Kia ako tātou mō te Leber Hereditary Optic Neuropathy (LHON) i roto i ngā kupu māmā noa iho!

Kua heke haere tō tirohanga, ā, kua tīmata te pōuriuri o ngā mea katoa? Tērā pea i tīmata i te kanohi kotahi, ā, i muri i ētahi marama, kua pāngia anō hoki te kanohi kē atu e tēnei mate? Me tino āwangawanga koe mō ēnei mea. I tēnei rā ka kōrero tātou mō tētahi mate onge ka puta ai ēnei tohu, engari e kiia ana ko te Leber Hereditary Optic Neuropathy, ko te ``(LHON)`` rānei.

He aha te mate pukupuku whatu tuku iho o Leber (LHON)?

Ki te whakarapopoto, ko te Leber hereditary optic neuropathy, arā, ko te LHON, he mate ira e tukuna iho ana i ia whakatipuranga. Ko te mea nui ka tupu ko te ngaro o tō tirohanga. Ina riro mai i te nuinga o te tangata tēnei mate, ka tīmata te pōuriuri o ō rātou tirohanga, kātahi ka kino haere i roto i te ono marama pea. I ētahi wā, ka tīmata i te kanohi kotahi, kātahi ka pā ki te kanohi kē atu i muri i ētahi marama. I te nuinga o te wā ka tīmata i te mutunga o te tamarikitanga, i te pakeketanga rānei. Engari, he maha ngā tāngata whai LHON ka taea te matapo ture. Ko te tikanga tēnei ka iti haere ō rātou tirohanga ki te wāhi e kiia ai rātou he matapo ture.

E kiia ana hoki ko te mate a Leber. Nā te mea ko te tākuta tuatahi i ako i tēnei mate ko Tākuta Theodore Leber. Ko te tikanga o te kupu "hereditary" he mea tuku iho ki a koe . Ko te tikanga o te "optic neuropathy" he mate e pā ana ki tō io whatu . He rite tō io whatu ki te taura e haere ana mai i te kāmera ki tō roro. Ko ngā mea e kitea ana e ō kanohi, arā, ko ngā tohu tirohanga, ka haria ki tō roro e tēnei io whatu. Koinei te wāhi e "kite" ai te roro. Nō reira, ki te pakaru tēnei io whatu, koinei tētahi o ngā huarahi matua ka ngaro tō tirohanga.

He rerekē ngā momo LHON?

Āe, mēnā ko te LHON te tino pānga ki ō io whatu, ko te tikanga ko te ngaronga o te tirohanga anake te tohu, ka kiia ko te mate LHON noa. Koinei te mate e pāngia ana e te nuinga o te hunga e pāngia ana e te mate Leber.

Heoi, he tino onge te kitea o ētahi atu tohumate i te taha o te LHON. Ka pā pea ēnei ki ētahi atu wāhanga o tō rātou pūnaha io, tae atu ki tō rātou ngākau i ētahi wā. Ka karangatia tēnei mate e ngā tākuta ko "Leber plus." He uaua ake tēnei, nā te mea ka pā mai ētahi atu raruraru hauora, ehara i te mea ko ngā raruraru tirohanga anake.

He pēhea te noa o te LHON?

Kāore i te tino mōhiotia te whānui o te LHON. Heoi, e ai ki ngā whakatau tata, kotahi i roto i te 25,000 ki te 50,000 tāngata ka pāngia e te mate. He mea nui hoki kia mōhiotia ko te 80% ki te 90% o te hunga whai LHON he tāne .

He aha ngā tohu o te LHON (mate a Leber)?

Ka puta te ngaronga o te tirohanga mā te `(LHON)` , me te kore mamae, ā, mā te haere mārire (subacute)., ko te tikanga ka puta mārire i roto i ētahi marama. Ko ngā huringa tuatahi ka kitea pea e koe kei roto i tō tirohanga matua . Ko te tirohanga matua ko te mea ka kitea e koe ina titiro tika koe ki mua - ko te tirohanga e whakamahia ana e koe hei taraiwa, hei pānui pukapuka, hei mōhio ki ngā kanohi. Tērā pea ka tīmata te ahua pōuri o tō tirohanga matua, ka puta rānei he ira pango i waenganui o tō tirohanga, pērā i te ira matapo. Tērā pea ka tīmata tēnei i te kanohi kotahi, kātahi ka horapa ki te kanohi kē.

