He aha ka phenylketonuria, nā hōʻailona nui a pehea e hana ai ka lāʻau

Anter

• Nā hōʻailona nui
• Pehea e hana ai i ka lāʻau
• Aia kekahi lāʻau no phenylketonuria?
• Pehea e hana ʻia ai ke kuhi

ʻO Phenylketonuria kahi maʻi maʻi laha ʻole i ʻike ʻia e ke kū ʻana o kahi mutation e pili ana i ka hoʻololi ʻana i ka hana o kahi enzyme i loko o ke kino nāna e hoʻohuli i ka amino acid phenylalanine i ka tyrosine, kahi e alakaʻi ai i ka hōʻiliʻili o phenylalanine i loko o ke koko a kiʻekiʻe. ʻawaʻawa nā concentrations i ka meaola, i hiki ke kumu i ke kīnā ʻole o ka naʻau a me ka hopu ʻana, no ka laʻana.

He ʻano recessive autosomal kēia maʻi genetical, ʻo ia hoʻi, no ke keiki e hānau ʻia me kēia mutation, pono nā mākua ʻelua e lawe i ka hoʻololi. ʻO ke kuhi o phenylketonuria hiki ke hana pono ma hope o ka hānau ʻana ma o ka hoʻāʻo ʻana o ka kuʻekuʻe wāwae, a laila hiki ke hoʻokū mua i ka lāʻau.

ʻAʻohe lāʻau o Phenylketonuria, akā hana ʻia kāna mālama ʻana ma o ka meaʻai, a he mea kūpono e hōʻalo ai i ka ʻai ʻana o nā meaʻai i loko o ka phenylalanine, e like me nā cheeses a me nā ʻiʻo, no ka laʻana.

Nā hōʻailona nui

ʻO nā keiki hānau me phenylketonuria i ka mua ʻaʻohe ʻōuli, akā ʻike ʻia nā ʻōuli i kekahi mau mahina ma hope, ʻo nā mea nui:

• ʻO nā ʻeha o ka ʻili e like me ka eczema;
• ʻAla ʻoluʻolu ʻole, ʻano o ka hōʻiliʻili o phenylalanine i ke koko;
• Nusea a me ka luaʻi;
• ʻAno hōʻeuʻeu;
• Hyperactivity;
• Hoʻoiho i ka noʻonoʻo, ʻoi aku ka paʻakikī a me ka hiki ʻole ke hoʻihoʻi ʻia;
• Huikau;
• Nā pilikia pili i ka lawena a me ka pilikanaka.

Mālama pinepine ʻia kēia mau ʻōuli e ka meaʻai kūpono a haʻahaʻa i nā meaʻai kumu phenylalanine. Eia hou, he mea nui e kiaʻi mau ʻia ke kanaka me ka phenylketonuria e ka pediatrician a me ka mea hānai mai ka hānai ʻana i mea hānai ʻaʻohe pilikia koʻikoʻi a ʻaʻole hoʻohoka ʻia ka ulu ʻana o ke keiki.

Pehea e hana ai i ka lāʻau

ʻO ka pahuhopu nui o ka mālama ʻana i ka phenylketonuria e hoʻoliʻiliʻi i ka nui o ka phenylalanine i loko o ke koko a, no laila, hōʻike pinepine ʻia e ukali i ka papaʻai haʻahaʻa i nā meaʻai i loaʻa ka phenylalanine, e like me nā meaʻai o nā holoholona, ​​no ka laʻana.

He mea nui kēia mau hoʻololi i ka papaʻai e alakaʻi ʻia e ka nutristist, no ka mea he pono paha e hoʻohui i kekahi mau huaora a i ʻole nā ​​minelala i hiki ʻole ke loaʻa i ka papaʻai maʻamau. E ʻike pehea e pono ai ka meaʻai i ka hihia o phenylketonuria.

ʻO ka wahine me ka phenylketonuria e makemake ana e hāpai, pono ke alakaʻi ʻana mai ka mea hānau a me ka mea hānai e pili ana i nā makaʻu o ka hoʻonui ʻana i ka nui o ka phenylalanine i loko o ke koko. No laila, he mea nui e loiloi ʻia e ke kauka i kēlā me kēia manawa, me ka hoʻohui ʻana i ka papaʻai kūpono no ka maʻi a, malia paha, e hoʻohui ana i kekahi mau meaola no ke olakino o ka makuahine a me ke keiki.

Paipai ʻia hoʻi e kiaʻi ʻia ka pēpē me ka phenylketonuria i loko o kona ola a me ka maʻa mau e hōʻalo i nā pilikia, e like me ka hōʻino ʻia o ka ʻōnaehana, e laʻa me. E aʻo pehea e mālama ai i kāu pēpē me phenylketonuria.

Aia kekahi lāʻau no phenylketonuria?

ʻAʻohe lāʻau o Phenylketonuria a, no laila, hana wale ʻia ka lāʻau me ka kaohi i ka papaʻai. ʻO ka hōʻino a me ka hōʻino ʻia o ka ʻike e hiki ke hana ʻia me ka ʻai ʻana o nā meaʻai i loko o ka phenylalanine hiki ʻole ke hoʻololi ʻia i ka poʻe ʻaʻohe o ka enzyme a i ʻole ka enzyme kūpikipiki ʻole ʻole e pili ana i ka hoʻohuli o phenylalanine i tyrosine. Hiki ke hōʻalo maʻalahi ʻia kēlā ʻano ʻino e ka ʻai ʻana.

Pehea e hana ʻia ai ke kuhi

Hoʻomaopopo ʻia ke kuhi o phenylketonuria ma hope koke iho o ka hānau ʻana ma o ka hoʻāʻo prick heel, a pono e hana ʻia ma waena o nā hola he 48 a me 72 o ke ola o ka pēpē. Hiki i kēia hoʻāʻo ke kuhi ʻaʻole wale ka phenylketonuria i ka pēpē, akā ʻo ka anemia pūleona sickle a me ka fibrosis cystic. E ʻike he aha nā maʻi i ʻike ʻia e ka hoʻāʻo prick heel.

ʻO nā keiki i hōʻike ʻole ʻia me ka hoʻāʻo heʻe kuʻekuʻe, e loaʻa paha i ka hōʻailona e nā hoʻokolohua hoʻokolohua ka mea e nānā ai i ka nui o ka phenylalanine i loko o ke koko a, i ka hihia o ke kiʻekiʻe loa, hiki ke hana ʻia kahi hoʻokolohua e ʻike ai i ka mutation pili i ka maʻi.

Mai ka manawa i ʻike ʻia ai ka hoʻololi a me ka nui o ka phenylalanine i loko o ke koko, hiki i ke kauka ke nānā i ke kahua o ka maʻi a me ka likelike o nā pilikia. Eia kekahi, he mea nui kēia ʻike no ka mea hānai e hōʻike i ka papaʻai papaʻai kūpono loa no ke kūlana o ke kanaka.

He mea nui ka hana ʻana o nā phenylalanine i ke koko i nā manawa maʻamau. I ka hihia o nā pēpē, he mea nui e hana ʻia i kēlā me kēia pule a hiki i ka lilo ʻana o ka pēpē i 1 mau makahiki, ʻoiai no nā keiki ma waena o 2 a 6 mau makahiki e pono e hoʻokō ʻia ka hōʻike ma kēlā me kēia ʻelua pule a no nā keiki mai 7 mau makahiki, ka mahina.