Stargardt ọrịa: nkọwa, na-akpata, ihe mgbaàmà na ọgwụgwọ atụmatụ

Stargardt ọrịa bụ a degenerative usoro akpalite na macula. E nwere ọtụtụ ọrịa ọgwụ bụ nke yiri nke a daa ọrịa. Ha na-kpatara a mutation nke dị iche iche mkpụrụ ndụ ihe nketa. Ya mere, ọrịa bụ nke na udi nke butere n'aka pathologies.

Isi adakarị ngosipụta nke ọrịa a degenerative usoro na macula na etiti retinitis ink na-eme ka dobe nke mmepe nke a Central scotoma.

Atụmatụ nke ọrịa

Stargardt ọrịa bụ obere ma nnọọ oké pathologies. Ọ na-egosipụta onwe ya na nwata - si 6 afọ 20 na a ugboro ole nke 1: 20,000. Na ndị ọzọ afọ na edemede daa ọrịa, dị ka a na-achị, ọ bụghị vstrechvaetsya. The esi nke kpata ajọ ọrịa. Ọ bụghị chịrị si a zuru ezu ọnwụ nke ọhụụ.

The ọrịa nwere mkpụrụ ndụ ihe nketa ndabere. Dystrophic usoro emetụta retinal macular mpaghara na-esite na ink epithelium, nke na-eduga ná ọnwụ nke n'anya. Usoro dị bilateral.

enweghị nchịkwa ụdị

E nwere a doro anya nkewa nke daa ọrịa n'ime anọ na ụdị dabere na ebe nke mkpali orunótu:

Degenerative usoro nwere ike kwuru, sị:

• na n'etiti elu mpaghara;
• na macular region;
• na paracentral mpaghara.

Ọzọkwa, e nwere a mbuaha ụdị ọrịa nke na-agụnye mbufụt metụtara nanị akụkụ ụfọdụ na akụkụ bụ isi nke anya na mpụta.

Usoro nke ọrịa development

Na-akpata ọrịa na-kọwara dibịa Karl Stargardt ke akpa ọkara nke iri abụọ na narị afọ. Na ya nsọpụrụ na aha ya bụ ọrịa. The daa ọrịa na-metụtara macular region na, dị ka ọkà mmụta sayensị, na-agafere n'ime a nketa ezinụlọ. Abụkarị ẹkedọhọde polymorphic ophthalmoscopic picture, nke na-agba aha "atrophy nke ndị choroid," "ehi anya", "gbajiri ọla", na ndị ọzọ.

Site positional cloning ukwu site n'usoro locus matara na-akpata retinitis pigmentosa, bụ ihe kasị apụta ìhè ná photoreceptors. Na sayensị na-akpọ ABCR.

Ọrịa anya na-ketara na autosomal achị n'ụzọ. N'ihi pathologies ji orunótu nke mutated mkpụrụ ndụ ihe nketa na chromosomes 13q na 6ql4.

Mkpụrụ ndụ ihe nketa ndabere na-adịghị dabere na okike. Dị ka na-adịbeghị anya ọmụmụ nke doctor- Mkpụrụ Ndụ Ihe Nketa, na-achị ụdị nnyefe nke ọrịa, ọ na-ewe a obere bit mfe, ọ bụghị mgbe nile ime ka ìsì. Kasị cell anabata-anọgide na-arụ ọrụ. Na ndị a ọrịa, na daa ọrịa emee na di ntakiri mgbaàmà. Emetụta na-arụ ọrụ na-ekwe omume nke na-akwọ ụgbọala ugbo ala.

