Stargardt nechirwere: tsananguro, hunokonzera, zviratidzo uye kurapwa mamiriro

chirwere Stargardt raShe chinopedza kwacho chinokurwadzai iri macula. Pane zvirwere zvizhinji kiriniki iri rakafanana zvokurwara ichi. Vari yakakonzerwa mutation majini siyana. Saka, hosha ndohwaJehovha muchikwata che enhaka pathologies.

The chikuru kunotoda nekuratidza chirwere chinhu chinopedza kwacho macula uye nechepakati retinitis ruvara kukonzera kuderera kukura munhu nechepakati scotoma.

Zvakakurukurwa chirwere

chirwere Stargardt kwakaita haiwanzowaniki asi kwazvo pathologies. Kunoratidza pacharo pane muduku - kubva 6 kusvika 20 makore ane kakawanda 1: 20,000. Nemamwe zera Categories zvokurwara, sezvinoita mutemo, kwete vstrechvaetsya. Zvakaipira nemafashamo zvirwere. Not warambidzwa kurasikirwa wakakwana wezvakaonekwa.

The chirwere ane majini hwaro. Dystrophic muitiro kunokanganisa retinal macular nharaunda uye zvinobva mukati ruvara epithelium, iyo inotungamirira kurasikirwa kuona. Zvichiitika bilateral.

pathological chimiro

Pane kupesana pachena zvokurwara kupinda nezvemarudzi zvina zvichienderana nzvimbo anogumbura localization:

Chinopedza muitiro unogona kuonekwa:

• vari pakati peripheral mapazi;
• muna macular nharaunda;
• ari paracentral mapazi.

Uyewo, pane rakasangana chimiro chirwere kuti kunosanganisira kuzvimba vemunyika nechepakati chikamu ziso uye pamusoro arikunze.

Zvigadziriswe zvirwere kukura

Zvinokonzera chirwere zvinorondedzerwa chiremba Karl Stargardt rokutanga yezana remakore rechimakumi maviri. In rukudzo ake uye zita chirwere. The zvokurwara iri chokuita macular nharaunda uye, maererano musayendisiti, anonzi akatamisira mumhuri nhaka. Kazhinji akasarudzwa polymorphic ophthalmoscopic mufananidzo, iro rine zita "atrophy ose ari choroid," "ziso chenzombe", "ndarira yakaputsika", uye vamwe.

Kuburikidza positional cloning tenzi Gene locus akaziviswa chinokonzera retinitis pigmentosa, chinonyanya kuoneka photoreceptors. In sainzi rinonzi ABCR.

Ziso hosha iri nhaka munzvimbo autosomal kureva nenzira. For pathologies hwokuratidza localization pamusoro mutated majini mu okutodza 13q uye 6ql4.

The majini hwaro hakutsigirwi chechikadzi. Maererano zvichangobva kudzidzwa pamusoro doctor- geneticist, pamwe chikuru mhando kwemashoko chirwere, zvinotora zvishoma nyore, uye hakuiti kutungamirira upofu. Most Sero receptors kuramba ichishanda. In varwere aya, zvokurwara kunoitika shoma zviratidzo. Kwakaitwa anoramba achishanda uye mukana kutyaira motokari.

chirwere Stargardt kwakaita sei? Zvinokonzera macular degeneration masero nhema achishayiwa simba. Gene kuremara chinokurwadzai kugadzirwa chakaremara mapuroteni iyo Transfers ATP mamorekuru kuburikidza sero Kamudzira macular nechepakati chikamu the retina. It pfungwa mufananidzo Graphics uye ruvara.

symptomatology zvokurwara

Ndezvipi zvainyanya zvevanhu Stargardt chirwere? With kukura chirwere zvokurwara haina kuchengetwa mune dzimwe nhengo dzomukati. Kwacho kunokanganisa meso chete. The chirwere chinotanga kurasikirwa kutarisana acuity. Papera zviratidzo zviri kuwedzera, uye varipo kwakakomba kutyorwa omumwe ruvara zvose pamusoro vakanzwisisa maonero spectral kwaasingasvikiri.

Chinopedza kuchinja zviri fundus vanoonekwa vari zvinotevera nenhamba:

• mukuona vose kwakaratidza pigmented dzeraundi dot chimiro;
• pane dzimwe nzvimbo omunharaunda isina ruvara;
• akaona atrophic kwacho retinal ruvara epithelium.

pezvakaitwa kwakadaro kunogona kuitika munyika pamwe panowanikwa whitish-yellow makwapa pakati. Sezvo chirwere mberi mune retinal ruvara epithelium yakaratidza rakawedzera kuunganidza lipofuscin-kufanana fuma.

Varwere All kukanganiswa zvokurwara ichi, padzakacherechedza Mhedziso kana zvishomanana nechepakati scotoma, vane tsika dzakasiyana, nokuda kuwanda kwacho.

Kana zheltopyatnistoy shaped bhokisi murwere anogona kuramba kuipa-siyana, asi musimboti chiono ipfupi chaizvo.

Kakawanda kacho vanhu vanenge inini ari kuongorora, zvakanzi kuzivikanwa kutyorwa omumwe mavara deuteranopia mhando, tsvuku-rwegirini diskhromazii. Kana zheltopyatnistom dystrophic basa ruvara chiono kunogona zvachose dzisisina.

Dystrophy Stargardt kaviri kuchinja mune spatial kakawanda musiyano wanda (pakupfuura mumatanho 6 - 10 º pamwe zvinokosha rutapudzo munzvimbo yapakati spatial frequencies uye kusavapo zvizere dzamatunhu kakawanda nharaunda). The chirwere anonzi "mufananidzo cône dystrophy."

