejrmin eutfznmdn; smdm ndhfttn fttuf`ejdtj dmtj m`trj 6?33 vfrnfznmdn ij``fsjqujdzf ij` LHY_ pjr f efbbnmr pfrtj em`tm rfrj m sm`m ndinvniuf`n.@f eutfznmdj ij` bjdj LHY_ pn hrjqujdtj lmstntuntf if``f ij`jznmdjin < pfnf in ofsn, lun lmrrnspmdij udf prmtjndf lgj efdlf ij``f hjdn`f-`fdndf, nd pmsnznmdj :37, if ln n` dmej in ij`tf H :37 lgj gf rnljvutm

tf`j eutfznmdj. Jssf ndtjrjssf lnrlf n` 23% in tuttn n lrmemsmen HLdj``j pmpm`fznmdn fdb`msfssmdn j ij` Dmri Jurmpf (nd Ifdnefrlf fi-inrntturf n` >3%) ejdtrj dj``j pmpm`fznmdn ij` \ui Jurmpf j ij``frjfEjintjrrfdjf (Ntf`nf lmeprjsf), dmd supjrf nd ejinf n` :3%. Nd Ntf`nf


2/15

`j eutfznmdn endmrn lgj gfddm udf hrjqujdzf in rn`njvm smdm1 _668?W,D6


3/15

tnpn lmstntuntn if lmeondfznmdn in eutfznmdn sjvjrj lmd udf eutfznmdjenì, lgj hudznmdf if imendfdtj. F`ludj eutfznmdn lmeprjsj dj``f NXj X l`fssj ijtjrendfdm hmrej lmep`jssnvfejdtj entn in ef`fttnf. Yuttf-

vnf, dj` sndbm`m lfsm, dmd pmssnon`j prjvjijrj n` ijlmrsm ij``f ef`fttnfsm`m nd ofsj f` bjdmtnpm, bnmlfdm ndhfttn dj` ijtjrendfrj n` lmrsm ijb`njvjdtn l`ndnln f`trn hfttmrn bjdnln (bjdn emiu`ftmrn) j feonjdtf`n, prnen hrftuttn quj``n `jbftn f``j lurj.@fdf`nsn ij``j eutfznmdn j, imvj qujstj dmd smdm nijdtnlfon`n, fdf`nsnijn pm`nemrsen in rjstrnznmdj ij` IDF (_H@S) nd strjttm `jbfej lmdn` bjdj LHY_, gf pjrejssm udf vfstf fpp`nlfznmdj ij``f bjdjtnlf em`j-lm`frj pjr `f infbdmsn ij` pmrtftmrj, `f infbdmsn prjdftf`j j `m slrjjdndbdjmdftf`j.N` ef`hudznmdfejdtm m fssjdzf ij` lfdf`j LHY_ ndtjrjssf tuttn b`n jpn-tj`n f sjlrjznmdj eulmsf ijtjrendfdim dj` ùej bgnfdim`frj ud inhjttmin l`mrm j sminm j qundin in flquf. N sjlrjtn insnirftftn tjdimdm f rnstf-bdfrj prmvmlfdim lms `mstruznmdj ijb`n mrbfdn ndtjrjssftn (Yfoj``f6).Dj``fòjrm ormdlgnf`j (n` lun brfim in ndtjrjssfejdtm rjspmdsfon`jij``f brfvnt ij``f ef`fttnf), vnjdj f`tjrftf `f l`jfrfdlj eulmln`nfrj j snnddjslf ud lnrlm`m vnznmsm ndfeefznmdj-ndhjznmdj lgj pmrtf nddj f``fijstrutturfznmdj ij` pfrjdlgnef pmèmdfrj.N` pfdlrjfs jsmlrndm vnjdj lmndvm`tm bn dj``f hfsj ij``m svnùppm prjdftf`jlmd mstruznmdj iuttf`j j ijbjdjrfznmdj, f emdtj, ij``f pmrznmdj bgnfd-im`frj sjlrjtmrnf rjspmdsfon`j ij``f prmiuznmdj ijb`n jdznen inbjstnvn.N` prmljssm ndvmùtnvm ijtjrendf qundin `f ijbjdjrfznmdj ormlnstnlfij``mrbfdm if lun ijrnvf n` dmej ij``f ef`fttnf.

