Amaurosis Congénita de Leber

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N ico{`orni`ùo yb hngjn kb by{n bodbwlbknk kb jn irnj hnp qcin `odcwlni`ùo* kcokb yb k`yir{bo tnw`cy {àwl`ocy gw`oknkcy qcw k`y{`o{cy nr{cwby p njerony wbi`bo{by `otby{`eni`coby nibwin kb jn Nlnrwcy`y icoeào`{n kb Jbgbw (NIJ) {ckc by{c bo ro jboernmb icjc~r`nj qnwn ~rb {ckc n~rbj ~rb jc jbn qrbkn bo{bokbw p nqwbokbw lîy ycgwb by{b {bln/ Qnwn blqb}nw by `lqcw{no{b nijnwnw ~rb jn NIJ oc by jc l`ylc ~rb jn Nlnrwcy`y Dren}* qrby{c ~rb bo jn gýy~rbkn kb ron wbyqrby{n bj ryrnw`c qrbkb jjbenw n icodrok`w jcy {àwl`ocy/ Jn NIJ by ron bodbwlbknk nr{cyùl`in wbiby`tn (NW) irpn inryn by eboà{`in* y`bokc y`bokc kb by{b wnlc jn lîy dwbirbo{b kb kb{bw`cwc t`yrnj bo jni{no{by p o`þcy/ (Mnif M/ Fnoyf` 7115)/ Bj {àwl`oc kb nr{cyùl`ic wbiby`tc ~r`bwb kbi`w ~rb bo by{b qn{wùo hbwbk`{nw`c jcy kcy qnkwby kbj qni`bo{b yco qcw{nkcwby* p by inrynkn byqbiìd`inlbo{b qcw lr{ni`coby bo bj ebo N@QJ> (wbibq{cw kb nw`j h`kwcinwgrwcy

Qbwc Wcgbw{ F/ Fcbobfccq bo bj 7112 lboi`con jny lr{ni`coby bo 8 eboby (N@QJ>* IWG>* IWX* ERIP7K* @LQKH>* WKH>7* WQB42 WQEW@Q>) iclc inryno{by kb jn NIJ lcy{wnokc ro nj{c ewnkc kb hb{bwcebob`knk ijìo`in p eboà{`in/ Ybeýo `otby{`eni`coby wbi`bo{by jn N@QJ>* by obibynw`n qnwn jn t`ng`j`knk p droi`conl`bo{c kb jny iàjrjny dc{cwwbibq{cwny kb icoc* by kbi`w* ~rb by obibynw`n qnwn bj droi`conl`bo{c p jn yrqbwt`tboi`n kb jcy icocy qrby{c ~rb jn dnj{n kb N@QJ> qwckrib ron kbyby{ng`j`}ni`ùo wîq`kn kb jn gnwwn kb jn dcydck`by{bwnyn/ Jny dcydck`by{bwnyny c orijbnyny yco bo}`lny h`kwcjnyny ~rb in{nj`}no jn wrq{rwn kb jcy bojniby dcydck`ày{bw/ (Kbtj`o* [/ L/ 711;) Jcy icocy yco iàjrjny yboy`gjby n jn jr} ~rb yb boirbo{wno y`{rnkny bo jn wb{`on kb jcy tbw{bgwnkcy* bo jn jjnlnkn inqn dc{cwwbibq{cwn/ By{ny iàjrjny yco jny wbyqcoyngjby kb jn t`y`ùo bo icjcwby/ Jny ybþnjby ebobwnkny bo jcy icocy yb {wnoyl`{bo bo jn wb{`on n jny iàjrjny g`qcjnwby ~rb icobi{no ico jny iàjrjny enoej`conwby kb kcokb qnw{b bj obwt`c ùq{`ic ~rb botìn jn `odcwlni`ùo nj ibwbgwc/ (Nw{hrw I/ Erp{co*Mcho B/ Hnjj= D`y`cjceìn làk`in* 711>) Ron h`kwcjnyn by ron bo}`ln inqn} kb h`kwcj`}nw ro bojnib ~rìl`ic/ Qcw bmblqjc* ron bo}`ln ~rb in{nj`ib jn wbnii`ùo y`er`bo{b ybwî ron h`kwcjnyn= N ’G & H7C ↗ N’CH & G ’H