Zviratidzo zvinosanganisira Wavy chiratidzo zvinhu, mapofu makwapa, blurriness, ruvara uye kutyorwa kwadzakabva kuoma pamberi haachaoni chiedza.

chirwere kuongorora chirwere

Somugumisiro tsvakurudzo panguva majini pamwero pachena uchapupu kuti ziso chirwere Stargardt akasarudza allelic zvinetso locus ABCR.

Chirwere chirwere chinotanga kudzidza mamiriro ose fundus. Akaita fluorescein angiography pamusoro retinal vascular uye electrophysiological kuzviongorora. Panyaya iyi, kutsunga ukuru kukuvara runodiwa. Izvi zvinoita kuti tizive zvinoverengeka chirwere zviratidzo, kusanganisira giperflyuorestsiruyuschie muhomwe uye fluorescence pamusoro optic etsinga.

Sezvo chirwere chacho chichigarwa nhaka hunhu, nzira yakarurama zvikuru kuziva nechirwere ichi yaizova panguva DNA, izvo anokwanisa kuziva nhaka zvirwere monomonogennye.

differential kuongorora chirwere

chirwere Stargardt zvinofanira kuva kuparadzana cône-tsvimbo-pamwe, kuti cône, uye seinä-kubva cône dystrophy, mberi foveal dystrophy, kureva, Juvenile Retinoschisis, macular dystrophy vitelliformnoy.

Uyewo, differential chirwere kunoratidzwa kana pamuviri toxemia vakatodzidza yakafanira chipimo chemushonga degeneration (kunyanya kana hlorokvinovoy retinopathy).

Chinorapika chirwere Stargardt raMwari?

Etiologic kurapa haako. It anobvisa chirwere ari Stargardt raMwari? ebetsero hunhu Treatment kunosanganisira kushandisa parabulbar jekiseni taurine uye antioxidants sumo zvinoreva Achitambanudzira pangagara (pentoxifylline, nicotinic acid), uye steroids.

Untreated mavhitaminzi kuti pave mudziyo rusvingo uye kuvandudza kweropa. mavhitaminzi yaiwanzoshandiswa B, A, C, E.

It kunoratidzwa vakabata physiotherapy. Pakati pavo tinofanira kutaura electrophoresis kushandisa zvinodhaka, uye retinal kurudziro kupfurikidza Laser.

Zvakarurama revasulyarizatsiya the retina nokuda transplantation pamwe macular nzvimbo tsandanyama fibers.

Kare ophthalmologists kupota nyika vaidavira kuti hosha haina rinorapika. The pamusoro dzokurapa kubatsira chete zvishoma kunonotsa kuchiita hosha, asi hapana nyaya kutadzisa kuita izvozvo.

Kazhinji, mishonga haina kuponesa vanhu kubva kurasikirwa chiratidzo chakakwana. Vaifanira kuva nzira yaizoita aivabatsira kudzorerwa hunenge ocular hurongwa.

The itsva Nzira yokurapa

Pane pari zvinobudirira nzira kuti kubviswa zvokurwara akadai Stargardt chirwere? How kurapa chirwere chakakomba? Nzira yave wakatangwa muna 2009 naDr Aleksandrom Dmitrievichem Romaschenko, uyo ari musoro Ophthalmology Center "MUSI Clinic" (Saint-Petersburg, Ul. Marata, imba 69, kuvaka B). Vakanga nani kurapwa chirwere Ipfungwa inobva cellular tishu kurapwa.

Panobva kurapwa ndiko kushandiswa hunde mumasero adipose zvinyama hosha munhu. Nzira dzokurapwa yakazokudziridzwa nemasayendisiti kare VP Filatov. Nemhaka unyanzvi varwere vanopiwa mukana kudzorera vakarasika chiratidzo uye inopa upenyu huzere.

Dr. AD rakanyoreswa Romashchenko yakaoma anorapa achishandisa mitemo rwokugadzira uye patented zvinotevera nzira hunde sero kurapwa :

• nzira kwose nokubvisa nyoro chimiro chirwere;
• nzira yakaoma paitogeneticheskogo kurapa centre uye tapetoretinalnoy dystrophy.

Pane imwe kiriniki iri kurapwa?

Mishonga yezvirwere kunzwisisa kwave ophthalmologic centre "On Clinic". Muzinda riri muguta akafanana St. Petersburg. Kurapa chirwere Stargardt vanogona chete kuva nechepakati, sezvo chete kuRussia, apo michina yakadaro rikashandiswa.

Ko hunde Sero kurapa yakachengeteka?

Nyanzvi vanogona kusimbisa chivimbo kuti mabikirwo mishonga yakatanga AD Romashchenko, iri zvachose ngozi. Nokuti kurapa masero murwere anoshandiswa, iyo anobvisa mukana kurambwa kana vamwe nemigumisiro kunaka.

mhedziso

Shtargradta chirwere debut vachiri vaduku uye nokukurumidza kutungamirira neMhedziso kurasikirwa wezvakaonekwa. Mune zvisingawanzoitiki chaizvo zvinoitika, nhaka kureva mhando, ziso anodonhedza zvishoma nezvishoma. Varwere vari zano kushanyira imwe ophthalmologist, mavhitaminzi, uye akapfeka Sunglasses. Nzira inonyatsobudirira yokuita kuti kubvisa zvokurwara vaiona kurapa nokuda hunde masero.