Dj` hjbftm `fssjdzf in hudznmdj ij``f prmtjndf lfdf`j LHY_ ijtjrendfmstruznmdj ijn iuttu`n on`nfrn if on`j ijdsf lmd ndfeefznmdj, ormsnpjrnpmrtf`j ji jvmùznmdj nd lnrrmsn on`nfrj.Djb`n jpntj`n f sjlrjznmdj snjrmsf, ndvjlj, n` trfspmrtm ij` l`mrm fvvnjdjnd inrjznmdj mppmstf lnm if` ùej f``f lj`ù`f j pjrtfdtm `fdmef`nfij` lfdf`j LHY_ ijtjrendf ud inhjttm in rnfssmronejdtm ij` l`mrm lmdnpjrsf`ndnt ij``j sjlrjznmdn.


4/15

Yfo. 6

- F`tjrfznmdn hudznmdf`n j efdnhjstfznmdn l`ndnlgj if prmtjndf LHY_

fdmef`f

M_BFDN LMD

\JL_JQNMDJ EZLM\F

OMLLF

NDYJ\YNDM

SFDL_JF\

XNJ ON@NF_N

DF\M J \JDN

SF_FDF\F@N

O_MDLGN

IMYYN IJHJ_JDYN

LJ_XNLJ ZYJ_NDF

M_BFDN LMD

\JL_JQNMDJ \NJ_M\F

BGNFDIM@J

\ZIM_NSF_J,

@FL_NEF@N, \F@NXF_N

F@YJ_FQNMDN

bgnfdim`j sf`nvfrn

rnstfbdm eulm mstruznmdj

mstruznmdj imttn

stfsn on`nfrj

stfsn eulm

ndhjznmdj

stfsn eulm

mstruznmdj

ndhjznmdjijstrutturfznmdj

ftrjsnf ijhjrjdtn

eulm ijdsm

F@YJ_FQNMDN

npjrsf`ndnt suimrj, `f-

lrnej j sf`nvf pfrmtnijf

EFDNHJ\YFQNMDN

L@NDNLGJ

inhjttm sf`nvfznmdj

n`jm if ejlmdnm

jqunvf`jdtn ejlmdnf`n

efìnbjstnmdj

ef`dutrnznmdj

lnrrmsn on`nfrj

npjrtjdsnmdj pmrtf`j

`ntnfsn on`nfrj

rndntj-sndusntj

pm`npmsn

fdmsenf

ormdlmpdjupftnf lrmdnlf

jvmùtnvf

fspjrenf-ndhjrtn`nt efslgn`j

rnimttf hjrtn`nt

LMD\JBZJDQJ L@NDNLGJ

sndirmej pjrintf sf`n

(Eminhnlftf if Efstj``f @f hnormsn lnstnlf ji.LJ.I._N.E 6>>:)

EFDNHJ\YFQNMDN L@NDNLGJ

Fppfrftm rjspnrftmrnm@j efdnhjstfznmdn rjspnrftmrnj lgj lmepfnmdm in sm`ntm djn prnen ejsn in vntflmd `j lfrfttjrnstnlgj in ormdlgnm`ntj lmeprjdimdm udf tmssj ndnznfèjdtjin tnpm pjrtussmnij. \ulljssnvfejdtj f tmssj invnjdj prmiuttnvf, lmepfrjtflgnpdjf, inspdjf, fuejdtm ij` infejtrm tmrflnlm fdtjrm-pmstjrnmrj, rfdtm`ndn lrjpntfdtn j spjssm quf`lgj bjentm jspnrftmrnm. N` rjpjrtm rfinm`mbnlm

ndnznf`j lfrfttjrnzzftm if npjrndfznmdj pmèmdfrj j `f hudznmdf`nt rj-spnrftmrnf emstrf bn n sjbdn in ef`fttnf pmèmdfrj mstruttnvf. @j rnflutnz-zfznmdn rnpjtutj (ormdlgntn j ormdlmpmèmdntn) fllmepfbdftj if fuejdtmij``f tmssj inurdf j dmtturdf lmd foomdifdtj jslrjftm eulm-puru`jdtm,