Olee otú Stargardt ọrịa? Na-akpata macular mbelata mkpụrụ ndụ na-edina na a enweghị ike. Gene nkwarụ akpalite mmepụta nke a gahiere agahie protein nke Nyefe ATP ụmụ irighiri site cell akpụkpọ ahụ macular akụkụ bụ isi nke retina. Ọ lekwasịrị anya image ndịna na agba.

symptomatology daa ọrịa

Gịnị bụ ndị e ji mara ndị na-Stargardt ọrịa? Na mmepe nke ọrịa daa ọrịa na-adịghị hụrụ ndị ọzọ esịtidem akụkụ. The usoro na-emetụta naanị na anya. The ọrịa amalite na a na ọnwụ nke visual acuity. Mgbe e mesịrị ihe mgbaàmà na-amụba, na e nwere oké njọ imebi nke uzọ nke agba nile aghọta echiche nke spektral nso.

Degenerative mgbanwe nke fundus na-egosi na ndị na-esonụ ọnụ ọgụgụ:

• ma anya-akara pigmented okirikiri ntụpọ udi;
• e nwere obodo ebe ndebe nke pigmenti;
• kwuru, atrophic usoro ke retinal ink epithelium.

Ndị dị otú ahụ Filiks nwere ike ime na njikọ na omume nke whitish-acha odo odo tụrụ. Dị ka ọrịa na-aga n'ihu na retinal ink epithelium kpughere ubara ìgwè lipofuscin-dị ka umi.

All ọrịa emetụta a daa ọrịa, ahụrụ zuru ma ọ bụ ele mmadụ anya n'ihu Central scotoma, nke nwere dị iche iche ụkpụrụ, n'ihi na njupụta nke usoro.

Mgbe zheltopyatnistoy ekara igbe nke a mmadụ ndidi pụrụ ịnọgide na-na nkịtị nso, ma Central ọhụụ na-ịrịba belata.

Ihe ka ọtụtụ nke ikpe nke ndị agbanweela nnyocha e mere, e kwuru na a mebiri nke nghọta nke na agba deuteranopia ụdị, red-green diskhromazii. Mgbe zheltopyatnistom dystrophic usoro nke na agba ọhụụ nwere ike ịbụ kpamkpam unaffected.

Dystrophy Stargardt dabara a mgbanwe ke gbasara ohere ugboro iche uche (na usoro nkebi 6 - 10 º na ịrịba Mbelata nọ n'ógbè n'etiti gbasara ohere frequencies na zuru enweghị nke elu ugboro region). The syndrome a na-akpọ "ụkpụrụ nke cone dystrophy."

Mgbaàmà-agụnye wavy ọhụụ nke ihe, ndị ìsì tụrụ, blurriness, na agba na mebiri nke mmegharị isi ike na ọnụnọ nke inyoghi inyoghi ìhè.

ọrịa nchoputa

N'ihi nke nnyocha na mkpụrụ ndụ ihe nketa larịị na-kpughere na-egosi na ọrịa anya Stargardt kpebisiri allelic ọrịa locus ABCR.

Diagnosis nke ọrịa na-amalite na a na-amụ ala nke fundus. Rụrụ fluorescein angiography nke retinal vaskụla na electrophysiological nyochaa. Na nke a, mkpebi siri ike nke na ogo nke mmebi a chọrọ. Nke a na-eme ka o kwe omume na a mata a nọmba nke ọrịa na-egosi, gụnyere giperflyuorestsiruyuschie akpa na fluorescence nke optic akwara.

Ebe ọ bụ na ọrịa bụ butere n'aka agwa, nke kasị ezi ụzọ na-ekpebi a nchoputa ga na-na DNA, nke bụ ike ime ka a mata butere n'aka ọrịa monomonogennye.

esi nchoputa

Stargardt ọrịa kwesịrị a nkewa nke cone-mkpanaka-na, nke cone, na wand-of cone dystrophy, aga n'ihu foveal dystrophy, akara ererimbot, Juvenile Retinoschisis, macular dystrophy vitelliformnoy.

Ọzọkwa, esi nchoputa gosiri na ikpe nke na ime toxemia enwetara ruru onunu ogwu mbelata (akpan akpan, mgbe hlorokvinovoy retinopathy).

Nwere ike ọ ga gwọrọ Stargardt ọrịa?

