He aha ka maʻi Zellweger a pehea e mālama ai

Anter

• Nā hiʻohiʻona Syndrome
• He aha ke kumu o ka maʻi maʻi
• Pehea e hana ai i ka lāʻau

ʻO Zellweger Syndrome kahi maʻi maʻi laha ʻole e hiki ai ke hoʻololi i ka iwi iwi a me ke alo, a he pōʻino nui hoʻi i nā mea nui e like me ka puʻuwai, ka ate a me nā puʻupaʻa. Eia kekahi, ʻaʻohe o ka ikaika, paʻakikī i ka lohe a me ka hopu ʻana he mea maʻamau.

Hōʻike pinepine nā pēpē me kēia maʻi i nā hōʻailona a me nā ʻōuli i nā hola mua a i ʻole nā ​​lā ma hope o ka hānau ʻana, no laila e noi paha kahi pediatrician e hana i nā hoʻokolohua koko a me ka mimi e hōʻoia i ka ʻike.

ʻOiai ʻaʻohe lāʻau lapaʻau no kēia maʻi, kōkua ka lapaʻau e hoʻoponopono i kekahi o nā loli, hoʻonui i nā manawa o ke ola a ʻae i nā hoʻomaikaʻi i ka maikaʻi o ke ola. Eia nō naʻe, kaukaʻi ʻia i ke ʻano o nā loli organ, he awelika ke ola o kekahi mau pēpē ma lalo o 6 mau mahina.

Nā hiʻohiʻona Syndrome

ʻO nā ʻano kino nui o Zellweger Syndrome me:

• Ke alo pālahalaha;
• Ka ihu ākea a pālahalaha;
• Lae nui;
• ʻO ka waha o Warhead;
• Huli nā maka i luna;
• Nui a poʻo liʻiliʻi paha ke poʻo;
• Hoʻokaʻawale ʻia nā iwi iwi poʻo;
• ʻ largerlelo nui ma mua o ka maʻamau;
• Pelu ʻili i ka ʻāʻī.

Hoʻohui ʻia, hiki i kekahi mau hoʻololi ke kū i nā mea nui e like me ke ake, nā puʻupaʻa, ka lolo a me ka puʻuwai, kahi e pili ana i ka nui o nā malformations, hiki ke hoʻoweliweli i ke ola.

He mea maʻa mau ia i nā lā mua o ke ola, ʻaʻohe ikaika o ka pēpē i nā mākala, ka paʻakikī i ka hānai ʻana i ka waiū, ka haʻalulu a me ka paʻakikī o ka lohe a me ka ʻike ʻana.

He aha ke kumu o ka maʻi maʻi

Hoʻokumu ʻia ka maʻi e kahi autosomal recessive genetic hoʻololi i nā gene PEX, ʻo ia hoʻi inā aia nā hihia o ka maʻi i nā ʻohana makua ʻelua, ʻoiai inā ʻaʻohe maʻi nā mākua, aia ma kahi o 25% ka manawa o ka loaʻa he keiki me ka maʻi o Zellweger.

Pehea e hana ai i ka lāʻau

ʻAʻohe ʻano kikoʻī o ka mālama ʻana no ka maʻi Zellweger, a i kēlā me kēia hihia, pono ka pediatrician e loiloi i nā loli i hana ʻia e ka maʻi i loko o ka pēpē a paipai i ka lāʻau maikaʻi loa. Aia kekahi o nā koho i:

• Paʻa hānai: ke kau pono ʻana i kahi ʻōmole a hiki i ka ʻōpū e ʻae ai i ka ʻai e komo;
• Nā loli i ka puʻuwai, nā puʻupaʻa a me ke akepaʻa: hiki i ke kauka ke koho i kahi ʻoki e hoʻāʻo e hoʻomaikaʻi i ka malformation a hoʻohana i nā lāʻau e hōʻoluʻolu i nā ʻōuli;

Eia nō naʻe, i ka hapanui o nā hihia, ʻaʻole hiki ke hoʻoponopono ʻia nā loli i nā mea nui, e like me ke akepaʻa, ka puʻuwai a me ka lolo ma hope o ka hānau ʻana, no laila nui nā keiki me ka maikaʻi ʻole o ke ake, ke kahe ʻana o ke kahe a me nā pilikia hanu hoʻoweliweli ola. I nā mahina mua.

ʻO ka maʻa mau, nā hui lapaʻau no kēia ʻano syndrome i haku ʻia i kekahi ʻoihana olakino i hoʻohui ʻia i nā pediatricians, e like me ka poʻe cardiologists, neurosurgeons, ophthalmologists a me nā orthopedists, e laʻa me.