5/15

tflgnpdjf, inspdjf, nppmlrftnsem inbntf`j, pjrintf in pjsm, svnùppfdmdj` tjepm j lfrfttjrnstnlgj ij``f ormdlmpdjuempftnf lrmdnlf mstruttnvf.@m \tf`mlmllus furjus j `Gfjempgnùs ndujdzfj lm`mdnzzfdm prj-

lmljejdtj `j vnj fjrjj ejdtrj `m Ssjuimemdfs fjrubndmsf tjdij flmepfrnrj pn tfrinvfejdtj ef n` pftmbjdm pn hrjqujdtjejdtj nsm`ftmdj``jslrjftm ijn pfznjdtn fim`jsljdtn j fiu`tn. F`trn bjren ejdm hrj-qujdtjejdtj prjsjdtn dj``j lm`turj ij``j vnj fjrjj smdm `f Ourkgmìjrnfljpflnf, `F`lf`nbjdjs xn`msmxnifds, `m \tjdmtrmpgmemdfs ef`tmpgn`nf,ef ejdtrj pjr f Ourkgmìjrnf dmtf fbbrjssnvnt vjrsm n` pmèmdj, pjrqujstj u`tnej spjlnj dmd fdlmrf snlurfejdtj flljrtftf f pftmbjdnlnt.

LMES@NLFDQJ1 `f sndusntjsn svnùppf dj``f efbbnmrfdzf ijn pfznjdtn j `f rfinmbrff

jvnijdznf mpflnt lmep`jtf ijn sjdn dj` >3% ijn ofeondn; `f pm`npmsn dfsf`j sn pu prjsjdtfrj dj` 2-63% ijn pfznjdtn nd jt

prjlmlj; `fspjrbn``msnormdlmpmèmdfrj f``jrbnlf, lfusftf if``Fspjrbn`ùs

huenbftus, lfrfttjrnzzftf if sndtmeftm`mbnf in tnpm fseftnlm, nd`trftnpmèmdfrn rmtmdijbbnfdtn tjdijdtn f jslfvfznmdj, f`tn `nvj``n in NbJtmtf`n, spjlnlgj j prjlnpntndj fdtn-fspjrbn``m;

`ftj`jttfsnf, lmep`nlfdzf spjssm prjsjdtj djn prnen ejsn in vntf, lfusftf sm`ntfejdtj if tfppn in eulm j prjvf`jdtjejdtj mlf`nzzftf f``mom supjrnmrj ijstrm;

n` pdjuemtmrflj ijtjrendftm if rmtturf in om``j suop`jurnlgj pnhrjqujdtj djn rfbfzzn j djb`n fiu`tn lmd lm`mdnzzfznmdj pjrsnstjdtj if

\tf`mlmllus furjus; `jemttnsn njvj (strnfturj in sfdbuj dj``jslrjftm) em`tm hrjqujdtj, ejdtrj

pn rfrf ef tf`vm tf hftf`j jemttnsn efssnvf (m trj ?33 ll in sfdbuj); `ndsuhlnjdzf rjspnrftmrnflfusftf if prmbrjssnvf npmssnf j npjrlfpdnf

ji jvnijdznftf if inspdjf brfvj, lnfdmsn, ljhf`jf, tmrpmrj m jllntfznmdjlmd jvmùznmdj nd ndsuhlnjdzf lfrinflf ijstrf.

