A Stargardt mate: whakaahuatanga, ai, tohu me ngā āhuatanga maimoatanga

mate o Stargardt ko whakaoho he tukanga degenerative i roto i te tongi. He whare hauora maha mate i te mea rite ki tenei māuiuitanga. E meinga ratou e te irakētanga o ira rerekē. Na reira, no te mate ki te kāwai o pathologies tuku.

Ko te faaiteraa haumanu matua o te mate ko te tukanga degenerative i roto i te maturuturunga iho tongi me ngarahu retinitis pokapū meinga o te whanaketanga o te scotoma pokapū.

Āhuatanga o te mate

mate o Stargardt ko te pathologies onge, engari tino nanakia. faaite te reira ano i te kuao tau - i te 6 ki te 20 tau ki te auautanga o te 1: 20,000. I roto i te tahi atu ngā kāwai tau māuiuitanga, rite ki te tikanga, e kore e vstrechvaetsya. Ko te mau hopearaa o mate catastrophic. Ehara i kingi i roto i te mate oti o kite.

Ko te mate he pūtake ira. pānga tukanga Dystrophic rohe macular tuaroa, me te mea pūtake i roto i te epithelium ngarahu, e tika ana ki te mate o te titiro. He më te tukanga.

puka pathological

He he he mārama wehenga o māuiuitanga ki wha momo i runga i te rohe o te localization inflammatory rānei:

Ka taea te kite tukanga degenerative:

• i roto i te waenganui o te rohe piringa;
• i roto i te rohe macular;
• i roto i te rohe paracentral.

Ano, i reira te mea he ahua whakauru o te mate e whai wāhi mumura paetata i roto i te wahi pokapū o te kanohi, me te i runga i te taha.

Tikanga o te whanaketanga mate

Kua whakaahuatia te take o te mate rata Karl Stargardt i te hawhe tuatahi o te rautau rua tekau. I roto i tona kororia, me te ingoa i te mate. pā ana te māuiuitanga e ki te rohe macular me, rite ki te kaiputaiao, whakawhiti i roto i te kainga tupu te utuafare. Ko te tikanga whakaritea Polymorphic pikitia ophthalmoscopic, e huaina nei i te ingoa o "atrophy o te kamo," "te kanohi o puru", "parahi whati", me ētahi atu.

Na roto i tāutuhia tū wāhi cloning ariki ira i e ai retinitis pigmentosa, he tino kitea i roto i te photoreceptors. I roto i te pūtaiao e karangatia ana ko ABCR.

mate kanohi riro te roto i te tikanga tino autosomal. Hoki pathologies āhuatanga e localization o ira mutated i roto i ngā pūira 13q me 6ql4.

E kore te te pūtake ira i runga i te ira tangata. E ai ki te rangahau tata o geneticist doctor-, me te momo tino o te tuku o te mate, e te reira te iti moka māmā, me e kore e arahi tonu ki te matapo. tonu te nuinga o receptors pūtau ki te mahi. I roto i enei tūroro, puta te māuiuitanga tohumate iti. Pāngia pupuri ana mahi me te taea o te peia waka.

Pehea he mate o Stargardt? Ko te take o e hōloa macular nga pūtau takoto i roto i te kore o te pūngao. Gene koha whakaoho hanga o te pūmua hapa e whakawhiti ai ngā rāpoi ngota ATP roto i te membrane pūtau macular wahi pokapū o te etine. arotahi te reira i whakairoiro image me te tae.

māuiuitanga tohutanga

He aha e te mau huru o te iwi ki te mate o Stargardt? Ki te whanaketanga o te māuiuitanga mate kore e kitea i roto i te tahi atu whekau. pānga te tukanga nga kanohi anake. tīmata te mate ki te mate o te acuity ataata. Kei te piki haere tohu i muri, a reira he maumaui matotoru o te tirohanga o te tae katoa i runga i te tirohanga ka mohio o te whānuitanga spectral.

huringa degenerative e whakaaturia o te fundus i roto i te whika whai ake:

• i roto i te kanohi e rua tohua pigmented āhua ira porowhita;
• i reira he wāhi rohe kowaiwai o te ngarahu;
• tukanga atrophic ua faariro i roto i te epithelium ngarahu tuaroa.

kia puta taua tukanga i roto i te taha ki te takanga o wahi repa-kōwhai. Ka rite ki te mate kauneke i roto i te epithelium ngarahu tuaroa whakakitea nui ake haaputuputuraa o taonga lipofuscin-rite.

tūroro katoa pāngia e tenei māuiuitanga, kitea scotoma pokapū tino kanohi ranei, e whai uara rerekē, e tika ana ki te nuinga o te tukanga.

A, no te taea noho zheltopyatnistoy pouaka āhua o te manawanui tangata i roto i te whānuitanga noa, engari kei te mārika i heke te kitenga pokapū.

Ko te nuinga o ngā take o te iwi e kua pehia te rangahau, i tuhia ai he takahi o te tirohanga o ngā tae deuteranopia momo, diskhromazii whero-matomato. A, no te taea e tino watea tukanga dystrophic zheltopyatnistom o kite tae.

te whakamamaetia ma'ii'o Stargardt he huringa i roto i te tūtohutanga auau rerekē mokowā (i roto i te tukanga Mataora 6 - 10 º ki whakaiti nui i roto i te rohe o iarere mokowā waenga, me te i roto i te oti ngaro o te rohe auau tiketike). huaina te syndrome ko "tauira o tihi ma'ii'o."

