Ola kinoMaʻi a me nā kūlana

Duchenne myopathy - he maʻi o ka'emi ko kahua.

Maʻi o ka'emi'ōnaehana i mau ua noʻonoʻo iwaena o ka loa a me ka luna 'paʻakikī-i-lāʻau lapaʻau o pathological ana. O ke kumu ho i ka mea i ka'emi nenoaiu o ka loa holomua a me ka lahui differentiated 'ole o ke kino o ke kanaka, na keena o ka i kokoke ole hiki ke hoola ia ia iho. Inā he mea i ka lukuia ana o ka hookahi nā aʻalolo puluniu, keia kŰia loa irreversible. I ka hopena o kēia kaʻina hana, e 'ai pono i paʻa lilo o ooieoeiiaeuiuo nā mea hiki o ka anatomical aina, no ka mea, i ke aʻalolo olona, halawai i ka mea haule. He nui neurological kāna hana mau i ka hopena o ka aaiaoe mā i hiki ke pū i hiki mai ia hanauna aku ia hanauna aku.

A Kuhina Nui mamuli o ke pathological hoi, kena ae la ia e hereditary myopathy o Duchenne. Ia mai ua pū i hiki i ka autosomal recessive hereditary naoh a oi hoikeia mai i waena o ke kāne heluna o ko mākou Honua. Ka hope oiaio mea ku pono i ka mea i ka aina hooili ua ua hoʻopili i ka wahine chromosomes. Ma ka naau maʻi mea he'ōewe kina kuleana no ka hoʻololi kemikala nui o ka dystrophin kumuʻiʻo, a ua komo i loko o ka malama o ka anatomical 'ole o ka Muscle puluniu. Aie i ka nele o ke kumuʻiʻo dystrophin Muscle'aʻaʻa undergoes he moʻo o nā loli i ka hopena o ka a na nāʻiʻo i UAIAaIN e fibrous a me ka momoma'aʻaʻa, ma na olelo e ae, ka mea e atrophy. Aie i ka mea i ka Duchenne myopathy mea hereditary, e hoʻomaka e hele mai i kakahiaka nui meʻuʻuku mai. I nā mākua e lohe i ka mea kā lākou keiki papale hainaka maʻalahi mai ka iki physical exertion, i sluggish, koke ia ke hiki hoʻololi i ka gait a me ka ino iwi iwi.

A laila pathological kaʻina progresses wale mai ia mea malalo o ia e pāpā ikaika na nāʻiʻo a pau o ke kanaka kino. No keia pathology ua wehewehe aku ma ka myopathy maka. More nāʻiʻo i hoʻohuli ai i nā lala, torso, naau. I ka myocardial komo, aia no he haumāna cardiomyopathy, ka naau pepe pāpana. Pinepine ia mea he pathology o ka mea'ōnaehana mānowai koko'ōnaehana ua i ka papa kuhikuhiE kumu o ka make ana o nā mea maʻi me kaʻike, a Duchenne myopathy. 'ano'ē ano o ka hana maikaʻiʻole o kēia maʻi mea nō,ʻaʻole ma laila. Ka walaʻauʻana rula o ka lapaʻau - mea e hoʻoikaika 'i ka pono a me ka hoʻolōʻihi' ia 'o ke kanaka ola.

Myopathy Duchenne hiki ke compensated no ka lōʻihi manawa ma ka loaa ana corticosteroid ai '. Ma awelika, me ka i kela la i ahonui e pono ai ma kahi o 800 mg o prednisolone no kilogram o ke kino ke kaumaha. Ma ka loiloi mua kahua o ka hoʻonaʻauao, ua nui ka hoʻokō kumuʻiʻo eiiieaenu me kiʻekiʻe ka maʻiʻo o ka'amino nāʻakika, anabolic steroid i, i ka mea a pau i hiki ke hoʻoulu Muscle ulu. Ka iapaau ana Ua koi 'ia e nā L-carnitine, vasoactive ai', nā wikamina (B1, B6, B12, etc.) Biogenic stimulators (Aloe), alpha-lipoicʻakika, waiwai, mai ka pae ana o antioxidants. Ma ke koena o keia pathology hana e hanaʻia nō ia, a hoʻoikaika 'ana i ka trophism i loko o ka musculature. He He okoa physiotherapy i kūpono, physical Inc kau a me ka pololei, pākōlea lomilomi. A pau o keia kōkua e emi i ka Holomua o ka mai, a me ka hooulu ana o ka Muscle hui a me contractures, a nui kaupalena i ka Hawaii ha awina o ka hoʻomanawanui. E emi ke oolea orthopedic hana likeʻole maomeka a? Ieoaeunoaa iauaeoia. I oi loa hoopii, ka wā nō i contracture a me ka pale ole wale hele, akā, a hiki i ka noho, a moe i ka mea e holo palliativeʻoki kino.

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