He aha ka maʻi ʻo Marfan, nā ʻōuli a me nā lāʻau

Anter

• Nā hōʻailona nui
• Nā kumu o ka maʻi ʻo Marfan
• Pehea e hana ʻia ai ke kuhi
• Nā koho lapaʻau

ʻO Marfan Syndrome kahi maʻi maʻi e hoʻopili i nā mea hoʻopili, ʻo ia ke kuleana no ke kākoʻo a me ka lahilahi o nā mea like ʻole i ke kino. ʻO nā kānaka me kēia maʻi maʻi he kiʻekiʻe loa, lahilahi a lōʻihi loa nā manamana lima a me nā manamana wāwae a he hoʻololi paha i ko lākou puʻuwai, maka, iwi a me nā akemāmā.

Kū kēia maʻi ma muli o ka hemahema hoʻoilina i ka fibrillin-1 gen, ʻo ia ka mea nui o nā ligament, nā paia artery a me nā hono, e hoʻonāwaliwali ai kekahi mau ʻāpana a me nā lālā o ke kino. Hana ʻia ke kuhi e kahi loea ākea a pediatrician paha ma o ka mōʻaukala olakino o ke kanaka, nā hoʻokolohua koko a me ke kiʻi ʻana a me ka mālama ʻana i ke kākoʻo ʻana i ka hopena i hoʻokumu ʻia e ka maʻi.

Nā hōʻailona nui

ʻO Marfan Syndrome kahi maʻi maʻi e hoʻohuli i nā ʻōnaehana like ʻole o ke kino, e alakaʻi ana i nā hōʻailona a me nā ʻōuli i hiki ke ʻike ʻia i ka wā hānau a i ʻole i loko o ke ola holoʻokoʻa, ʻokoʻa ka nui o nā koʻikoʻi mai kekahi kanaka a i kekahi. Hiki ke ʻike ʻia kēia mau hōʻailona ma nā wahi aʻe:

• Puʻuwai: ʻo nā hopena nui o ka maʻi ʻo Marfan ka hoʻololi ʻana o ka puʻuwai, e alakaʻi ana i ka nalo ʻana o ke kākoʻo i ka paia o ke aʻa, kahi e hiki ai i ke aneurysm aortic, dilation ventricular a me ka mitral valve prolaps;
• Iwi: hana kēia maʻi i nā iwi e ulu nui a ʻike ʻia ma o ka hoʻonui nui ʻia o ke kiʻekiʻe o ke kanaka a me nā lima, nā manamana lima a me nā manamana wāwae he lōʻihi loa. Kāhea ʻia ka umauma hollowed-outpectus excavatum, ʻo ia ka manawa e puka ai kahi kaumaha i waenakonu o ka umauma;
• Maka: he mea maʻamau ia no ka poʻe i loaʻa i kēia maʻi ke hoʻoneʻe i ka retina, glaucoma, cataract, myopia a me ka ʻāpana keʻokeʻo loa o ka maka iʻoi aku ka polu;
• Spine: ʻike ʻia nā hiʻohiʻona o kēia maʻi i nā pilikia ʻōiwi e like me scoliosis, ʻo ia ka hoʻololi ʻana o ka iwi kuamoʻo i ka ʻaoʻao ʻākau a hema paha. Hiki nō hoʻi ke nānā i ka hoʻonui ʻana o ka ʻeke dural ma ka lumbar, ʻo ia ka membrane e uhi ana i ka ʻāʻī o ka iwi kuamoʻo.

ʻO nā ʻōuli ʻē aʻe e hiki ke ala aʻe ma muli o kēia maʻi ka looseness o nā ligament, nā deformities i ka pā, i ʻike ʻia ma ke kaupaku o ka waha, a me nā kapuaʻi pālahalaha, i hōʻike ʻia e nā kapuaʻi lōʻihi, me ka ʻole o ke kaʻe o ka wāwae. E ʻike hou aʻe he aha ka papaha a pehea e hana ai ka lāʻau.

Nā kumu o ka maʻi ʻo Marfan

Hoʻokumu ʻia ka maʻi o Marfan e kahi kīnā ma ka ʻāpana i kapa ʻia ʻo fibrillin-1 a i ʻole FBN1, i loaʻa ka hana o ka hōʻoia ʻana i ke kākoʻo a me ka hana ʻana i nā olonā elela o nā mea like ʻole i loko o ke kino, e like me nā iwi, ka puʻuwai, nā maka a me nā kuamoʻo.

I ka hapanui o nā hihia, hoʻoilina kēia hemahema, ʻo ia hoʻi ua lawe ʻia mai ka makuakāne a i ʻole ka makuahine i ke keiki a hiki ke hana ʻia i nā wahine a me nā kāne. Eia nō naʻe, i kekahi mau hihia rarer, hiki i kēia kīnā i ka moʻokūʻauhau ke loaʻa wale a no ke kumu ʻole.

Pehea e hana ʻia ai ke kuhi

ʻO ke kuhi ʻana o ka maʻi ʻo Marfan i hana ʻia e kahi loea ākea a pediatrician paha e pili ana i ka moʻolelo o ka ʻohana a me nā loli o ke kino, a me nā hoʻokolohua kiʻi, e like me echocardiography a me electrocardiogram, hiki ke kauoha ʻia e ʻike i nā pilikia i loko o ka puʻuwai, e like me ka dissection aorta. E aʻo hou aʻe e pili ana i ka dissection aortic a pehea e ʻike ai.

Hōʻike ʻia nā kukuna X, ka tomography i helu ʻia a i ʻole ke kiʻi resonance ʻume mākenēki e nānā i nā loli i nā ʻōnaehana ʻē aʻe a me nā hoʻokolohua koko, e like me nā hoʻokolohua kūlohelohe, i hiki ke ʻike i nā hoʻololi i ka ʻāpana kuleana no ka helehelena o kēia maʻi. Ma hope o ka puka ʻana o nā hopena o ka hoʻāʻo ʻana, e hāʻawi ke kauka i ka ʻōlelo aʻoaʻo kūlohelohe, kahi e hāʻawi ʻia ai nā ʻōlelo aʻoaʻo no nā genetics o ka ʻohana.

Nā koho lapaʻau

ʻAʻole mālama ʻia ka mālama ʻana i ka maʻi o Marfan i ka hoʻōla ʻana i ka maʻi, akā kōkua ia i ka hōʻemi ʻana i nā ʻōuli i mea e hoʻomaikaʻi ai i ke ola o nā kānaka me kēia maʻi maʻi a manaʻo e kōkua i ka hōʻemi ʻana i nā deformities ʻōiwi, hoʻomaikaʻi i nā neʻe o nā hono a hoʻemi i ka hiki o hoʻoneʻe.

No laila, pono nā mea maʻi me ka Marfan Syndrome e hōʻoia maʻamau i ka puʻuwai a me nā kīʻaha koko, a lawe i nā lāʻau e like me beta-blockers, e pale ai i ka hōʻino ʻana i ka ʻōnaehana cardiovascular. Hoʻohui ʻia, pono paha ka lāʻau ʻoki lāʻau e hoʻoponopono i nā leona i ke aʻa aorta, no ka laʻana.