ʻO herheritary spherocytosis: nā kumu, nā ʻōuli a me nā lāʻau

Anter

• He aha ke kumu o ka spherocytosis
• Nā hōʻailona nui
• Pehea e hana ai i ka lāʻau

ʻO ka heredher spherocytosis kahi maʻi maʻi i hōʻike ʻia e nā loli o ka membrane o ke koko ʻulaʻula, kahi e makemake ai i kona luku ʻia, a no laila i manaʻo ʻia he hemolytic anemia. ʻO nā loli i ka membrane o nā hunaola ʻulaʻula e liʻiliʻi a liʻiliʻi hoʻi i ke kūpaʻa ma mua o ka maʻamau, e luku maʻalahi ʻia e ka ʻāpala.

ʻO Spherocytosis kahi maʻi hoʻoilina, i hele pū me ke kanaka mai ka hānau ʻana, akā hiki iā ia ke holomua me ka anemia o nā ʻano like ʻole. No laila, i kekahi mau hihia ʻaʻohe paha o nā ʻōuli a ma nā mea ʻē aʻe, pallor, luhi, jaundice, hoʻonui ākea a me nā loli hoʻomohala, e laʻa paha.

ʻOiai ʻaʻohe mea e ola ai, loaʻa ka lāʻau spherocytosis, pono e alakaʻi ʻia e kahi hematologist, a e hōʻike ʻia paha ka hakahaka acid folic a, i nā hihia ʻoi loa, lawe ʻia ka spleen, i kapa ʻia he splenectomy, i mea e kaohi ai i ka maʻi. .

He aha ke kumu o ka spherocytosis

Hoʻokumu ʻia ka herheritary spherocytosis e kahi hoʻololi genetic i hopena i ka loli i ka nui a i ʻole ka maikaʻi o nā protein i hana i nā membrane o nā hunaola ʻulaʻula, i ʻike nui ʻia e nā ʻulaʻula koko. Hoʻololi nā hoʻololi i kēia mau protein i ka nalowale o ka ʻoʻoleʻa a me ka pale ʻana o ka membrane o ke koko ʻulaʻula, a ʻoi aku ka palupalu a me ka liʻiliʻi o ka nui, ʻoiai like ka ʻike, hana i nā ʻulaʻula liʻiliʻi, me nā ʻano o ka poepoe a me nā pigmented.

Kū aʻe ʻo Anemia ma muli o ke kāhea ʻia ʻana o nā hunaola ʻulaʻula i spherocytosis, e luku pinepine ʻia i ka ʻāpala, keu hoʻi ke koʻikoʻi nā hoʻololi a he nalowale o ka maʻalahi a me ke kūpaʻa e hele i ka microcirculation o ke koko mai kēia meaola.

Nā hōʻailona nui

Hiki ke hoʻokaʻawale ʻia ʻo Spherocytosis ma ke ʻano he akahai, akahai a koʻikoʻi paha. No laila, ʻaʻohe o nā ʻōuli e loaʻa i nā poʻe me ka spherocytosis akahai, ʻoiai ʻo ka poʻe me ka spherositosis liʻiliʻi a koʻikoʻi paha e loaʻa nā kekona o nā hōʻailona a me nā ʻōuli e like me:

• Anemia hoʻomau paʻa;
• Pallor;
• Ka luhi a me ka hoʻomanawanui ʻole i ka hoʻoikaika kino;
• Hoʻonui ka bilirubin i ke koko a me ka jaundice, ʻo ia ke kala melemele o ka ʻili a me nā membrane mucous;
• Ke hoʻokumu ʻia ʻana o nā pōhaku bilirubin i loko o ka hipa;
• Hoʻonui i ka nui o ka spleen.

I mea e ʻike pono ai i ka spherocytosis hereditary, i ka hoʻohui o ka loiloi klinikal, hiki i ka hematologist ke kauoha i nā hoʻokolohua koko e like me ka helu o ke koko, ka helu retikulite, ka ana bilirubin a me ke kahe o ke koko ākea e hōʻike ana i nā loli e hōʻike nei i kēia ʻano anemia.Hōʻike ʻia hoʻi ka hoʻokolokolo ʻana no ka palupalu osmotic, kahi e ana ai i ke kūpaʻa ʻana o ka membrane ʻulaʻula koko.

Pehea e hana ai i ka lāʻau

ʻAʻohe lāʻau i ka heredher spherocytosis, akā naʻe, koi paha ka hematologist i nā lāʻau e hiki ai ke hōʻemi i ka maʻi a me nā ʻōuli, e like me ka makemake o ka mea maʻi. I ka hihia o ka poʻe hōʻike ʻole i nā ʻōuli o ka maʻi, ʻaʻohe pono kūpono e pono ai.

Paipai ʻia ka hoʻololi ʻana o ka waikawa folic no ka mea, ma muli o ka hoʻonui ʻia o ka haki o nā hunaola ʻulaʻula, ʻoi aku ka pono o kēia mea no ke kūkulu ʻana i nā hunaola hou i ka momona.

ʻO ke ʻano nui o ka mālama ʻana ka wehe ʻana i ka māpala e ka ʻoki ʻana, i hōʻike pinepine ʻia i nā keiki ma mua o 5 a 6 mau makahiki paha i loaʻa ka anemia koʻikoʻi, e like me ka poʻe i loaʻa ka hemoglobin ma lalo o 8 mg / dl i ka helu o ke koko. aiʻole ma lalo o 10 mg / dl inā he mau hōʻailona koʻikoʻi a i ʻole nā ​​pilikia e like me nā pōhaku gall gall. Hiki ke hana ʻoki kino i nā keiki i loaʻa ka lohi o ka ulu ʻana ma muli o ka maʻi.

ʻO ka poʻe i loaʻa i ka heʻe lūlū e hoʻomohala i kekahi mau maʻi a i ʻole thromboses, no laila pono nā lāʻau āpau, e like me ka pneumococcal, me ka hoʻohana ʻana o ASA e kaohi i ke kāpili ʻana o ke koko. E nānā pehea e hana ai ke ʻoki ʻana no ka hemo ʻana o ka pelele a me ka mālama kūpono.