Etiologic ọgwụ adịghị adị. Ọ eliminates Stargardt ọrịa? inyeaka agwa Ọgwụgwọ-agụnye iji parabulbar ntụtụ taurine na antioxidants iwebata pụtara inyịme agbanye (pentoxifylline, nicotinic acid), na Steroid.

Untreated vitamin na-akwalite arịa mgbidi ma melite ọbara ekesa. vitamin ejikarị B, A, C, E.

Ọ na-egosi na-ejide physiotherapy. N'etiti ha ka anyị ga-egosi electrophoresis iji ọgwụ ọjọọ eme ihe, na retinal mmalite site laser.

Applied revasulyarizatsiya retina site transplantation na macular ebe muscle emep uta eri.

Na mbụ ophthalmologists gburugburu ụwa kweere na ọrịa abụghị ngwọta. The n'elu usoro ọgwụgwọ enyere ubé oge na progression nke ọrịa, ma ọ bụrụ na-adịghị egbochi ya.

Dị ka a na-achị, na ọgwụgwọ na-anapụta ndị na-zuru ezu ọnwụ nke ọhụụ. Chọrọ ịzụlite a ụkpụrụ nke ga-ikwado mweghachi nke mebiri emebi ocular owuwu.

Otutu Usoro nke ọgwụ

Ọ bụ n'ebe ahụ ugbu a na-irè usoro maka mwepụ nke ndị dị otú ahụ daa ọrịa dị ka Stargardt ọrịa? Olee otú na-emeso a ọrịa? The usoro e obụp mi ke 2009 site na Dr. Aleksandrom Dmitrievichem Romaschenko, onye bụ isi nke Ophthalmology Center "ON Clinic" (Saint-Petersburg, ul. Marata, ụlọ 69, ewu B). Ha mma ọrịa ọgwụgwọ nhọrọ dabeere na cellular anụ ahụ ọgwụgwọ.

Ndabere nke omume bụ ngwa nke sel nke adipose anụ ahụ ọrịa onye. The ọgwụgwọ e mepụtara usoro ndị ọkà mmụta sayensị na mbụ VP Filatov. N'ihi nkà na ụzụ ọhụrụ nke ọrịa na-enye ha ohere iji weghachi furu efu ọhụụ na-enye a zuru ndụ.

Dr. AD e debara aha Romashchenko mgbagwoju Biomedical technology na patented ndị na-esonụ ụzọ nke azuokokoosisi cell ọgwụgwọ :

• jikọtara usoro nke wepụ mmiri n'ụdị nke ọrịa;
• mgbagwoju usoro paitogeneticheskogo ọgwụ center na tapetoretinalnoy dystrophy.

N'otu ụlọ ọgwụ dị na ọgwụgwọ?

The ọgwụgwọ nke mgbagwoju ọrịa kemgbe ophthalmologic center "On Clinic". The center na-emi odude ke a obodo dị ka St. Petersburg. Emeso Stargardt ọrịa nwere ike na-na-na center, ebe ọ bụ na ọ bụ nanị otu onye n'ime Russia, ebe ndị dị otú technology na-etinyere.

Bụ azuokokoosisi cell ọgwụ bụ nchebe?

Professionals pụrụ iji obi ike na-egosi na nkà na ụzụ ọgwụ mepụtara AD Romashchenko, bụ nnọọ mma. Maka ọgwụgwọ nke ọrịa mkpụrụ ndụ na-eji, nke eliminates ekwe omume nke ojuju ma ọ bụ nke ndị ọzọ na-ezighị ezi pụta.

ọgwụgwụ

Shtargradta ọrịa mpụta mbụ na nwata na ngwa ngwa iduga onye zuru ọnwụ nke ọhụụ. Na nnọọ obere mgbe, ihe nketa kasị ụdị, anya tụlee nwayọọ nwayọọ. Ọrịa na-gwara ileta otu ophthalmologist, vitamin na yi ugogbe anya maka anwụ. Kasị dị irè ụzọ iji kpochapụ daa ọrịa na-ewere ọgwụ site sel.