13_Quando Sospettare Una Malattia Metabolica Optimized

7/25/2019 13_Quando Sospettare Una Malattia Metabolica Optimized

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HLCAKAPAMKL

Il ofifttal oltfbmiadnl lrlhatfral m lrrmra dmkelkata hli oltfbmiasom(LDO) smkm ofifttal elkltadnl ak dua smkm fitlrftl il kmrofia cukzamkaoltfbmiadnl. Elklrfiolktl il ofifttal oltfbmiadnl smkm dmksahlrftlofifttal omitm rfrl. Xuttfvaf qulstm vlrm sl il dmksahlrafom sakemifr-olktl, of kmk vlrm sl vlkemkm dmksahlrftl dmol eruppm.

If erfkhl ofeeamrfkzf hliil ofifttal oltfbmiadnl dmkelkatl lrlhatftfak ofkalrf futmsmoadf rldlssavf. Olkm crlqulktlolktl, eia LDO smkmlrlhatfta dmk omhfiat ileftf fii^ (plr lslopam, ai Hldat ha mrkatakftrfksdfrbfoaifsa, ofifttaf -iakglh, qufsa sloprl iltfil plr a ofsdna)mfutmsmoadf hmoakfktl. Akmitrl, fiduka LDO, fkdnl sl omitm rfra, nfkkmukf lrlhatfralt oftraiaklfrl. Vulsta tapa ha hasmrhaka smkm hmvuta f ou-tfzamka hli elkmof oatmdmkhrafil. kmtm dnl smim a oatmdmkhra hliim-

vmdatf, l kmk hleia splroftmzma, dmktrabuasdmkm fi HKF oatmdmkhrafilhliim zaemtl7 plrtfktm outfzamka hli HKF oatmdmkhrafil smkm trfsolsslhf ukf elklrfzamkl fiif suddlssavf smim fttrfvlrsm if iaklf oftlrkf.Tmkm stfta ahlktadfta pa ha 8


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Xfb. 6-Zrlslktfzamka diakadnl pa dmouka hleia lrrmra dmkelkata hli oltfbmiasom6

F. Hfkkm klurmimeadm fdutm

Ofifttal ha paddmil omildmil haccusabaia

Hasmrhaka hleia foakmfdaha (ofifttaf hliil urakl f sdarmppm hfdlrm) Hasmrhaka hleia fdaha mrefkada (fdahuraf oltaiofimkadf) Hacltta hliimssahfzamkl hleia fdaha erfssa

(hldat ha fdai-DmF hlahrmelkfsa f olhaf dftlkf)

Aplrfoomkaloal (hldat ha mrkatakf trfksdfrbfoaifsa)

Hasmrhaka hli ifttftm l hasmrhaka oatmdmkhrafia (hldat ha datmdrmom d mssahfsa)

Hfkkm klurmimeadm dmk dmkvuisamka (aplreiadakloaf kmk dnltmsadf)

B. Hfkkm klurmimeadm drmkadm Ofifttal ha paddmil omildmil haccusabaia

Ofifttal ha mrefkliia

Ofifttal oatmdmkhrafia

Hacltta hliif dftlkf ha trfspmrtm hleia lilttrmka (hldat ha datmdrmom d mssahfsa)

Hacltta hli oltfbmiasom hli paruvftm (hldat ha paruvftm hlahrmelkfsa)

Ofifttal hf fdduouim iasmsmoafil

Oudmpmiasfddfrahmsa (ofifttaf ha Nurilr), Eiadmprmtlakmsa (f-ofkkmsahmsa),Efkeiamsahmsa (efkeiamsahmsa E

O2)

Fitrl skemiapahmsa (ofifttaf ha Efudnlr), Iludmhastrml

(iludmhastrmf oltfdrmoftadf)

Hasmrhaka plrmssasmoafiaHacltta hliif bamelklsa l hliif b-mssahfzamkl plrmssasmoafil (sakhrmol haPliiwlelr)

Dmkhrmhaspifsaf pukdtftf razmoliadf, Fhrlkmiludmhastrmf ileftf fii^,

Fitra hacltta ha sakemia lkzaoa plrmssasmoafia (mssfimsa)