Tohu ngā kitenga mawhatu o mea, wahi matapo, blurriness, tae me te takahi o te fifi urutaunga i te aroaro o te marama atarua.

tātaritanga mate

Ka rite ki te hua o te rangahau i te taumata iranga e whakakitea taunakitanga e takoto te mate kanohi Stargardt disorders allelic wāhi ABCR.

Tātaritanga o te mate haamata ki te ako o te kāwanatanga o te fundus. Whakamana angiography fluorescein o iaia tuaroa, me te whakamātautau electrophysiological. I roto i tenei take, e hiahiatia ana te faaotiraa o te tohu o te kino te. hanga tenei e taea ki te tautuhi i te maha o ngā tohu mate, tae atu ki ngā whitiki giperflyuorestsiruyuschie me fluorescence o te karere whatu.

Mai te mate, ko te āhua tuku, te ara tika te nuinga o te whakatau tenei tātaritanga e kia i te DNA, i te mea ahei ki te tautuhi i mate tuku monomonogennye.

tātaritanga pārōnaki

kia mate a Stargardt te wehenga o te tihi-tokotoko-ki, o koeko, me te rakau-o tihi ma'ii'o, te ma'ii'o foveal ahu whakamua, tino, Juvenile Retinoschisis, macular ma'ii'o vitelliformnoy.

Ano, whakaaturia pārōnaki tātaritanga kua i roto i te take o te hapūtanga toxemia riro e hōloa inenga tika (i roto i ngā, ka retinopathy hlorokvinovoy).

Ka taea te ora i te reira mate o Stargardt?

e kore e therapy Etiologic tīariari. faaore te reira i mate o te Stargardt? Treatment pūāhua pŭpŭ tauturu whai wāhi te werohanga whakamahi parabulbar o taurine me te tikanga antioxidants introduction kuhunga whakawhānui (pentoxifylline, waikawa nicotinic), me steroids.

huaora kāore e whakatairanga taiepa oko, me te whakapai ake i tohanga toto. huaora whakamahia nuitia B, A, C, E.

Kei te whakaaturia e pupuri ana te reira i tua. I roto ia ratou e ngā tatou electrophoresis te whakamahi i taero, me whakaongaonga tuaroa i te taiaho.

revasulyarizatsiya etine tīpakongia i transplantation ki wāhi macular o muka uaua.

I mua whakapono ophthalmologists huri noa i te ao, e kore te mea treatable te mate. Ko te whakaora i runga te āwhina paku anake whakaroa te ahunga whakamua o te mate, engari i roto i kahore take e kore e ārai i te reira.

Ka rite ki te tikanga, e kore te maimoatanga i whakaora i te iwi i te mate oti o kite. E hiahiatia ana ki te whakawhanake i tētahi huarahi e e whakahaere i te faaho'iraa mai o te hanganga ocular papaka.

Ko te tikanga auaha o te therapy

He reira tēnei wā he tikanga whai hua mō te tango o taua māuiuitanga rite te mate o Stargardt? Me pēhea te ki te hamani i te mate nui? Kua te tikanga kua whakaarohia i roto i te 2009 e Dr. Aleksandrom Dmitrievichem Romaschenko, e ko te upoko o te Center Ophthalmology "ON hauora" (Saint-Petersburg, ul. Marata, whare 69, hanga B). i ake ratou kōwhiringa mate maimoatanga e hāngai ana i runga i te maimoatanga kiko pūtau.

Ko te pūtake o te maimoatanga te tono o ngā pūtau kakau o te kiko tangata i pangia adipose he. I whakawhanakehia e te tikanga whakaora e pūtaiao mua VP Filatov. Ngā mihi ki te hangarau auaha o te tūroro e hoatu te whai wāhi ki te whakahoki i te kite ngaro me whakarato he ora tonu.

rēhita Dr. AD i Romashchenko hangarau biomedical matatini, me te arai i nga tikanga e whai ake nei o te maimoatanga kakau pūtau :

• tikanga ngā o te tango i te ahua mākū o te mate;
• tikanga matatini paitogeneticheskogo pokapū therapy me tapetoretinalnoy ma'ii'o.

I tetahi whare hauora ko te maimoatanga?

kua te maimoatanga o mate matatini kua pokapū ophthalmologic "I hauora". Kei te pokapū i roto i te pa kia rite ki St Petersburg. Hamani mate o Stargardt taea anake e i te pokapū, mai te mea te reira i te kotahi anake i roto i Russia, te wahi tono taua hangarau te.

He he haumaru therapy kakau pūtau?

He pakari taea ngaio whakaū e te therapy hangarau whakawhanakehia AD Romashchenko, he haumaru tino. Hoki te maimoatanga o ngā pūtau o te manawanui e whakamahia, e faaore te taea o te pato'iraa o te tahi atu mau hopearaa ino ranei.

mutunga

Shtargradta tuatahi mate i te wawe tau, me te hohoro arahi ki te mate tino o te kite. I roto i te wā tino onge, kainga momo tino, pata kanohi āta. E tohutohu tūroro ki te haere ki te ophthalmologist, huaora, me te mau mohiti. Ko te ara whai hua tino ki te whakakore i te māuiuitanga therapy whakaaro e pūtau kakau.