He aha ka Mucopolysaccharidosis a pehea e mālama ʻia ai

Anter

• Nā ʻano o mucopolysaccharidosis
• Nā kumu kūpono
• He aha nā hōʻailona
• He aha ke kuhi
• Pehea e hana ai i ka lāʻau

Hoʻohālikelike ʻia ka Mucopolysaccharidosis e kahi hui o nā maʻi hoʻoilina i loaʻa mai i ka loaʻa ʻole o kahi enzyme, ka mea i loaʻa ka hana o ke kō ʻana i kahi kō i kapa ʻia ʻo mucopolysaccharide, i kapa ʻia ʻo glucosaminoglycan.

He laha ʻole a paʻakikī hoʻi kēia e hōʻike i ka maʻi, no ka mea, hōʻike ia i nā ʻōuli e like loa me nā maʻi ʻē aʻe, e like me ka hoʻonui ʻia o ka ake a me nā ʻāpala, nā deformities o nā iwi a me nā hono, nā haunaele a me nā pilikia hanu, no ka laʻana.

ʻAʻohe lāʻau i ka Mucopolysaccharidosis, akā hiki ke hana i kahi lāʻau e hoʻolohi ai i ka loiloi o ka maʻi a hāʻawi i kahi ola maikaʻi no ke kanaka. Pili ka mālama ʻana i ke ʻano o ka mucopolysaccharidosis a hiki ke hana ʻia me ka hoʻololi ʻana i ka enzyme, nā transplant o ka iwi iwi, ka hoʻōla kino a i ʻole ka lāʻau lapaʻau paha.

Nā ʻano o mucopolysaccharidosis

Hiki i nā mucopolysaccharidosis kekahi mau ʻano, i pili i ka enzyme i hiki ʻole i ke kino ke hana, no laila ke hōʻike nei i nā ʻōuli like ʻole no kēlā me kēia maʻi. ʻO nā ʻano ʻokoʻa o mucopolysaccharidosis:

• ʻAno 1: ʻO Hurler, Hurler-Schele a i ʻole Schele syndrome;
• ʻAno 2: Hunter syndrome;
• ʻAno 3: Sanfilippo maʻi maʻi;
• ʻAno 4: ʻO Morquio Syndrome. E aʻo hou aʻe e pili ana i ka mucopolysaccharidosis ʻano 4;
• ʻAno 6: Maʻi Maroteux-Lamy;
• ʻAno 7: ʻO Sly Syndrome.

Nā kumu kūpono

ʻO Mucopolysaccharidosis kahi maʻi hoʻoilina i hoʻoilina ʻia, ʻo ia hoʻi ke hele ia mai nā mākua a hiki i nā keiki a he maʻi recessive autosomal, koe ka ʻano II. Hoʻohālikelike ʻia kēia maʻi e ka hiki ʻole o ke kino ke hoʻohua i kekahi enzyme e hoʻohaʻahaʻa i nā mucopolysaccharides.

ʻO nā Mucopolysaccharides he mau kō lōʻihi lōʻihi, he mea nui no ke kūkulu ʻana i nā ʻano kino like ʻole, e like me ka ʻili, nā iwi, nā iwi a me nā aʻa, e hōʻiliʻili i kēia mau mea, akā pono e hoʻohou hou. No kēia, pono nā enzyme e wāwahi iā lākou, i hiki ai iā lākou ke hemo a pani ʻia me nā mucopolysaccharides hou.

Eia nō naʻe, i ka poʻe me ka mucopolysaccharidosis, ʻaʻole paha kekahi o kēia mau enzyme no ka haki o ka mucopolysaccharide, e hoʻohiolo ai ka pōʻaiapuni hou, e alakaʻi ana i ka hōʻiliʻili ʻana o kēia mau kō i nā lysosome o nā pūnaewele o ke kino, e hoʻopilikia nei i kā lākou hana a hāʻawi e ala aʻe i nā maʻi ʻē aʻe a me nā hanaʻino.

He aha nā hōʻailona

ʻO nā ʻōuli o ka mucopolysaccharidosis kaukaʻi ʻia i ke ʻano o ka maʻi i loaʻa i ke kanaka a holomua ia, ʻo ia hoʻi ke ʻino lākou i ka holo ʻana o ka maʻi. ʻO kekahi o nā hōʻailona a me nā ʻōuli ke:

• Hoʻonui i ke ake a me ka lepe;
• Hoʻololi iwi;
• Nā pilikia hui a me ka neʻe;
• Pōkole;
• Nā maʻi hanu;
• Hernil a i ʻole hernia hernia;
• Nā maʻi hanu a me ka maʻi puʻuwai;
• Nā pilikia o ka lohe a me ka ʻike ʻana;
• ʻAno moe;
• Nā Hoʻololi i ka Pūnaewele Nervous Waena;
• Hoʻonui ʻia ke poʻo.

Eia hou, ʻo ka hapanui o ka poʻe i ʻeha i kēia maʻi he ʻano like pū kekahi me ka morphology o ke alo.

He aha ke kuhi

ʻO ka maʻamau, ʻo ka hōʻoia o mucopolysaccharidosis ka loiloi o nā hōʻailona a me nā ʻōuli a me nā hoʻokolohua hoʻokolohua.

Pehea e hana ai i ka lāʻau

Pili ka mālama ʻana i ke ʻano o ka mucopolysaccharidosis i loaʻa i ke kanaka, ke kūlana o ka maʻi a me nā pilikia e ala aʻe a pono e hana koke ʻia.

Hiki paha i ke kauka ke koi aku i ka hoʻomaʻemaʻe ʻana i ka enzyme, ka hoʻoneʻe ʻana o ka iwi iwi a i ʻole nā ​​wā hoʻomaʻamaʻa kino. Eia kekahi, pono e mālama ʻia nā pilikia i hoʻokumu ʻia e ka maʻi.