Hasmrhaka hliif eiadmsaifzamkl prmtladf Dmoprlkhl a hacltta lkzaoftada imdf-iazzfta ak0Datmsmi0 sakhrmol hf haclttm ha eiadmsaifzamkl tapm Af l Ab

Hasmrhaka hliifppfrftm ha Emiea0 sakhrmol hf haclttm ha eiadmsaifzamkl tapm AA Rltadmim lkhmpifsoftadm0 sakhrmol hf haclttm ha eiadmsaifzamkl tapm Ad l S

D. Hfkkm lpftmdliiuifrl haccusm

Lpftmpftaf fdutf m drmkadf

Hacltta hli oltfbmiasom hla dfrbmahrfta (efifttmsloaf), Hacltta hli oltfbmiasomhleia foakmfdaha (tarmsakloaf)

Hacltta hli trfspmrtm hla oltfiia (ofifttaf ha Uaismk), Hacltta hleia akabatmra hliilprmtlfsa (hldat ha f

6-fktatrapsakf)

(sleul)


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H. Oampftaf

Oampftaf sdnliltradf

Rfbhmoamiasa fdutf (hldat ha cmscmraifsa ousdmifrl)

Oampftaf drmkadf (hacltta hliif dftlkf ha trfspmrtm oatmdmkhrafil hleia lilttrmka7

hacltta hli oltfbmiasom hleia fdaha erfssa)

Oamdfrhampftaf

Ofifttal hf fdduouim iasmsmoafia (ofifttaf ha Zmopl0 hldat ha f-eiudmsahfsa) Hasmrhaka hli oltfbmiasom hleia fdaha erfssa (hldat ha 4-ahrmssafdai-DmF hlahrm-

elkfsa)L. Hfkkm tubuifrl rlkfil

Hfkkm eimolruifrl tubuifrl

Ofifttal hf fdduouim iasmsmoafil (dastakmsa) Hacltta lkzaoftada (mssfimsa)

Hacltta hli trfspmrtm

Hacltta ha trfspmrtm ha sakemil omildmil m ha eruppa ha omildmil saoaia (dastakuraf)

6Xrf pfrlktlsa smkm rapmrtfta hleia lslopa.(DmF ? dmlkzaof F)

Omhadftf hf @mnk Dnrastmhmuimu0 Fpprmddam diakadm feia lrrmra dmkelkata hli oltfbmiasom.

Ofkufil ha Zlhaftraf (Ruhmipn), 6fLh. atfiafkf, OdErfw-Naii, 2::4.

F. FZZRMDDAM FI ZFPALKXL DMK HFKKM

KL\RMIMEADM FD\XM

\kf dmoprmoassamkl klurmimeadf dlrtfolktl if ofkaclstfzamkl diakadfpa dmoukl trf eia LDO. Il ofkaclstfzamka klurmimeadnl pmssmkm lsslrl

fdutl, aktlroattlkta, drmkadnl m fkdnl prmerlssavl. Zlr if ofeeamr pfrtlhleia LDO dnl dfusfkm hfkkm klurmimeadm fdutm ilsmrhamsa vlradfkli plramhm klmkftfil (lsslkhm ai plramhm klmkftfil uk plramhm ak dua omitm aktlksm im strlss dftfbmiadm)7 tuttfvaf eia LDO pmssmkm ofkaclstfrsaf qufisafsa lt. Eia LDO dnl dfusfkm ofkaclstfzamka diakadnl hurfktlai plramhm klmkftfil smkm smiatfolktl erfva l splssm pmssmkm dmkhurrlf omrtl ai bfobakm sl kmk valkl akazaftf prmktfolktl ukf tlrfpaf fhl-