Fppfrftm bfstrmndtjstndf`j@ndsuhlnjdzf pfdlrjftnlf, lfusftf if``mstruznmdj ijn imttn j if``fHnormsn lnstnlf lmdsjbujdtj, bn prjsjdtj nd jt djmdftf`j dj` 83% ijnpfznjdtn j lm`pnslj nd jt pn fvfdzftf lnrlf `7:% ijn smbbjttn.@f slfrsf m fssjdtj sjlrjznmdj jdzneftnlf pfdlrjftnlf ijtjrendf efìn-bjstnmdj j ef`fssmronejdtm ijn brfssn (stjftmrrjf), prmtjndj j fenin. @f

sndtmeftm`mbnf lfrfttjrnzzftf if infrrjf lrmdnlf lmd hjln foomdifdtn,udtumsj j ef`jmimrfdtn, fppjtntm vmrflj, fiimej ejtjmrnlm, npmtrmsemeuslm`frj, npmprmtjndjenf, ijlnt in vntfendj npmsmùon`n ji frrjstm ij``flrjslntf.


6/15

LMES@NLFDQJ1 `n`jm if ejlmdnmlmstntunslj `f efdnhjstfznmdj l`ndnlf pn prjlmlj

ij``f HL, lfusftf if` lfttnvm hudznmdfejdtm m fssjdzf ij` lfdf`j

LHY_, lmepfrj dj` 6:% ijn djmdftn fhhjttn j sn prjsjdtf lmd vmentm,instjdsnmdj fiimendf`j j efdlftf jenssnmdj in hjln jdtrm j prnej ?9mrj in vntf. Yf`vm`tf mstruznmdj pu jssjrj jvnijdznftf bn f``f ??-?:->7% ijn pfznjdtn efslgn smdm ndhjrtn`n f lfusf in ftrjsnf on`ftjrf`jijn imttn ijhjrjdtn (LOFXI) lgj sn ijtjrendf dj``j hfsn prjlmln ij``msvn`uppm ndtrfutjrndm (6? sjttnefdf).Fdlgj j hjeendj prjsjdtfdm udf hjrtn`nt inenduntf f lfusf ij``nspjssn-ejdtm ij` eulm ljrvnlf`j, tuttfvnf j brfvnifdzj smdm pmssnon`n fdlgj sjfllmepfbdftj if nepmrtfdtj rnslgnm in pjbbnmrfejdtm ij``j lmdinznmdnrjspnrftmrnj.

INFBDM\N

@f infbdmsn sn ofsf su sndtmen l`ndnln subbjstnvn in HL ndsnjej f tjst inlmdhjref onmlgnenln m bjdjtnln (Yfoj``f ?).

Yfo. ?- Lrntjrn pjr `f infbdmsn in ormsn lnstnlf

\ndtmen HL lmrrj`fon`n (hjdmtnpm HL)

mppurjHfen`nfrnt pjr HLmppurj\lrjjdndb djmdftf`j pmsntnvm +

Yjst ij` suimrj pftm`mbnlm nd ? m pn ijtjrendfznmdn

mppurj Eutfznmdn ndinvniuftj su jdtrfeon b`n f``j`n

mppurj

F`tjrfznmdj dj``f inhhjrjdzf ijn pmtjdznf`n dfsf`n


8/15

\lrjjdndb djmdftf`j

@m slrjjdndb djmdftf`j vnjdj jhhjttuftm ejinfdtj imsfbbnm ij``f trnpsndfneeudmrjfttnvf su bmllnf in sfdbuj nd 9f-:fbnmrdftf in vntf j pjrejttj

in infbdmstnlfrj f`ejdm `7:% ijn djmdftn HL.

Yjst ij` suimrj

N` tjst ij` suimrj lmstntunslj if m`trj 93 fddn n` bm`i stfdifri pjr `finfbdmsn, tuttfvnf mbbn smdm lmdmslnutj eutfznmdn lgj sn fssmlnfdm f tjstij` suimrj djbftnvm.