Ka kino haere tēnei āhua i roto i ngā marama torutoru e whai ake nei. Tērā pea ka tīmata koe ki te ngaro i tō tirohanga tae - arā, kāore pea koe e kite i ētahi tae, kāore rānei e taea te wehewehe i a rātou. Ko te ngaronga o te tirohanga ka pumau i roto i ngā marama e ono ki te tau i muri i te tīmatanga o ngā tohu tuatahi. I tēnei wā, ko te koi o te tirohanga a te nuinga o te tangata he 20/200, he kino atu rānei, koinei te taumata o te matapo ture. Tērā pea kei a koe tonu te kite mārama, engari me ako koe ki te noho me te tirohanga iti. Whakaarohia, me te mea kei te pōuriuri te ao.

He aha ngā tohu o te "Leber's plus"?

E ai ki tā mātou i kōrero ai i mua ake nei, tera pea ka pāngia te hunga whai "Leber's plus" e ētahi atu tohu, tae atu ki te ngaronga o te tirohanga. Ko ētahi o ēnei ko:

• Ngā mate nekehanga , hei tauira te wiri.
• Ngā raruraru taurite me te taunga (ataxia) . Pērā i te tūpekepeke i te wā e hikoi ana, te kore rānei e taea te hopu tika i ngā mea.
• Ngā raruraru o te nekehanga o te ngakau , hei tauira, te koretake o te ngakau (arrhythmias).
• Ngā tohu o te sclerosis maha (MS) , pērā i te ngoikore o te uaua me te ngenge nui.

He aha ngā pūtake o te mate io tuku iho o Leber?

Ko te mate pūkahukahu optic tuku iho a Leber he mate mitochondria . He mate ira ka riro mai i a koe mā roto i ngā mitochondria i roto i ō pūtau. Inaianei kei te whakaaro pea koe he aha ngā mitochondria. I te kī poto, he rite ngā mitochondria ki ngā kaihanga hiko iti i roto i ō pūtau. Ka tangohia e rātou te hāora me ngā matūkai, ka whakaputa i te pūngao e hiahiatia ana e ō pūtau hei mahi. He rite ki te kaihanga hiko e whakakaha ana i tō tātou whare.

Nō reira, i roto i ngā mate mitochondrial, ka whakararuraruhia tēnei tukanga whakaputa pūngao. Kātahi ka kore e whiwhi ngā pūtau i te pūngao e hiahiatia ana e rātou. Nā tēnei, ka mutu pea te mahi tika a ētahi pūtau, ka mate rānei.

Ina kore e mahi tika ō mitochondria, ko ngā wāhanga o tō tinana e tino whakawhirinaki ana ki te pūngao mitochondria ko rātou te tuatahi ka pāngia e ngā pānga. Ko te mea nui o ēnei ko ngā wāhanga o ō kanohi, inā koa ko tō io whatu.. Mena he nui ō mitochondria hapa, kāore ēnei wāhanga e whiwhi i te pūngao e hiahiatia ana hei mahi tika. Koinei te mea ka tupu i roto i te mate a Leber. Ko te mutunga mai ka heke haere tō io whatu (`whakaheke`) . Koinei te āhua o te ngaro o tō tirohanga me te `(LHON)`.

He pēhea te tuku iho o te LHON?

Ko te LHON e puta mai ana i te whakarerekētanga ira i roto i te DNA i roto i ō mitochondria. Ina koa, ko ngā whakarerekētanga ira o ngā ira MT-ND1, MT-ND4, MT-ND4L, MT-ND6 rānei ka puta ai te LHON.

He mea nui, ko ō mitochondria katoa ka riro mai i tō whaea, ehara i tō pāpā. Nō reira, ko ngā whaea anake ka taea te tuku i tēnei whakarerekētanga ira ki ā rātou tamariki. Ahakoa he mate tō te pāpā, e kore ia e tuku i tēnei whakarerekētanga ira ki āna tamariki.

