Stargardt ka maʻi: ano, ke kumu, symptoms a me ka hana hiʻona

Stargardt ka maʻi o ka degenerative kaʻina ka hoʻonāukiukiʻana i loko o ka macula. Aia, ua nui na maʻi? Aaieou i mea like me keia pathology. Ka mea, i ke kumu ma ka mutation o okoa nā'ōewe. No laila, i ka maʻi e pili ana i ka waeʻano o hereditary pathologies.

I ka papa kuhikuhiE lapaʻau hoike ana o ka maʻi o ka degenerative kaʻina hana i loko o ke kulu macula a kikowaena retinitis pigment ka hoʻomake 'ana i ka hooulu ana o kekahi kikowaena scotoma.

Hiʻona o ka maʻi

Stargardt ka maʻi mea he pohihihi keia akā, kaumaha loa pathologies. Ua hoike mai ai ia ia iho ma ka manawa'ōpio wale nō - mai ka 6 a hiki i 20 makahiki me ka alapine (frequency) o 1: 20,000. Ma nā makahiki waeʻano pathology, e like me ka rula,ʻaʻole vstrechvaetsya. I ka hopena o ka nalu p ka maʻi. 'Aʻole i alii iho la oia mai i ka piha lilo o ka hihiʻo.

I ka maʻi i ka aaiaoe kēia manawa. Dystrophic kaʻina e loli ai retinal macular māhele 'āina, a originates i loko o ka pigment epithelium, a hiki aku i ka poho o ka maka. Ke kaʻina Uaʻeluaʻaoʻao.

pathological palapala

Aia ka he maopopo mahele o ka pathology i loko o eha ano ke kaumaha ma muli o ka wahi o ka hoʻoʻino IIeEeAIEUAA:

Degenerative kaʻina hana hiki e malama.

• ma ka waenakonu o kaʻike ākea ciia;
• i loko o ka macular māhele'āina;
• i loko o ka paracentral ciia.

No hoi, aia no he hui ano o ka maʻi i komo ai li nui IIeEeAIEUIeXAIIUE ma ke kikowaena hapa o ka maka, a ma luna o ka periphery.

Maomeka o ka maʻi ulu

i ho'ākāka 'ia ma nā kumu o ka maʻi kahuna lapaau Karl Stargardt i loko o ka hapa mua o ka iwakalua kenekulia. I kona hanohano a me ka inoa o ka maʻi. Ke pathology ua pili i ka macular aina, a, e like me ke nä känaka 'epekema, ua hoolilo i loko o ka' ohana hooilina. Ana i koho polymorphic ophthalmoscopic kiʻi, a koho i ka inoa o "atrophy o ka choroid," "bipi ka maka", "i haki keleawe", a me na mea e ae.

Ma positional cloning haku'ōewe locus i hōʻike 'ia ke kumu no retinitis pigmentosa, he mea akaka ma ka photoreceptors. I ka 'epekema ka mea, ua kapaia o ABCR.

Eye maʻi ua ili ma ka autosomal Hoʻohaku ano. No ka pathologies wehewehe aku ma ka IIeEeAIEUAA o mutated nā'ōewe ma chromosomes 13q a me 6ql4.

Ke aaiaoe kumu ua i ka nānā 'ana ma luna o ka wahine. E like me nā haʻawina o ka doctor- geneticist, a me ka Hoʻohakuʻano o EYI o ka maʻi, ka mea, i kona wahi uuku, a 'aʻole i mau alakai i ka makapo. Hapanui aeea receptors hoʻomau i ke kuleana pili i. Ma kēia mau nā mea maʻi, i ka pathology ia lŘlŘ me ka palena haʻahaʻa symptoms. Loli ana i mālama i ka hana a me ka manawa kūpono o ka holo kaa.

