Osteopetrosis: he aha ia, nā ʻōuli a me nā lāʻau

Anter

• Nā hōʻailona osteopetrosis
• Nā kumu o ka osteopetrosis
• Pehea e hana ai i ka lāʻau

ʻO ka Osteopetrosis kahi maʻi osteometabolic hereditary hereditary kahi i ʻoi aku ai ka nui o nā iwi ma mua o ka maʻamau, i hana ʻia ma muli o ke kaulike ʻole o nā hunaola ke kuleana no ke kaʻina o ka iwi a me ka haki, e paipai ana i ka hoʻonui ākea o ka iwi iwi a hopena i ka hiʻona kekahi mau ʻōuli e like me e like me nā iwi lahilahi, paʻakikī i ka hoʻolohe a me nā loli i ka hoʻomohala neuronal, no ka laʻana.

ʻO ka mālama ʻana no ka osteopetrosis e pono e koi ʻia e kahi hui kauka e komo pū me ka pediatrician, hematologist a me ka orthopedist, a me ka hoʻohuli ʻana i ka iwi iwi i mea e hoʻomaikaʻi ai i ka hana o nā hunaola e pili ana i ka hoʻokumu iwi.

Nā hōʻailona osteopetrosis

Hiki ke ʻike ʻia nā hōʻailona a me nā ʻōuli o ka osteopetrosis ma hope koke iho o ka hānau ʻana, no ka mea he maʻi hānau ia, a i ʻole he hōʻailona a hōʻailona paha i ka wā makua. ʻO ka hiʻohiʻona nui o ka osteopetrosis ka hoʻonui ʻana i ka nui o ka iwi, i hiki ke ʻike ʻia e ka nānā ʻana i ka iwi densitometry.

Hoʻohui ʻia, aia kekahi ʻoi aku ka nui o ka haki, ʻoiai ma muli o ka disregulation o nā hunaola ke kuleana no ke kaʻina hana o ka iwi a me ka luku ʻana, ʻoi aku ka maʻalahi o nā iwi.

Pili nā ʻōuli o ka osteopetrosis i ka ʻoiaʻiʻo ʻoi aku ka nui o ka waiho ʻana o nā mea iwi i loko o ke kino, i hiki ke hopena i nā loli i loko o ke kino, nā hōʻailona nui:

• ʻIke hilahila;
• Lohe paʻakikī;
• Nā maʻi hou o nā niho a me nā niho;
• Ka hoʻonui ʻana i ke ake a me nā ʻāpala, e hopena ana i ka hana i hoʻololi ʻia o nā hunaola koko;
• Hoʻololi i ka ulu neuronal;
• Hoopanee i ka hanau ana o na niho;
• Hoʻonui i ka kaomi intracranial.

ʻO ka ʻike ʻana i ka osteopetrosis i hana ʻia e ka orthopedist ma o nā loiloi kiʻi e like me X-ray a me ka iwi densitometry, kahi hōʻike maʻalahi a ʻeha ʻole e manaʻo ʻia e hōʻoia i ka momona o nā iwi o ke kanaka, e ʻae ana e nānā i ka makaʻu o nā haki, no ka laʻana. Hoʻomaopopo i ka osteopetrosis a pehea e hana ai.

Eia nō naʻe, e hōʻoia i ke ʻano a me nā pilikia o ka osteopetrosis, kauoha paha ke kauka i nā hoʻokolohua diagnostics ʻē aʻe, e like me ka tomography compute a i ʻole ke kiʻi ʻana i ka resonance magnet e nānā i ke kū ʻana o nā liona i kekahi mau lālā e like me nā maka a me nā pepeiao, me nā hoʻokolohua koko.

