18_Fibrosi Cistica (Mucoviscidosi) Optimized
-
Upload
com-edizioni -
Category
Documents
-
view
226 -
download
0
Transcript of 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
-
7/25/2019 18_Fibrosi Cistica (Mucoviscidosi) Optimized
2/15
`j eutfznmdn endmrn lgj gfddm udf hrjqujdzf in rn`njvm smdm1 _668?W,D6
-
7/25/2019 18_Fibrosi Cistica (Mucoviscidosi) Optimized
3/15
tnpn lmstntuntn if lmeondfznmdn in eutfznmdn sjvjrj lmd udf eutfznmdjen`i, 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` `uej 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``f`ojrm 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 pm`emdfrj.N` pfdlrjfs jsmlrndm vnjdj lmndvm`tm bn dj``f hfsj ij``m svn`uppm 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 ndvm`utnvm 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 jvm`uznmdj nd lnrrmsn on`nfrj.Djb`n jpntj`n f sjlrjznmdj snjrmsf, ndvjlj, n` trfspmrtm ij` l`mrm fvvnjdjnd inrjznmdj mppmstf lnm if` `uej f``f lj``u`f j pjrtfdtm `fdmef`nfij` lfdf`j LHY_ ijtjrendf ud inhjttm in rnfssmronejdtm ij` l`mrm lmdnpjrsf`ndnt ij``j sjlrjznmdn.
-
7/25/2019 18_Fibrosi Cistica (Mucoviscidosi) Optimized
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
ef`inbjstnmdj
ef`dutrnznmdj
lnrrmsn on`nfrj
npjrtjdsnmdj pmrtf`j
`ntnfsn on`nfrj
rndntj-sndusntj
pm`npmsn
fdmsenf
ormdlmpdjupftnf lrmdnlf
jvm`utnvf
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 ndnznf`ejdtjin 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 pm`emdfrj j `f hudznmdf`nt rj-spnrftmrnf emstrf bn n sjbdn in ef`fttnf pm`emdfrj mstruttnvf. @j rnflutnz-zfznmdn rnpjtutj (ormdlgntn j ormdlmpm`emdntn) fllmepfbdftj if fuejdtmij``f tmssj inurdf j dmtturdf lmd foomdifdtj jslrjftm eulm-puru`jdtm,
-
7/25/2019 18_Fibrosi Cistica (Mucoviscidosi) Optimized
5/15
tflgnpdjf, inspdjf, nppmlrftnsem inbntf`j, pjrintf in pjsm, svn`uppfdmdj` tjepm j lfrfttjrnstnlgj ij``f ormdlmpdjuempftnf lrmdnlf mstruttnvf.@m \tf`mlmllus furjus j `Gfjempgn`us 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 Ourkgm`ijrnfljpflnf, `F`lf`nbjdjs xn`msmxnifds, `m \tjdmtrmpgmemdfs ef`tmpgn`nf,ef ejdtrj pjr f Ourkgm`ijrnf dmtf fbbrjssnvnt vjrsm n` pm`emdj, pjrqujstj u`tnej spjlnj dmd fdlmrf snlurfejdtj flljrtftf f pftmbjdnlnt.
LMES@NLFDQJ1 `f sndusntjsn svn`uppf 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``msnormdlmpm`emdfrj f``jrbnlf, lfusftf if``Fspjrbn``us
huenbftus, lfrfttjrnzzftf if sndtmeftm`mbnf in tnpm fseftnlm, nd`trftnpm`emdfrn 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 jvm`uznmdj 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 ef`in-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 npmsm`uon`n ji frrjstm ij``flrjslntf.
-
7/25/2019 18_Fibrosi Cistica (Mucoviscidosi) Optimized
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
-
7/25/2019 18_Fibrosi Cistica (Mucoviscidosi) Optimized
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
-
7/25/2019 18_Fibrosi Cistica (Mucoviscidosi) Optimized
9/15
Y_FYYFEJDYM
@f tjrfpnf ij``f HL, jstrjefejdtj lmep`jssf j frtnlm`ftf, sn ofsf su``j
tjrfpnj sndtmeftnlgj rmutndfrnj (Yfoj``f
-
7/25/2019 18_Fibrosi Cistica (Mucoviscidosi) Optimized
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
-
7/25/2019 18_Fibrosi Cistica (Mucoviscidosi) Optimized
11/15
N`jm if ejlmdnm
N` trfttfejdtm ijvj jssjrj pmssnon`ejdtj 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 sm`uznmdn 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 rnsm`uznmdj.@f tjrfpnf ejinlf ijvj jssjrj prjhjrntf j pu prmljijrj f tfppj f sjlmdifij``f tm``jrfdzf bfstrnlf1 `fvfbbnm ndtjstndf`j lmd \J@B; qujstf sm`uznmdj vf smeendnstrftf pjr
ms m pjr smdif dfsm-bfstrnlf, qufdim dmd snf prjsjdtj vmentm, lmd ud
vm`uej 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,
-
7/25/2019 18_Fibrosi Cistica (Mucoviscidosi) Optimized
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`
-
7/25/2019 18_Fibrosi Cistica (Mucoviscidosi) Optimized
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` onmhn`e 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 vf`utfrj 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 onpm`emdfrj.
Yrfpnfdtm onpm`emdfrj1 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 pm`emdfrj 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 ormdlmpm`emdfrn
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 f`ejdm 69 bnmrdn(Yfoj``j 7 j >).
-
7/25/2019 18_Fibrosi Cistica (Mucoviscidosi) Optimized
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: -
-
7/25/2019 18_Fibrosi Cistica (Mucoviscidosi) Optimized
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.