Pehea o Stargardt ka maʻi? Nā kumu o macular degeneration na keena moe i loko o ka nele o ka ikehu. 'ōewe he hewa ma ka hoʻonāukiukiʻana? Iecaianoaaiiuo o kekahi hemahema kumuʻiʻo i hoʻoili ATP nā lātoma ma ka halepaahaoʻili lahilahi macular kikowaena hapa o ka retina. Uaʻeleu kiʻi kiʻi a me nā waihoʻoluʻu.

symptomatology pathology

He aha nō nā 'ano o nā kanaka me Stargardt ka maʻi? Me ka hooulu ana o ka maʻi pathology uaʻaʻole hoʻi i mālama i loko o'ē aʻe na nā loko. Ke kaʻina hana e loli wale na maka. Ka maʻi hoʻomaka me ka lilo o ka pilikia makapō acuity. Hope symptoms e mahuahua, a ma laila nō 'aʻe' loa aku la no ka pokole o ke kala a pau ma luna o ka ike nānaina o ka spectral laulā.

Degenerative loli o na fundus i ike ia i loko o ka penei huahelu:

• i loko o nā maka ia pigmented hoʻopoepoe kiko shape;
• ma laila nō i nā wahi devoid o pigment;
• ae nānā mau atrophic kaʻina hana i loko o ka retinal pigment epithelium.

Ia keʻano o ka hanaʻana i hana i loko o ka huipūʻana i ka hanana o whitish-yellow mau kiko. E like me ka maʻi progresses i loko o ka retinal pigment epithelium hoike loa hoʻomāhuahua o lipofuscin-me ka waiwai.

A pau nā mea maʻi loli ana ma keia pathology, ia akulla loa paha anoe kikowaena scotoma, a i okoa cia, aie i ka prevalence o ke kaʻina.

I ka zheltopyatnistoy hoʻokino pahu o ke kanaka hoomanawanui ke noho i loko o ka maʻamau huahelu, akā, ke kikowaena hihiʻo ua markedly hoemiia.

Ka hapanui o nā hihia o kanaka, ka poe i undergone ka anamanaʻo, ka mea i kaulana i ka 'aʻe' ana i ka pokole o ka'ōniʻoniʻo deuteranopiaʻano, ula-uliuli diskhromazii. I ka zheltopyatnistom dystrophic kaʻina hana o ke ano hihio hiki e loa unaffected.

Dystrophy Stargardt undergoes i ka loli i loko o ka māhele alapine Akä naÿe pā lonoaʻili (ma ka kaʻina kahua o ka hoʻonaʻauao 6 - 10 º me ka nui ka ho'ēmiʻana i loko o ka wahi o ka waena māhele nä alapine, a me ka piha wa e kaawale aku o ka mea kiʻekiʻe alapine māhele 'āina). Ka maʻi pale ua kapaia "kumu o kaʻohana dystrophy."

Symptoms nā wavy hihiʻo o ka mea, makapo wahi lilelile, blurriness, waihoʻoluʻu a me ka 'aʻe' ana o ka hoʻololiʻana o ka pilikia ma ke alo o ka pōwehiwehi malamalama.

maʻiʻike,

E like me ka hopena o ka noiʻi ma ka aaiaoe kiʻekiʻe e hoikeia mai kekahi hōʻike i ka maka maʻi Stargardt manao allelic kāna hana locus ABCR.

ʻike, no ka maʻi hoʻomaka me ke ao ana o ka moku'āina o ka fundus. Hana fluorescein angiography o retinal vascular a me electrophysiological ninaninau. Ma keia hihia, me ka manao paa o ke degere o ka poho ua koi 'ia. Keia i ka mea hiki ke kuhikuhi i ka helu o ka maʻi nā hoailona, me giperflyuorestsiruyuschieʻekeʻeke a me fluorescence o ka optic nā aʻalolo.

Mai ka maʻi mea hereditary ano, ka loa pololei aoao o ka hoomaopopo ana i kēiaʻike, makemake ia i ka DNA, i mea hiki ia oe, e hōʻike hereditary maʻi monomonogennye.

palena pauʻike,

Stargardt ka maʻi E e ke kaawale ana o kaʻohana-kookoo-me, o kaʻohana, a me ka maile-o kaʻohana dystrophy, e holumua ai foveal dystrophy, Hoʻohaku, nā 'ōpio Retinoschisis, macular dystrophy vitelliformnoy.

Nō hoʻi, palena pauʻike, ua hōʻike 'ia ma ka hihia o ka hāpai keiki toxemia lilo ka laʻau heluna degeneration (ma ka nui, ka wā hlorokvinovoy retinopathy).

E hiki anei ia e hoola iho la Stargardt ka maʻi?