Nā kumu o ka osteopetrosis

Hoʻokumu ʻia ka Osteopetrosis e nā kīnā i loko o hoʻokahi a ʻoi paha o nā genes kuleana no ka hoʻokumu a me ka hoʻomohala ʻana o nā osteoclasts, ʻo ia nā hunaola e hoʻoneʻe i nā iwi iwi kahiko a hoʻololi iā ia me kahi mea olakino hou. Kaukaʻi ʻia i ke kumu o nā ʻāpana i hoʻololi ʻia, hiki ke hoʻololi i ke ʻano o ka osteopetrosis:

• Osteopetrosis keiki kamaliʻi: loaʻa i ke keiki ka maʻi mai ka hānau ʻana ma muli o nā hemahema i nā ʻāpana i hoʻoilina ʻia mai ka makuakāne a me ka makuahine;
• Osteopetrosis makua: ʻike wale ʻia ka osteopetrosis i ka wā ʻōpio a i ʻole ke kanaka makua, ke kumu ʻia e nā gen i hoʻololi ʻia i hoʻoilina ʻia mai ka makuakāne a makuahine paha.

I ka hihia o ka osteopetrosis makua, hiki i ka hoʻololi ʻana o nā genes ke kumu ʻia e ka hoʻololi, me ka loaʻa ʻole o ka hoʻoilina mai nā mākua mai.

Pehea e hana ai i ka lāʻau

Pono e alakaʻi ʻia ka mālama ʻana no ka osteopetrosis e kahi kime o nā ʻoihana olakino, e like me ka pediatrician, he orthopedist, he hematologist, he endocrinologist a me kahi physiotherapist.

I kēia manawa, ʻo ka lapaʻau kūpono loa no ka osteopetrosis ka hoʻoneʻe ʻana o ka iwi iwi, no ka mea, hana ʻia nā hunaola i hana ʻia i loko o kēlā meaola. No laila, ke hana ʻana i ke transplant, hiki i ka mea ke hoʻoponopono i ka hana o nā hunaola ke kuleana no ka hoʻokumu a me ka luku ʻana o nā iwi, e hakakā ana me ka osteopetrosis. Hoʻomaopopo i ka hana ʻia ʻana o ka iwi iwi.

ʻOiai ʻo ka hoʻoneʻe ʻana o ka iwi iwi ka lāʻau i koi ʻia no ka hoʻōla ʻana i ka maʻi, hiki ke ʻōlelo ʻia nā lāʻau ʻē aʻe i mea e hāpai ai i ke kōkua ʻana i nā ʻōuli, e like me:

• Nā Injections me Interferon gamma-1b, kahi lāʻau hiki ke hoʻolohi i ka ulu ʻana o ka maʻi;
• Hoʻokomo ʻia ʻo Calcitriol, ʻo ia kahi ʻano hana o ka wikamina D e kōkua ai i ka hoʻoulu ʻana i nā hunaola iwi e hoʻomohala maʻamau a hoʻoliʻiliʻi i ka iwi iwi;
• ʻEdʻai Prednisone, kahi hone e like me ka cortisone i hiki ke hoʻomaikaʻi i ka hana ʻana o nā hunaola pale i loko o ke kino, i hana ʻia i loko o nā iwi;
• Nā kau Physiotherapy, i ko lākou hoʻomaikaʻi ʻana i ka ikaika o ka mea maʻi, kōkua i ka pale ʻana i nā haʻi iwi a hoʻomaikaʻi i ke kūʻokoʻa i kekahi mau hana o kēlā me kēia lā.

E ʻōlelo paha ke kauka iā ʻoe e nīnau i ke kauka meaʻai e hoʻopili i kāu papaʻai e hoʻopili i nā meaʻai e kōkua ai i ka hoʻomohala ʻana i ke kino a me ka iwi, keu hoʻi i ka wā kamaliʻi.

Eia hou, he mea nui e kipa mau i ka ophthalmologist, otolaryngologist a me ka dentist e loiloi i ka hoʻomohala a me ke ʻano o kekahi mau liona a i ʻole malformations i nā maka, nā niho, ka ihu, nā pepeiao a me ka puʻu, no ka laʻana.