Duchenne muscular dystrophy a me Becker: lapaʻau

Aaiaoe maʻi no a hiki i ka pae ana o kaumaha pathologies ka iapaau ana o ka i keia mea pilikia. I waena o kēia mau chromosomal abnormalities i loaʻa likeʻole aʻe '. He nui no ia i neurologic symptoms. Ano he kumu hoʻohālike i Duchenne muscular dystrophy, Becker. Mau maʻi hoʻomōhala i ka wā kamaliʻi, a i ka holumua papa. I loko nō o ka hana 'ia he o neuroscience, ua pathologies i pilikia i ka pono. Kēia mea ma muli o ke chromosomal loli i i waiho i ke kahua o ke kino.

Description o Duchenne muscular dystrophy

Duchenne muscular dystrophy - he aaiaoe maʻi wehewehe aku e holumua ai Muscle kāna hana pa alima. Ke pathology mea pohihihi keia. Ke prevalence o ka anomaly mea o ka hola 3 kanaka no 10 000 kāne. Maʻi i aneane na hihia a pau, e loli ai keikikane. Aka hoi, i ka ulu ana o Duchenne muscular kaikamāhine ka mea, i hoʻokoe '. Kēia pathology hoike mai ai ia ia iho i loko o kakahiaka kamaliʻi.

Other maʻi i i ka ia kumu, a symptoms mea myodystrophy Becker. He ua he oi maikaʻi papa. Muscle'aʻaʻa poho ia lŘlŘ nui hope - ma ka adolescence. Ma keia hihia, symptoms hoʻomōhala ke kiʻekiʻe o, a me ka hoʻomanawanui i hoopaa mai ai i ka hiki i ka hana i loko o kekahi mau makahiki. E like me Duchenne muscular dystrophy, keia pathology mea a pau mawaena o ke kane heluna. Ka mea hiki wale mai ka 1 kanaka no ka 20 tausani keikikane.

Duchenne muscular dystrophy: neuroimmunology maʻi

Ke kumu no nā pathologies waiho ma ka 'aʻe ana i ka X chromosome. Aaiaoe loli i hana ma Duchenne a Becker muscular dystrophy, aʻo i loko o ka 30-ies o ka hope kenekulia. Aka etiological Inc iʻaʻole naʻe, ua loaʻa. anomalies recessive pololei o ka'āina hoʻoili. Kēia 'o ia hoʻi ia ina ka'āno'ē'ōewe ka makana i loko o kekahi o na makua, i ka pahiki o ka maʻi keiki hanau o 25%. Ka X chromosome o ka loihi i loko o ke kino. Me nā 'ano dystrophies nahae ia lŘlŘ i loko o kekahi, a me ka ia locus (p21). Keia poho hiki aku ai i ka emi ma ka kumuʻiʻo hoʻololi kemikala, i mea he hapa o ka halepaahao membranes o Muscle'aʻaʻa. Duchenne muscular dystrophy ia ia i ka pau kahi e. No laila, 'aʻe' ana nui mamua. Becker muscular dystrophy ka wā i kumuʻiʻo ua synthesized ma ka liʻiliʻi dala a mea pathological.

Ka lapaʻau kiʻi o muscular dystrophy

Duchenne muscular dystrophy ua wehewehe aku ma lesions o ka neuromuscular nenoaiu. Ka maʻi i ke mahuʻi mua ma ka makahiki o ka 2-3 makahiki. Iloko o keia manawa, ua lilo ka nohoaliʻi i ke keiki lags ma ke kua o ka physical ulu o ko lākou mau na¯, heleʻino, holo a me Lele. No laila, na keiki paakiki, e pii i ka anuu, oia pinepine haule iho. I ka make ana o na nāʻiʻo hoʻomaka me ka laloʻaoʻao. Ma hope, e uhi i ka proximal nāʻiʻo. Degeneration ia lŘlŘ i loko o ke keena poʻohiwi kaei, quadriceps. Ma kēia wahi he mea thinning o na nāʻiʻo. Over na makahiki, myodystrophy progresses. I ka make ana o na nāʻiʻo a me ka ikaika haawe ana ma luna o ia alakai i contractures - paakiki ei lala. Eia hou, na nā mea maʻi me Duchenne muscular dystrophy malama naau maʻi, i kekahi manawa, i iho ikeia. Hoi kekahi poe kanaka no keia maʻi ua wehewehe aku ma ka emi o ka 'imi naʻauao nā mea hiki (ʻaʻole loa'ōlelo).