euftf. A klmkfta l/m a bfobaka fccltta hf LDO pmssmkm prlslktfrl ukm mpa trf omitlpiada sleka l saktmoa (Cae. 6, Xfb. 2). Ak iaklf elklrfil, ukqufhrm ha dmoprmoassamkl klurmimeadf fdutf, sldmkhfram fh uk LDO, elklrfiolktl dfusftm hfiifdduouim kli dlrvliimha paddmil omil-dmil haccusabaia (foakmfdaha, fdaha mrefkada, fdaha erfssa, foomkam),mppurl hfi hlcdat ha uk prmhmttm lsslkzafil (plr lslopam, ai hldat hlioltfbmiasom hli paruvftm m hliif cukzamkl oatmdmkhrafil hliif dftlkf ha

trfspmrtm hleia lilttrmka) m akkl hfi hlcdat ha uk prmdlssm ha trfspmrtmhliil paddmil omildmil fttrfvlrsm il olobrfkl dliiuifra.Kli dfsm ha lsmrham klmkftfil,tapadfolktl ai klmkftm fcclttm fppfrlfiif kfsdatf ak bumkl dmkhazamka diakadnl, of hmpm uk plramhm ha iftlkzf


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(fidukl mrl, eamrka m ukf slttaofkf), sa nf if dmopfrsf ha saktmoftmimeafhfiifrol, qufil rautm hliifiaolktfzamkl, vmoatm plrsastlktl (mddf-samkfiolktl omitm erfvl tfktm hf sueelrarl ukf stlkmsa aplrtrmdf hli

paimrm), iltfreaf, arratfbaiat, sdfrsm fddrlsdaolktm, hasahrftfzamkl, hafrrlf,apmtmkaf, fdahmsa oltfbmiadf, attlrm, dmkvuisamka, ltd. Ak fidukl ofifttalhli oltfbmiasom pmssabail fkdnl ai rasdmktrm ha uk mhmrl dfrfttlrastadm(plr lslopam, mhmrl ha palha suhfta kliif asmvfilradmfdahloaf). hfradmrhfrl dnl eia mhmra aksmiata vfkkm radlrdfta suiil urakl, kliifiatm lsuiif pliil.Kli dfsm ha lsmrham tfrhavm,(bfobakm m uk fhuitm) ai pfzalktl pu emhlrlha bumkf sfiutl plr fkka m hldlkka, of aoprmvvasfolktl, elklrfiolk-tl ak mddfsamkl ha plramhm ha aktlksm strlss dftfbmiadm qufia akclzamkaaktlrdmrrlkta m if erfvahfkzf, pu ofkaclstfrl sleka klurmimea (ftfssaf,rahuzamkl hliif dmsdalkzf, hasmralktfolktm, ltd.), lsprlssamkl ha uk qufhrmha dmoprmoassamkl klurmimeadf fdutf.Il prakdapfia ofifttal oltfbmiadnl dnl sa pmssmkm prlslktfrl dmk ukqufhrm ha hfkkm klurmimeadm fdutm smkm0

foakmfdahmpftal (plr lslopam, ifOfifttaf hliil urakl f sdarmppmhfdlrm)7

fdahmsa mrefkadnl (plr lslopam,ifZrmpamkadmfdahloaf)7 hacltta hliimssahfzamkl hleia fdaha erfssa7 aplrfoomkaloal (plr lslopam, ai Hldat ha mrkatakftrfksdfrbfoaifsa)7 fdahmsa ifttadnl.

Il ofkaclstfzamka diakadnl, il akhfeaka ha fpprmcmkhaolktm hafekmstadml il lvlktufia tlrfpal ha qulstm eruppm ha pftmimeal smkm rapmrtftl akXfbliif 8.

HAFEKMTA

Dmol rapmrtftm smprf, kli dfsm ha lsmrham klmkftfil, plr omita LDO asleka diakada smkm smiatfolktl fspldada (hacdmit kliifiaolktfzamkl,

vmoatm, attlrm, saktmoftmimeaf fdutf klurmimeadf ltd.) l saoaia f quliiadnl sa msslrvfkm kla ifttfkta dmk akclzamkl elklrfiazzftf (slpsa). Zlrtfktmak uk klmkftm erfvlolktl ofiftm, kliif hafekmsa hacclrlkzafil basmekfsloprl dmksahlrfrl if pmssabaiat ha uk LDO l, sl iakhadl ha smsplttm fitm, lcclttufrl akhfeaka spldadnl plr ukf dmkclrof hafekmstadf (Cae.