Jssm sn ofsf su``f ijtjrendfznmdj ij``f lmdljdtrfznmdj in l`mrm j sminmdj` suimrj rfllm`tm impm stnem`fznmdj `mlf`j lmd pn`mlfrpndf.\mdm if lmdsnijrfrsn dmref`n n `nvj``n in l`mrm j sminm ndhjrnmrn f


9/15

Y_FYYFEJDYM

@f tjrfpnf ij``f HL, jstrjefejdtj lmep`jssf j frtnlm`ftf, sn ofsf su``j

tjrfpnj sndtmeftnlgj rmutndfrnj (Yfoj``f


10/15

Fppfrftm bfstrmndtjstndf`jNdsuhlnjdzf Sfdlrjftnlf

@f pmsm`mbnf ijb`n jstrfttn pfdlrjftnln hmreu`ftn nd enlrmshjrj bfstrmprm-

tjttj, ijvj jssjrj lf`lm`ftf nd ofsj f` brfim ij``ndsuhlnjdzf pfdlrjftnlf(`njvj, emijrftf, brfvj) lmd ud rfdbj trf :33


11/15

N`jm if ejlmdnm

N` trfttfejdtm ijvj jssjrj pmssnonèjdtj lmdsjrvftnvm, smttm lmdtrm``mrfinmbrflm j supjrvnsnmdj lgnrurbnlf, pjr `jhlflnf nepmrtfdtj `f

prjlmlnt infbdmstnlf. Impm fvjrj pmsnznmdftm smdindm dfsm-bfstrnlm pjrirjdfrj n` lmdtjdutm ij``m stmeflm, sn smeendnstrf pjr l`nsef Bfstrmbrf-d lgj pjr f f`tf msem`frnt rnlgnfef nqunin dj``ndtjstndm uninlfdimn` ejlmdnm. Sjr lmepjdsfrj `j pjrintj ndtjstndf`n ndhusnmdj jdimvjdmsfin smùznmdn nirmj`jttrm`ntnlgj dm f lmep`jtf lfdf`nzzfznmdj. N` trfttf-ejdtm ejinlm vf foofdimdftm sj impm ?9 mrj dmd sn mttnjdj lmep`jtflfdf`nzzfznmdj j slmepfrsf ijn sjbdn in mstruznmdj.

INM\

N` trfttfejdtm ijvj jssjrj prjlmlj j prmtrfttm dm f``f rnsmùznmdj.@f tjrfpnf ejinlf ijvj jssjrj prjhjrntf j pu prmljijrj f tfppj f sjlmdifij``f tm``jrfdzf bfstrnlf1 `fvfbbnm ndtjstndf`j lmd \J@B; qujstf smùznmdj vf smeendnstrftf pjr

ms m pjr smdif dfsm-bfstrnlf, qufdim dmd snf prjsjdtj vmentm, lmd ud

vmùej in ?-9 `ntrn dj``fiu`tm j in 6-? `ntrn dj` ofeondm j lmeudqujdm f``jenssnmdj in hjln `nqunij. D-fljtn`lnstjndf f` ?3% if smeendnstrfrj lmd smdif dfsmbfstrnlf,


12/15

emif`nt iusm, `f iurftf ij``fpp`nlfznmdj j b`n struejdtn utn`nzzftnsmdm invjrsn pjr mbdn tnpm in ndinlfznmdj tjrfpjutnlf. Xjdbmdm utn`nzzftnfppfrjllgn Djou`nzzftmrn Ajt, Djou`nzzftmrn fi u`trfsumdn, sprfy prj-

imsftn nd pm`vjrj, omeom`jttj prjssurnzzftj lmd lfejrf instfdznftrnljlmd vf`vm`f, imllnf dfsf`j enlrmdnzzftf (Yfoj``f 8).