Becker muscular dystrophy i ka ia symptoms, akā, hoʻomōhala hope. Ka mua hoike ana i loko o 10-15 makahiki. Aia He He gradual loli ma gait naueue wale no hoi i Konakele. Hope hoʻomōhala contracture. Aʻe 'ana o ka mea'ōnaehana mānowai koko'ōnaehana i mehana. Manao ma keia maʻi ua IeAUPIIe, IAa IO i hoemiia.

Pehea e diagnose muscular dystrophy?

ʻike, "Duchenne muscular dystrophy" (a Becker) hiki i ka hoao ana i ka neurologist. Ma ka wahi mua ka mea, ua nānā 'ana ma luna o ka lapaʻau kiʻi o kēia mau maʻi. Noonoo ana ua hōʻailonaʻia i symptoms e like me thinning o ka proximal nāʻiʻo, i ka gastrocnemius Muscle hypertrophy wahahee (ia lŘlŘ ma muli o ka fibrosis a me ka momona hoʻokomo kālā). Mau symptoms i kokoke mau hui me ka mea'ōnaehana mānowai koko pathologies. Ke ECG hiki ke ike dysrhythmia, hema ventricular hypertrophy.

Nō hoʻi, nā mea maʻi me Duchenne muscular dystrophy iki lag ma hope o kā lākou mau na¯ ma ka 'imi naʻauao' ana. No ka hoʻoholo 'ana i kēia, ke keiki hana me ka psychologist. A i ka maʻi ua kēia lawe myography (ka wehewehena o ka uilaʻia Muscle) a me EhoKS - ninaninau ana o ka naau keʻena. I mea e pololei hooholo i ke alo o ka maʻi, aaiaoeʻike, ua hana. I ka wā e e malama i kekahi mau poe akamai i Duchenne a me Becker muscular dystrophy nā mea maʻi. Iwaena o lakou - he neurologist, he psychologist a me ka cardiologist.

lapaʻau

Eia naʻe, i ka etiological lapaʻau o Duchenne muscular dystrophy a me Becker ole ua ulu. Eia naʻe, e hoomanawanui i hōʻike symptomatic a me ka hope Inc. I ka kuʻina kakahiaka nui o ka maʻi i lawe mai nā papa o ka physiotherapy, lomilomi. Me ka nui kīnā o ke kino e pono ai e hana OE neʻe o na lala. E 'ūlōlohi i ka Holomua o ka palena manawa contractures, ma laila i ka hoʻopunipuni nā wāwae i ka hiamoe. Malama Inc ke hooloihi i nā ola o nā mea maʻi, a emi symptoms. E hoʻohana i kalipuna pakuʻi ai, laau "galantamine" a me ka "Neostigmine". Ma kekahi mau hihia, i hoakaka ia hormonal konohiki,ʻano nui "Prednisolone". I ka wā o ka holumua ai kāna hana o ka naau koho i cardioprotectors.

Duchenne muscular dystrophy a me Becker Forecast

Duchenne muscular dystrophy prognosis Ua noho lakou malaila. Ke kakahiaka nui kūpono 'ana o symptoms a me ka kokololio Holomua o ka maʻi hiki aku ai i kīnā i loko o kamaliʻi. Nā mea maʻi i kēia kāna hana pono a eia i malama. Ka awelika ola expectancy o nā mea maʻi nei ma kahi o 20 makahiki. Becker muscular dystrophy ua wehewehe aku ma ke ano papa. Ma lalo o ia a eia nā limahana o ke kauka a me ka manaʻo o ko lākou mau olelo operability o nā mea maʻi me ka la i ka malamaia mai i 30-35 makahiki.