6). \kfkfoklsa ha prmerlssavm hltlramrfolktm diakadm ak uk klmkftmprldlhlktl kmrofil hlvl sloprl sueelrarl uk lrrmrl dmkelkatm hlioltfbmiasom. Fa ka hafekmstada utail radmrhfrl dnl omitl ofifttal el-kltadnl m ilsamka plrakftfia dfusfkm fitlrfzamka hfi omolktm stlssm hliif


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kfsdatf.Fiilsfol mbalttavm pmssabail rasdmktrfrl sleka diakada fspldada(lpftmolefiaf, dmakvmieaolktm hli sastlof klrvmsm dlktrfil). Mddfsam-kfiolktl, ai rasdmktrm ha uk mhmrl aksmiatm pu ravlifrsa cmkhfolktfil

plr if hafekmsa. Ak omita dfsa bfstfkm pmdnl akhfeaka ha ifbmrftmramha praom iavliim plr ahlktacdfrl ai eruppm ha ofifttal (plr lslopam,if eiadloaf, ilquaiabram fdahm-bfsl, ifoomkaloaf, ifdahm ifttadm, ltd.),dmol aiiustrftm kliif Xfbliif 4.If hltlroakfzamkl hliil dmkdlktrfzamka salradnl hliifoomkam lhliilquaiabram fdahm-bfsl (pN l Badfrbmkfta) rasuitf splssm omitmutail kli hacclrlkzafrl il prakdapfia dfusl ha hasmrhaka oltfbmiada (Cae.6). Ai rasdmktrm ha vfimra lilvfta ha foomkaloaf, ak fsslkzf ha fdahmsaoltfbmiadf, smiatfolktl hltlroakftm hf hacltta lkzaoftada hli dadimhliiurlf (cmrof praoatavf ha aplrfoomkaloaf), dmol pmssabailkmtfrl kliif ceurf 6.Xuttfvaf blkl radmrhfrl dnl iaplrfoomkaloaf kli klmkftm pu lsslrlsldmkhfraf f dfusl kmk ha kfturf elkltadf, qufia0 fsssaf klmkftfil,

hastrlss rlsparftmram hli prlofturm, bfssm plsm fiif kfsdatf, aplrfoom-kaloaf trfksatmraf hli prlofturm, akclzamka urakfral hf Zrmtlus, snuktpmrtm-sastloada, aksucdalkzf lpftadf fdutf.

If hafekmsaha dlrtlzzfsmiatfolktl radnalhl ukf vfralt ha akhfeaka haifbmrftmram spldadnl (plr lslopam, ifoakmfdahloaf, eia fdaha mrefkada

urakfra, ifdahm mrmtadm urakfram, ai prmim hliil fdaidfrkatakl pifsoftadnl

ltd.).Zurtrmppm plr fidukl ofifttal oltfbmiadnl kmk sloprl pmssabailahlktadfrl ai haclttm lkzaoftadm7 tuttfvaf hlkarl if hafekmsa spldadf

cmkhfolktfil plr cmrkarl if dmksuilkzf elkltadf fiif cfoaeiaf. F tfikl, sa hmvrlbbl dlrdfrl ha cfrl meka tlktftavm plr hltlroakfrl if haf-ekmsa olktrl ai bfobakm fkdmrf vavm, hftm dnl ilsfol futmptadm kmksloprl utail.Zlr qufktm raeufrhf eia LDO rlspmksfbaia ha ukf dmoprmoassamklklurmimeadf fdutf dmk aksmrelkzf tfrhavf,if hafekmsa rasuitf omitlvmitl pa hacdail, ak qufktm ak qulstm eruppm ha ofifttal ilsmrham pa

aksahamsm. A pfzalkta fccltta hf qulstl cmrol ha LDO pmssmkm emhlrl habumkf sfiutl plr fkka m hldlkka l ofkaclstfrl pma fiiaoprmvvasm ai haclttmoltfbmiadm dmk uk qufhrm klurmimeadm fdutm (elklrfiolktl sdftlkfta hfukm strlss m hf uk lpasmham fspldadm dmol ukakclzamkl fkdnl bfkfil).

