ʻO ka maʻi o Ehlers-Danlos
Le ʻO Ehlers-Danlos maʻi he pūʻulu o nā maʻi genetic i hōʻike ʻia e a ʻinoʻino o ke kino pili, ʻo ia hoʻi, nā ʻiʻo kākoʻo.
Aia nā ʻano like ʻole o ka maʻi1, loaʻa ka hapa nui a hyperlaxity o na ami, ka ʻili elastic loa a nā kīʻaha koko palupalu. ʻAʻole pili ka maʻi i ka hiki ke noʻonoʻo.
Ua kapa ʻia ka maʻi ʻo Ehlers-Danlos ma hope o ʻelua mau kauka lāʻau lapaʻau, hoʻokahi Denemaka, ʻo Edvard Ehlers a me kekahi Palani ʻo Henri-Alexandre Danlos. Ua wehewehe lākou i ka maʻi i ka makahiki 1899 a me 1908. |
Nā kumu
ʻO ka maʻi ʻo Ehlers-Danlos kahi maʻi genetic e pili ana i ka hana ʻana o ka collagen, kahi protein e hāʻawi i ka elasticity a me ka ikaika i nā ʻiʻo pili e like me ka ʻili, tendons, ligaments, a me nā paia o nā kino a me nā ʻāpana. nā kīʻaha koko. ʻO ka hoʻololi ʻana i nā genes like ʻole (e like me ADAMTS2, COL1A1, COL1A2, COL3A1) ke kuleana no nā hōʻailona like ʻole e like me nā ʻano like ʻole o ka maʻi.
ʻO ka hapa nui o nā ʻano maʻi Ehlers-Danlos (EDS) i hoʻoili ʻia e nā kūlana autosomal dominant. No laila he 50% ka nui o ka hiki i ka makua ke lawe i ka maʻi i kēlā me kēia o kā lākou mau keiki. Hōʻike ʻia kekahi mau hihia ma muli o ka hoʻololi ʻana.
Nā pilikia
ʻO ka hapa nui o ka poʻe me ka maʻi Ehlers-Danlos e alakaʻi i nā ola maʻamau, ʻoiai he mau kapu lākou i nā hana kino. Pili nā pilikia i ke ʻano o ka ADS i pili.
- maika nā'ōpala nui.
- maika ʻeha hui pū.
- ʻAiʻi mua.
- Un kūnewa mua ma muli o ka puka ʻana o ka lā.
- Osteoporosis.
Pilikia ka poʻe me ka EDS (type IV SED) no nā pilikia koʻikoʻi, e like me ka haki ʻana o nā kīʻaha koko koʻikoʻi a i ʻole nā ʻōkana e like me ka ʻōpū a i ʻole ka ʻōpū. Hiki ke make kēia mau pilikia.
ʻO ke kuhikuhina
ʻO ka laha o nā ʻano āpau o ka maʻi Ehlers-Danlos ma ka honua holoʻokoʻa ma kahi o 1 i 5000 poʻe. ʻano hypermobile, ka mea maʻamau, ua manaʻo ʻia ma 1 i ka 10, ʻoiai ka ʻano vascular, rarer, aia ma 1 i 250 hihia. Me he mea lā, pili ka maʻi i nā wāhine a me nā kāne.