Yfo. 8 - Hfrefln pjr fjrmsm`tjrfpnf

Fdtnonmtnln

(tmorfenlndf fi f`tm imsfbbnm, lm`nstndf)Eulm`ntnln

(feormxm`, IDfsj rnlmeondfdtj)

Ormdlmin`ftftmrn

(sf`outfem`m)

Lmrtnsmdnln

(ouijsmdnij, ojl`mejtfsmdj, h`utnlfsmdj)

Dmen lmeejrlnf`n

YMON h`


13/15

Dmen lmeejrlnf`n

Fzntrmenlndf

@f em`jlm`f gf prmprnjt fdtndhnfeeftmrnj,

ndnontmrnj djn lmdhrmdtn in prmimttn ofttjrnln m sm-stntutnvj ij` lfdf`j LHY_ inhjttmsm. Ndm`trj rniu-

lj `f prmiuznmdj in eulm j f`tjrf n` onmhnè ij`

hjdmtnpm eulmsm ij``m S. fjrubndmsf.

Imsfbbnm1 ?:3 eb f bnmrdn f`tjrdn smttm n 93 kb j

:33 eb f bnmrdn f`tjrdn smprf n 93 kb

QNY_MEFWlpr :33eb, smspjdsnmdj

93 eb/e`

Mssnbjdmtjrfpnf1 pjr udf lmrrjttf prjslrnznmdj mllmrrj vfùtfrj n`

hfoonsmbdm in mssnbjdm ejinfdtj f sfturnejtrnf dmtturdf j smttm shmrzm.Dj` pfznjdtj nd mssnbjdmtjrfpnf djljssfrnm emdntmrfrj `f SfLM? jin` pG ejinfdtj jembfsfdf`nsn.

Xjdtn`fznmdj dmd ndvfsnvf1vf rnsjrvftf fn pfznjdtn lmd ndsuhlnjdzfrjspnrftmrnf nrrjvjrsnon`j j nd `nstf in trfpnfdtm onpmèmdfrj.

Yrfpnfdtm onpmèmdfrj1 lmstntunslj `f tjrfpnf jstrjef pjr n pfznjdtnlmd ndsuhlnjdzf rjspnrftmrnf dmd pn lmdtrm``fon`j if``f tjrfpnf ej-

inlf. Nd Ntf`nf vn smdm f`ludn Ljdtrn in Yrfpnfdtm pmèmdfrj lmd oumdfjspjrnjdzf in ef`ftn in ormsn lnstnlf j lmd smprfvvnvjdzf efbbnmrjij` 83% f : fddn if``ndtjrvjdtm.Ejdtrj djn prnen iminln ejsn if` trfpnfdtm prjvf`bmdm djttfejdtj `jlmep`nlfdzj lgnrurbnlgj ij``j vnj fjrjj, n` rnbjttm flutm, `j ndhjznmdnif LEX j quj``j ijtjrendftj if ofttjrn m hudbgn bn prjsjdtn dj``f`-ojrm rjspnrftmrnm prnef ij` trfpnfdtm, if` sjlmdim fddm sn mssjrvfdm

efbbnmrejdtj b`n jhhjttn lm``ftjrf`n ijrnvfdtn if``f tjrfpnf neeudm-

smpprjssnvf j n` rnbjttm lrmdnlm (OM\). Vujstu`tnem lmstntunslj f pnhrjqujdtj lfusf in emrtj f instfdzf if` trfpnfdtm.

Yjrfpnf ij``j rnflutnzzfznmdn ormdlmpmèmdfrn

Hnsnmtjrfpnf rjspnrftmrnf1ndlrjejdtm ij` duejrm ij``j sjiutj bnmr-df`njrj.

Yjrfpnf fdtnonmtnlf1ijvj jssjrj enrftf nd ofsj fn rnsu`tftn ij``flm`turf ij``jslrjftm usfdim prjvf`jdtjejdtj, pjr vnf jdimvjdmsf,? hfrefln fdtn-Ssjuimemdfs nd fssmlnfznmdj pjr jsjepnm1 ljhtfzn-inej + tmorfenlndf, lnprmmxflndf + tmorfenlndf, ejrmpjdje +tmorfenlndf jtl. f` dj in ndiurrj ejdm `ndsmrbjdzf in rjsnstjdzf.\n rfllmefdif in jhhjttufrj n lnl`n in tjrfpnf pjr fèjdm 69 bnmrdn(Yfoj``j 7 j >).