Omitm prmbfbaiolktl, qulsta pfzalkta pmssalhmkm ukfttavat lkzaoftadfrlsahuf sucdalktl plr pmtlr dftfbmiazzfrl ai substrftm ak dmkhazamka

ha blklsslrl diakadm7 tuttfvaf, ak dmkhazamka ha strlss dftfbmiadm (qufia

erfvahfkzf, akclzamka aktlrdmrrlkta, ltd.) ifttavat lkzaoftadf rlsahufkmk ak erfhm ha prlvlkarl ifdduouim hliil smstfkzl tmssadnl. Akmitrl,ak fiduka pfzalkta, il praol ofkaclstfzamka diakadnl pmssmkm lsslrl fspl-dadnl (ratfrhm olktfil, hldat omtmra, dmkvuisamka, lpftmpftaf) lh lsslrl


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lrrmklfolktl fttrabuatl f dfusl plrakftfia m lrrmklfolktl hafekmstadftldmol Takhrmol ha Rlyl. Ak iaklf elklrfil eia LDO dmk lsmrham tfrhavmhmvrlbblrm lsslrl prlsa ak dmksahlrfzamkl meka qufi vmitf uk bfobakm

prlslktf ukf m pa hliil sleulkta ofkaclstfzamka0 ratfrhm olktfil sakldfusf, ratfrhm hliim svaiuppm, hldat omtmra m dmkvuisamka, mhmrl aksmiatm(pfrtadmifrolktl ak mddfsamkl ha uk lpasmham fdutm), lpasmha aktlroattlktaha vmoatm akspalefbail, fdahmsa, hltlramrfolktm olktfil, dmof, lpftm-olefiaf, dfidmimsa rlkfil l akclrtaiat.

Cae. 6


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Xfb. 2- Tleka diakada fspldada ak pfzalkta dmk LDO

Taktmoa elklrfia

Ttlktftm fddrlsdaolktm

Taktmoa efstrmaktlstakfia

SmoatmRautm hliifiaolktfzamkl

Hastlksamkl fhhmoakfilLpftmolefiaf

Hafrrlf

Taktmoa rlsparftmra

ZmiapklfDrasa ha fpklf

Taktmoa klurmimeada

Tmkkmilkzf, tmrpmrl prmcmkhm, dmofXrlomra, dmkvuisamka, turbl hli tmkm ousdmifrlHasturba hliif suzamkl m hliif hleiutazamklFitlrfzamkl hla ralssa frdfada


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Xfb. 4-Fkmrofiat bamdnaoadnl lh loftmimeadnl fssmdaftl f ofifttal oltfbm-iadnl rlspmksfbaia ha saktmoftmimeaf fdutf kli klmkftm m paddmim ifttfktl

AZLRFOOMKALOAFTakhrmoa aplrfoomkaloadnl hf hldat hleia lkzaoa hlidadim hliiurlf, Mrefkadmfdahural, Hldat ha ZaruvftmDfrbmssaifsa, Hldat ha dfrkatakf{

DNLXMTA

Ofifttaf hliil urakl f sdarmppm hfdlrm, MrefkadmfdahuralEiadmelkmsa tapm A, Hldat ha Cruttmsam-6,3-hacmscftfsaHldat ha Zaruvftm Dfrbmssaifsa{

FDAHMTA

OLXFBMIADF

Mrefkadmfdahural, Ofifttaf hliil urakl f sdarmppm hfdlrmEiadmelkmsa tapm A, Efifttmsloaf, Aktmiilrfkzf lrlhatfraf ficruttmsamIfttadmfdahmsa dmkelkatl{

AZMEIADLOAF

Ofifttaf hliil urakl f sdarmppm hfdlrm, EiadmelkmsaMrefkadmfdahural, Xarmsakloaf tapm A fdutfEfifttmsloaf, Aktmiilrfkzf lrlhatfraf fi cruttmsamHldat ha cruttmsam-6,3-hacmscftfsa, Hldat ha paruvftmdfrbmssaifsaHldat ha dfrkatakf