Ro`tbwy`knk Nr{ùocln kb Nernyinj`bo{by Jn h`kwùj`y`y by ron wbnii`ùo ~rìl`in bo{wb nern p c{wn yry{noi`n* iclc ynjby* (qrbkb kbi`wyb ~rb bj nern wbnii`con 'wclq`bokc bj iclqrby{c')/ By{c qwckrib ro kbyqjn}nl`bo{c kbj b~r`j`gw`c kb k`yci`ni`ùo kbj nern p iclc icoybirboi`n yb lck`d`in bj tnjcw kbj qH/ Jn in{îj`y`y by bj qwcibyc qcw bj irnj yb nrlbo{n c k`yl`orpb jn tbjci`knk kb ron wbnii`ùo ~rìl`in/ (Rjjlnoo%y Boipijcqbk`n cd  @okry{w`nj Ihbl`y{wp 7117) Jcy bojniby dcydck`ày{bw yco byboi`njby qnwn jn t`kn* qrby yco jcy wbyqcoyngjby kbj by~rbjb{c kb jny hbgwny kb NKO p NWO/ Jn h`kwùj`y`y kb jcy bojniby dcydck`ày{bw qrbkb ybw in{nj`}nkn qcw jn nii`ùo kb jny dcydck`by{nwnyny* ~rb mrbeno ro qnqbj `lqcw{no{b bo jn wbqnwni`ùo kb jny ybirboi`ny kb NKO/ (Kbtj`o* [/ L/ 711;/ G`c~rìl`in* ;ª bk`i`ùo/ Wbtbw{à* Gnwibjcon/) Bxqbw`lbo{cy wbi`bo{by ocy hngjno kb ~rb jn ewntbknk kb jn bodbwlbknk yb wbdjbmn bo jn qàwk`kn iclqjb{n kb t`y`ùo p kb wîq`kn kbebobwni`ùo kb jcy dc{cwwbibq{cwby bo jn wb{`on kb jcy wn{coby kbd`i`bo{by bo N@QJ>/ (Hrl/ Lcj/ Ebob{/ (71>1) >0 (4)= >154,>185) Jcy qîwwndcy no{bw`cwby bxqj`ino ~rb lr{ni`coby bo bj ebo N@QJ> ciny`cono ron (Kbq{/ ewntb iberbwn `odno{`j/ cd  Cqh{hnjlcjcep* UT Ro`tbwy`{p Bpb @oy{`{r{b* Cob Y{nk`rl Kw/* Q/ C/ Gcx 0>09* Lcweno{cuo* Ybq{* 7110)/ By{n bodbwlbknk yb qwbybo{n ico iberbwn nj onibw c bo jcy qw`lbwcy nþcy kb t`kn/ Lrihcy o`þcy ndbi{nkcy tbo lbmcw ico ron `jrl`oni`ùo gw`jjno{b/ (Mnif M/ Fnoyf` 2{n bk`i`ùo/)

Nj onibw jcy qni`bo{by yco i`becy c ybtbwnlbo{b kbd`i`bo{by t`yrnjby qrbkb hngbw o`y{nelc* dc{cdcg`n p wbyqrby{n qrq`jnw jbo{n c nrybo{b/ (Gwrib B Lccwb >005) Jn NIJ by ron bodbwlbknk kbj {nqb{b wb{`o`noc ~rb icoy`y{b bo k`y{wcd`n p inlg`cy kbebobwn{`tcy bo jny obrwcony enoej`conwby* bq`{bj`c q`elbo{nw`c p ybelbo{cy bx{bwocy kb jcy dc{cwwbibq{cwby/ Bj dcokc kb cmc kbj `odno{b (qnw{b qcy{bw`cw c `o{bw`cw kbj cmc bxnl`onkc kbykb drbwn n {wntày kb jn qrq`jn `lnebo ijìo`in/ Lbki`ijcqàk`n) qrbkb qnwbibw ocwlnj p ico bj qnyc kbj {`blqc kbynwwcjjnw= N{wcd`n ùq{`in* wb{`ocqn{ìn q`elbo{nw`n* kwryny p inlg`cy q`elbo{nw`cy p n{wùd`icy lnirjnwby lîy ewntby* n{borni`ùo kb jcy tnycy kb jn wb{`on p ~rbwn{cicoc qbwc oc nyci`nkc nj ebo (Gwrib B Lccwb >005) p (Mnif M/ Fnoyf` 7115) C{wcy hnjjn}ecy bo dcokc kb cmc yco bkbln qnq`jnw* inlg`cy bo ‚ynj p q`l`bo{n“* qro{cy gjnoicy k`drycy* icjcgcln lnirjnw p lnirjcqn{ìn ‚bo cmc kb grbp“/ (Mnif M/ Fnoyf`

2{n bk`i`ùo/) Yrbjb qwckri`wyb {nlg`ào qnj`kb} qnq`jnw p n{borni`ùo kb jny nw{bw`cjny wb{`o`nony/ C{wcy y`eocy cirjnwby yco= 

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Ro`tbwy`knk Nr{ùocln kb Nernyinj`bo{by Jn BWE (Bjbi{wcwb{`ocewndìn) yrbjb ybw `okb{bi{ngjb `oijryc bo jcy inycy qwbiciby ico ro dcokc kb cmc ocwlnj/ (Mnif M Fnoyf` 2{n bk`i`ùo)

‚T`y`ùo ro nþc kbyqrày kb jn {bwnq`n eào`in

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