Heoi, ehara i te mea ko te hunga katoa e mau ana i tēnei whakarerekētanga ira ka pāngia e ngā tohu o te `(LHON)`. Nō reira, tera pea ētahi tāngata kāore e mōhio kei a rātou tēnei whakarerekētanga ira. He ahua uaua tēnei, kāore? Ko te tikanga ahakoa kei te whaea tēnei whakarerekētanga ira, tera pea ka pāngia te tamaiti e ngā tohu, kāore rānei.

He āhuatanga mōrearea kei reira hei whakawhanake i te LHON?

He pātai tino nui anō hoki tēnei. Kāore anō kia mārama te whakaaro o ngā kairangahau he aha ētahi tāngata whai whakarerekētanga ira ka puta ai ngā tohu o te `(LHON)`, ko ētahi kāore.

Heoi anō, e kī ana ētahi taunakitanga ka whai wāhi pea te ahotea ā-tinana me ngā paitini taiao ki te tīmatanga o ngā tohu. Ko te tikanga o tēnei ka tāpirihia e ēnei āhuatanga o waho he ahotea tāpiri ki ngā pūnaha kua pāngia kētia e te whakarerekētanga ira. I roto i te wā, ko te whakaaro ka piki haere ēnei ahotea, ā, ka mutu, ka puta ngā tohu.

Anei ētahi mea ka taea te waiho hei take mōrearea:

• Te momi hikareti.
• Te whakamahinga o te waipiro.
• Te pānga ki ngā paitini taiao (hei tauira, ētahi patu pepeke, ngā matū ahumahi).
• Kei a koe ētahi atu mate pūnaha.
• Te ahotea hinengaro nui .

Me pēhea te tātaritanga o te LHON?

Ka whakahaerehia e tō tohunga tiaki kanohi he raupapa o ngā whakamātautau kanohi paerewa hei tirotiro i tō tirohanga me te kimi i te pūtake o te raruraru. Tērā pea kāore e kitea he hapa o tō kanohi, o tō io whatu rānei i ngā wāhanga tōmua, arā, i roto i ngā marama tuatahi e ono i muri i te tīmatanga o ngā tohu. Ka kitea nuitia te kino i muri i ngā marama tuatahi e ono i muri i te tīmatanga o ngā tohu.

Mena kei te whakapae tō tākuta kei a koe te LHON, ka tūtohu ia i te whakamātautau ira . Koinei anake te huarahi hei mōhio māu mēnā kei a koe tētahi o ngā whakarerekētanga ira i kōrerohia e tātou. Ko tēnei whakamātautau anake te huarahi hei whakaū i te mate.

He rongoā, he rongoā katoa rānei mō te LHON?

Engari, kāore anō kia kitea he rongoā mō te LHON i tēnei wā. Ko te rongoā anake kua whakaaetia e te FDA i tēnei wā ko te momo hangai o te Coenzyme Q10 e kiia nei ko Idebenone. Kua whakaatuhia e ngā whakamātautau matapōkeretia he whakapainga ake i te tirohanga o te hunga whai LHON. Kei roto hoki i te wāhanga rangahau ētahi atu rongoā rite.

Kei te rangahau hoki ngā kairangahau i te rongoā ira , ka taea te whakamahi hei rongoā ā muri ake nei mō te LHON. Engari kei roto tonu i te wāhanga rangahau ēnei. Nō reira, i tēnei wā, he uaua ki te tumanako ka ora katoa. Heoi anō, kei reira ngā huarahi hei whakahaere i ngā tohu me te whakahaere i te hekenga o te tirohanga ki tētahi taumata.

He aha te tirohanga mō LHON?

Ina pā mai te ngaronga o te tirohanga nā te LHON, ka tere, ka kino, ā, he maha ngā wā ka pumau . He maha ngā tāngata me ako ki te noho me te tirohanga iti. Ko te tirohanga iti he taumata ngoikore ki te kino o te tirohanga, engari ehara i te mea he matapo katoa. Ko te tirohanga iti ngawari he whakamātautau koi tirohanga o te 20/70. Ko te tirohanga iti kino he whakamātautau koi tirohanga o te 20/200, iti iho rānei. He whānuitanga whānui tēnei o te koi tirohanga ka taea e koe te whakatutuki i te mutunga.