{

TLEKA

LOFXMIMEADA

Eiadmelkmsa tapm Ab,

MrefkadmfdahuralXarmsakloaf tapm A fdutfHldat ha DfrbfoaicmscftmTaktltfsa

KL\XRMZLKAF s

ZAFTXRAKMZLKAF s

FKLOAF s

Mrefkadmfdahural, EfifttmsloafAktmiilrfkzf lrlhatfraf fi cruttmsam

Mrefkadmfdahural, EfifttmsloafAktmiilrfkzf lrlhatfraf fi cruttmsam

{


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ZRMEKMTA

A cfttmra dnl smkm hltlroakfkta plr if prmekmsa hliil ofifttal oltfbmiadnl

dnl dfusfkm uk qufhrm ha dmoprmoassamkl klurmimeadf fdutf smkm havlrsa,of dlrtfolktl trf a pa aopmrtfkta fbbafom0 lt fiif hafekmsa, erfvat hliqufhrm diakadm klmkftfil (plr il cmrol dmk lsmrham klmkftfil), elkmtapm,dmktrmiim oltfbmiadm f iukem tlroakl.

XRFXXFOLKXM

\kf vmitf pmstf if hafekmsa cmkhfolktfil, smprfttuttm plr eia lrrmradmkelkata hli oltfbmiasom dnl sa prlslktfkm kli plramhm klmkftfil, aka-zafrl aoolhaftfolktl ukf tlrfpaf spldacdf(sl haspmkabail), plr rahurrlfi oakaom ai rasdnam ha hfkkm klurmimeadm plrofklktl.Hurfktl if cfsl fdutf, cmkhfolktfil plr qulsta pfzalkta rahurrl ifd-duouim ha oltfbmiata hfkkmsa prlslkta ak dardmim, ak pfrtadmifrl f iavliimdlrlbrfil. Da ak fidukl pftmimeal rlfiazzfbail fttrfvlrsm iusm ha haltlspldafia(plr lslopam, if haltf apmprmtladf plr il mrefkadmfdahural l plra hacltta hli dadim hliiurlf), ha aktlerftmra(ls. oasdlil ha foakmfdahapravl hliifoakmfdahm prldursmrl hfkkmsm, dmol kli dfsm hliif Of-ifttaf hliil urakl f sdarmppm hfdlrm), fttrfvlrsm if staomifzamkl ha valoltfbmiadnl fitlrkftavlplr dmkslktarl if liaoakfzamkl hli dmopmstmtmssadm smttm cmrof ha uk substrftm kmk hfkkmsm l cfdaiolktl liaoakfbailhfiimrefkasom Wls. smhamclkaibutarrftm (Foomkfps) m smham blkzmftm plril aplrfoomkaloal_ m fttrfvlrsm if smooakastrfzamkl ha dmcfttmra(plr

lslopam, if Bamtakf plr if Zrmpamkadmfdahloaf m if vatfoakf B62 ak fidukadfsa ha Fdahloaf oltaiofimkadf) l akkl fttrfvlrsm iusm ha oltfbmiata

dfrlkta (plr lslopam, ifreakakf kla Hacltta hli dadim hliiurlf). cmkhfolktfil akmitrl vfiutfrl sloprl fttlktfolktl im stftm haahrftfzamkl, cmrkarl ukfhleuftf fiaolktfzamkl (kmrom-aplrdfimradf),dmrrleelrl lvlktufia squaiabra oltfbmiada sldmkhfra fssmdafta (qufia if-dahmsa l/m iaplrfoomkaloaf plr il mrefkadmfdahural, iapmeiadloaf plr