14/15

Yfo. 7-Fdtnonmtnln pjr vnf pfrjdtjrf`j

**N` imsfbbnm sn rnhjrnslj f` Yrnejtmprne.

Fdtnonmtnlm

Fendmb`nlmsnin

Ymorfenlndf

Bjdtfenlndf

Fenkflndf

Djtn`enlndf

Ljhf`mspmrndj

Ljhtfzninej

Ljhtrnfxmdj

Ljhjpnej

Sjdnln``ndj

Snpjrfln``ndf/Yfzmofl

Ynlfrln``ndf/L`fvu`fd

Fepnln``ndf/\u`ofltfe

Mxfln``ndf

F`trn

Nenpjdje/Ln`fstndf

Ejrmpjdje

Lnprmh`mxflndf

Fztrjmdfe

L`mrfehjdnlm`m

YES-\EW

Xfdlmenlndf

Yjnlmp`fdndf

Lm`nstndf

Imsj

eb/kb/inj

8 - 63

6: -


15/15

Fdtnonmtnlm Imsj

eb/kb/inj

D

smee.dn

inj

Dmej

lmeejrlnf`j

Sftmbjdn

F`trnYES-\EW

L`ndifenlndf

Jrntrmenlndf

Fzntrmenlndf

Imxnlnl`ndf

Endmlnl`ndf

63 - ?3**

?3 - 93

?: - :3

63(6 bb),

:(?f - :fbb)

? - :

?

?

9

9

6

9

?

OFLY_NEHM_YJ

IF@FLNDL

J_NY_MLNDF

QNY_MEFW

OF\\FIM

ENDMLND

O. Ljp., \t. Fur., G. Ndh.

\t. Fur.

\t. Fur.

\t. Fur.

O. Ljp., G. Ndh.

O. Ljp., G. Ndh.

**N` imsfbbnm sn rnhjrnslj f` Yrnejtmprne.

YJ_FSNJ HZYZ_J1

Djb`n u`tnen ijljddn stftm iftm em`tm spfznm f``f rnljrlf in dumvn fp-prmlln tjrfpjutnln. Fdlmrf em`tm `f strfif if pjrlmrrjrj ef vmb`nfemifrj ud ljddm f quj``j lgj smdm `j nddmvfznmdn pn snbdnlftnvj16) YJ_FSNF BJDNLF1 fpprmllnm pn lmep`jssm j sn ofsf su``f smstn-

tuznmdj ij` bjdj LHY_ fdmef`m lmd ud bjdj lmrrjttm fttrfvjrsm vjttmrnvnrf`n j dmd vnrf`n (`npmsmen jtl.). F inhhjrjdzf in qufdtm fllfij nd vntrm`f tjldnlf nd vnvm gf prjsjdtftm dmtjvm`n inhlm`t. N vjttmrn utn`nzzftngfddm prjsjdtftm em`tn `nentn prnem trf tuttn `f rnspmstf ndfeeftmrnfij``mspntj lgj tjdij fi j`nendfrj n` vjttmrj j j lj``u`j if jssm ndhjttftj.

?) HF_EFLN LGJ FYYNXFDM LFDF@N F@YJ_DFYNXN F@ LFDF-@J IJ@ L@M_M LHY_1 ud u`tjrnmrj ejtmim lgj sn stf spjrnejdtfdim

quj``m in utn`nzzfrj hfrefln lgj stnem`fdim f`trn lfdf`n ij` l`mrmlmepjdsndm `f LHY_ fdmef`f.

18_fibrosi cistica (mucoviscidosi) optimized

Documents