Etiologic Inc hakuʻole. Ua eliminates ka Stargardt ka maʻi? kākoʻo ano lapaau komo ai i ka hoʻohana 'parabulbar ka pahu kui o ka taurine a me antioxidants hoike o ia hoʻi, e holo ana wahi (pentoxifylline, nicotinicʻakika), a me nā steroid.

Untreated nā wikamina e paipai moku paia a me ka hoʻoikaika 'ana i ke koko holo. nā wikamina mau hoʻohana 'ia B, A, C, E.

Ua Ua hōʻike paʻa ana physiotherapy. I waena o lākou mākou e kahińuli electrophoresis ka hoʻohana 'ana ai', a me ka retinal stimulation ma kukuna.

Haʻapili revasulyarizatsiya retina ma ka transplantation me macular 'āpana o ke Muscle olona.

Mua ophthalmologists a puni ka honua, manaoio aku i ka maʻi mea ole treatable. Ka luna therapies kōkua wale iki e hookaulua i ka Holomua o ka maʻi, akā, ma kekahi hihia, mai ole ia.

E like me ka rula, i ka iapaau ana i i hoola kanaka, mai ka piha poho o ka hihiʻo. Koi 'ia e kūkulu i ka hoʻomanaʻo i makemake kōkua i ka hoihoi hou ia ana o ka pōʻino, ua make ocular hale.

Ke kū hoʻokahi 'ana i kūpono o ka Inc

Ua loaʻa i ka hoʻokō hana no ka hoopau ana o ka pathology me Stargardt ka maʻi a ianoiyuaa a? Pehea e hana i kekahi maʻi nui? Ka papa hana i ua manaoia ma 2009 ma Dr. Aleksandrom Dmitrievichem Romaschenko, i mea no ka poo o ka Ophthalmology Center "ON ke Kalinika" (Saint-Petersburg, Ul. Marata, ka hale 69, kukulu ana i na B). Ka mea, ua hou maʻi lapaʻau hōʻoia ma muli o ka ke kelepona hele'aʻaʻa lapaau.

Ke kumu o ka lapaau o ka palapala noi o ke kumu keena o ka adipose'aʻaʻa mai kanaka. Ka pākōlea iaoia? Ac? Ia e kānakaʻepekema mua aʻe Filatov. Mahalo i ka mea kū hoʻokahi, 'ike loea hou o na nā mea maʻi i haawi mai ai i ka manawa kūpono i ka nalowale hihio i ka hoihoi a me ka' oi aku ka piha ola.

Dr. AD Ua kakau Romashchenko biomedicalʻenehana eiiieaena, a Palapala Sila Nui i ka keia mau ki ina hana o anei i aeea lapaau :

• hui hana o ka wehe i ka pulu ano o ka maʻi;
• eiiieaena hana paitogeneticheskogo Inc oaio a me ka tapetoretinalnoy dystrophy.

Ma kekahi? Aaieou o ka iapaau ana?

Ka iapaau ana o ka luna 'mai i Ua ophthalmologic oaio "Ma ke Kalinika". Ke kikowaena o Nyos i loko o ke kūlanakauhale e like St. Petersburg. Hana Stargardt ka maʻi hiki wale nō ia i ka konu, no ka mea, o ka mea wale nō i kekahi i loko o Rusia, kahi i 'ike loea e like me ua noi ia.

Mea anei i aeea Inc mea pakele?

hiki aku 'Oihana hōʻoia ana i ka' enehana Inc ulu AD Romashchenko, Ua loa palekana. No ka lapaau ana o ka hoʻomanawanui ka aeee i hoʻohana ', i eliminates ka manawa kūpono o ka rejection' ole o ka 'ē aʻe io hopena.

ka hopena

Shtargradta maʻi Debut ma kekahi kakahiaka nui kona mau makahiki, a koke alakai i ka loa emi o ka hihiʻo. I loa pohihihi keia hoopii, hooilina Hoʻohaku type, maka kulu lohi. Hoʻomanawanui iki mai i ka manao, e kipa i ka ophthalmologist, nā wikamina, a ua hoʻokomoʻia nā maka aniani. Ka loa ka hoʻokō ala, e hoʻopau i ka pathology noonoo Inc ma anei i keena.