a hacltta hliimssahfzamkl hleia fdaha erfssa). Omitl vmitl, qulsta pfzalktasmkm dmstrltta f sleuarl haltl omitm rastrlttl, plrtfktm plr cfvmrarl ukfhleuftm svaiuppm cmkhfolktfil suppilolktfrl if haltf dmk aktlerftmram vatfoakl.Eia mbalttava hliif tlrfpaf f iukem tlroakl hliil ofifttal oltf-bmiadnl dnl dfusfkm uk qufhrm ha dmoprmoassamkl klurmimeadf fdutfsmkm0 ofktlklrl kliif kmrof il dmkdlktrfzamka pifsoftadnl l urakfral

ha oltfbmiata tmssada, cfvmrarl ukf drlsdatf kmrofil l uk fhleuftm stftmkutrazamkfil, dmkslktarl uk kmrofil svaiuppm psadmomtmram l akkldlrdfrl ha lvatfrl lpasmha ha sdmoplksm oltfbmiadm (ls. fdahmsa plr ilmrefkadmfdahural).


10/23

Xrf a kumva fpprmdda tlrfplutadafppiadfta fh fidukl ofifttal oltfbm-iadnl, fbbafom a trfpafkta (plr lslopam, plr il Mrefkadmfdahural) l iftlrfpaf elkadf7 tuttfvaf a hfta haspmkabaia ak ilttlrfturf kmk smkm fkdmrf

omitm akdmrfeeafkta.Kli dmrsm hli cmiimw-up, ha erfkhl aopmrtfkzf kli bfobakm dmk ofifttaloltfbmiadnl, vfiutfrl plramhadfolktl ilquaiabram oltfbmiadm, if drlsdatf,ifhleuftlzzf hliifppmrtm kutrazamkfil, im svaiuppm psadmomtmram l ifprlslkzf ha lvlktufia prmbiloa psadmimeada. Akkl, omitm aopmrtfktl

omkatmrfrl fttlktfolktl if dmopfrsf ha lvlktufia dmopiadfkzl sastloadnl(plr lslopam, iaksucdalkzf rlkfil drmkadf kla pfzalkta dmk Oltaiofimka-dmfdahloaf l if dfrhamoampftaf kla pfzalkta dmk Zrmpamkadmfdahloaf) plrpmtlr dlrdfrl ha olttlrl ak fttm (sl lsastmkm) fhleuftl strftleal prlvlktavlplr oaeiamrfrl if qufiat ha vatf ha qulsta pfzalkta.


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B. FZZRMDDAM FI ZFPALKXL DMK HFKKM

KL\RMIMEADM DRMKADM

F hacclrlkzf hli hfkkm klurmimeadm fdutm, if dua hrfooftadat ha prl-slktfzamkl diakadf akhudl pa cfdaiolktl f smsplttfrl ukf ofifttaf oltf-bmiadf, if hafekmsa ha uk bfobakm dmk uk ratfrhm ha svaiuppm ilktfolktlprmerlssavm m kmk prmerlssavm, asmiftm mh fssmdaftm fh fitrm hasmrhaklklurmimeadm m fh fitrl ofkaclstfzamka sastloadnl, uk prmbilof pa dm-oukl l ha pa hacdail rasmiuzamkl. Kmkmstfktl qulstf hacdmit, il dfusl

elkltadnl hlvmkm sloprl lsslrl dmksahlrftl lh lsdiusl plr il aopiadfzamkatlrfplutadnl l ha dmksaeiam elkltadm fiif cfoaeiaf.

Il prakdapfia ofifttal oltfbmiadnl dnl sa pmssmkm prlslktfrl dmk ukqufhrm ha hfkkm klurmimeadm drmkadm smkm0 if clkaidnltmkuraf7 imomdastakuraf7 il ofifttal iasmsmoafia7 il ofifttal oatmdmkhrafia7 il ofifttal plrmssasmoafia7 il vfrafkta olkm erfva hliil lkzaompftal dnl, kliif cmrof difssadf,

smkm fssmdaftl f hfkkm klurmimeadm fdutm(hasdussl prldlhlktlolktl).

Il ofkaclstfzamka diakadnl, il akhfeaka ha fpprmcmkhaolktm hafekmstadml il lvlktufia tlrfpal ha qulstm eruppm ha pftmimeal smkm rapmrtftl aktfbliif


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