Ahakoa kei te taha ngawari, kei te taha kino rānei koe o tēnei awhe , he mea nui tonu te waimarie. Ka taea e te maimoatanga te hanga rerekētanga, engari ko te whakarerekētanga ira kei a koe te mea nui rawa atu. Ka hoki anō te tirohanga o ētahi tāngata whai LHON i muri i te tau kotahi o te ngaronga tirohanga. He rerekē te tūponotanga o tēnei momo whakaora tirohanga i runga i ngā whakarerekētanga ira rerekē. Heoi, ahakoa ka hoki anō te tirohanga, tera pea me noho tonu koe me te tirohanga iti.

He ara hei ārai i te LHON?

Ahakoa ko te nuinga o te hunga e whiwhi ana i ngā ira e pā ana ki te LHON kāore e pāngia e te ngaronga o te tirohanga, kāore anō kia mōhiotia he huarahi hei ārai i tēnei. Mā te tango i ngā antioxidants me te karo i ngā neurotoxins pēnei i te waipiro me te tupeka ka whakaiti i tō tupono, engari kāore anō kia whakamātauhia tēnei.

Kia maumahara, ko ngā wāhine katoa kei a rātou ēnei ira ka tuku iho i ēnei ki ā rātou tamariki. Ka taea e te tohutohu ira te āwhina i a koe ki te whakaaro ki tēnei mōrearea. Mena kei roto i tō whānau tēnei mate, ki te kitea rānei e koe kei a koe tēnei whakarerekētanga ira, he mea tino nui kia whiwhi tohutohu ira i mua i te tīmatanga o tētahi whānau.

Ahakoa e mōhio ana koe ki tētahi hītori whānau o LHON, he mea ohorere rānei māu, ka taea e te ngaronga ohorere o tō tirohanga te waiho hei wheako whakamataku, whakapouri hoki. Tērā pea kāore kōrua ko tō tākuta e mārama ki te mea e tupu ana i te tuatahi. Engari ina mōhio koe ki tēnei mea, he maha ngā mea hei ako māu i a koe e urutau ana ki tō ao hou.

Kia maumahara, ehara i te mea ko koe anake i tēnei haerenga - he ao whānui ngā rauemi hei āwhina i a koe. He whakahaere, he taputapu, me te tautoko hauora hinengaro e wātea ana ki te hunga he iti te tirohanga.

Hei whakamutunga, ngā mea hei maumahara

Āe, ko tāku tumanako kei a koe ētahi whakaaro mō te `(LHON)` i kōrerohia e tātou. He kaupapa uaua tēnei, engari he mea nui kia mōhio koe ki tēnei.

• (LHON) he mate tuku iho e kino ai te io whatu, ā, ka ngaro ai te tirohanga.
• Nā te whakarerekētanga ira o te DNA mitochondrial tēnei i puta ai, ka tukuna iho mai i te whaea ki te tamaiti .
• I te nuinga o te wā ka puta mai ngā tohu i te tamarikitanga, ā, ka heke haere te tirohanga, kāore he mamae.
• Haunga te tirohanga, ka pā mai pea ētahi atu raruraru pūnaha io i roto i te āhuatanga e kiia nei ko "Leber plus" .
• Ko ngā mea pēnei i te momi hikareti me te waipiro ka noho hei take mō te whanaketanga o ngā tohu.
• Kāore anō kia kitea he rongoā motuhake i tēnei wā, engari kei reira ngā rongoā pēnei i te 'Idebenone' me ngā huarahi hei urutau ki te tirohanga pōuri.
• Mena he ruarua āu mō tēnei, me toro atu ki tētahi tākuta kanohi, me te whakamātautau ira.
• He uaua pea te noho me tēnei āhuatanga, engari kāore koe i te mokemoke, rapua he āwhina.

Ko tāku tumanako he whai hua ēnei mōhiohio ki a koe. Mēnā he pātai anō āu mō tēnei, kaua e mangere ki te kōrero ki te tākuta. Kia ora tonu koe!

` Neuropathie whatu tuku iho o Leber, LHON, ngaronga tirohanga, io whatu, whakarerekētanga ira, mate mitochondrial, matapo tuku iho