Hōʻikeʻike Pyruvate Kinase

Anter

• No ke aha e kauoha ʻia ai kahi hōʻike Pyruvate Kinase?
• Pehea e hoʻokau ʻia ai ka hōʻike?
• He aha nā pilikia o ka hoʻāʻo?
• Ka hoʻomaopopo ʻana i kāu hopena

Hōʻikeʻike Pyruvate Kinase

Hāpai nā hunaola ʻulaʻula (RBC) i ka oxygen ma loko o kou kino. ʻO kahi enzyme i kapa ʻia ʻo pyruvate kinase e pono ai i kou kino e hana i nā RBC a hana kūpono. ʻO ka pyruvate kinase testis kahi hōʻike koko e hoʻohana ʻia e ana i nā pae o ka pyruvate kinase i kou kino.

Ke loaʻa ʻoe i kahi kinase pyruvate liʻiliʻi, haki wikiwiki kāu RBC ma mua o ka maʻamau. Hoʻoemi kēia i ka helu o nā RBC i loaʻa e halihali i ka oxygen i nā meaola nui, nā aʻa, a me nā hunaola. ʻO ke ʻano o ka hopena i ʻike ʻia ʻo hemolytic anemia a hiki ke loaʻa i nā hopena olakino koʻikoʻi.

ʻO nā ʻōuli o ka hemolytic anemia me:

• jaundice (melemele o ka ʻili)
• ka hoʻonui ʻana o ka pākana (ʻo ka hana mua o ka iwi lūlū ʻo ke kānana i ke koko a luku i nā RBC kahiko a hōʻino ʻia)
• anemia (kahi hemahema o nā RBC olakino)
• ʻili mae
• luhi

Hiki i kāu kauka ke hoʻoholo inā loaʻa iā ʻoe kahi hemahema pyruvate kinase e pili ana i nā hopena o kēia a me nā hoʻokolohua diagnostics ʻē aʻe.

No ke aha e kauoha ʻia ai kahi hōʻike Pyruvate Kinase?

ʻO ka hemahema o Pyruvate kinase kahi maʻi genetical i kū i ke autosomal recessive. ʻO kēia ke lawe nei kēlā me kēia mākua i ka ʻāpana hemahema no kēia maʻi. ʻOiai ʻaʻole hōʻike ʻia ka moʻokūʻauhau i kekahi o nā mākua (ʻo ia hoʻi ʻaʻohe o ka pyruvate kinase deficit), he 1-in-4 ka manawa kūpono o ka recessive e hōʻike ʻia i nā keiki a nā mākua i hui pū ai.

ʻO nā keiki i hānau ʻia e nā mākua me ka gene deficit kinase pyruvate e hoʻāʻo ʻia no ka maʻi e hoʻohana ana i ka hoʻāʻo kinase pyruvate. Hiki paha i kāu kauka ke kauoha i ka hoʻokolohua ma ka ʻike ʻana i nā hōʻailona o ka hemahema pyruvate kinase. ʻO nā ʻikepili i hōʻiliʻili ʻia mai kahi hoʻokolohua kino, ka hoʻāʻo kinase pyruvate, a me nā hoʻokolohua koko ʻē aʻe e kōkua i ka hōʻoia ʻana i kahi hōʻailona.

Pehea e hoʻokau ʻia ai ka hōʻike?

ʻAʻole pono ʻoe e hana i kekahi mea kikoʻī e hoʻomākaukau ai no ka hoʻokolohua pyruvate kinase. Eia nō naʻe, lawelawe pinepine ʻia ka hōʻike i nā keiki ʻōpio, no laila makemake paha nā mākua e kamaʻilio me kā lākou keiki e pili ana i ka hopena o ka hoʻāʻo. Hiki iā ʻoe ke hōʻike i ka hoʻokolohua ma kahi pēpē e kōkua ai e hōʻemi i ka hopohopo o kāu keiki.

Hana ʻia ka pyruvate kinase test ma ke koko i lawe ʻia i ka wā o ke kaha kiʻi maʻamau. E lawe kahi mea mālama ola i kahi laʻana o ke koko mai kou lima a lima paha e hoʻohana ana i kahi nila liʻiliʻi a i ʻole kahi pahi i kapa ʻia he lancet.

E hōʻiliʻili ke koko i loko o kahi paipu a hele i kahi lab no ka loiloi. Hiki i kāu kauka ke hāʻawi iā ʻoe i ka ʻike e pili ana i nā hopena a me nā manaʻo o lākou.

He aha nā pilikia o ka hoʻāʻo?

ʻO nā mea maʻi e hana ana i ka hoʻāʻo kinase pyruvate e ʻike paha i kekahi mau hōʻoluʻolu ʻole i ke kahakaha ʻana o ke koko. Aia paha he ʻeha ma ka pūnaewele injection mai nā lāʻau o ka nila. Ma hope, ʻike paha ka poʻe maʻi i ka ʻeha, ʻeha a i ʻole ka ʻū ʻana i ka pūnaewele injection.

Heʻuʻuku ka makaʻu o ka hoʻāʻo. Loaʻa i nā makaʻi kūpono o kekahi kahakaha koko:

• pilikia i ka loaʻa ʻana o kahi laʻana, e hopena ana i nā lāʻau ʻo ka nui
• nui ke kahe ʻana ma ke kahua o ka nila
• maule ma muli o ke kahe o ke koko
• ka hōʻiliʻili o ke koko ma lalo o ka ʻili, ʻike ʻia he hematoma
• hoʻomohala o ka maʻi maʻi kahi e haki ai ka ʻili e ka nila

Ka hoʻomaopopo ʻana i kāu hopena

E ʻokoʻa nā hopena o ka hoʻokolohua kinase pyruvate ma muli o ke keʻena hoʻokolohua e kālailai ana i ka hāpana koko. ʻO ke kumukūʻai maʻamau no ka hoʻokolohua kinase pyruvate ka mea maʻamau 179 plus a i ʻole hoʻemi ʻia he 16 mau paukū pyruvate kinase no 100 milliliters o RBC. Hōʻike nā pae haʻahaʻa o pyruvate kinase i ka loaʻa ʻana o ka hemahema pyruvate kinase.

ʻAʻohe lāʻau lapaʻau no ka hemahema pyruvate kinase. Inā ʻike ʻia ʻoe me kēia ʻano, hiki i kāu kauka ke koi aku i nā ʻano lāʻau like ʻole. I nā manawa he nui, pono ka poʻe maʻi me ka hemahema pyruvate kinase e hana i ke kahe o ke koko e pani i nā RBC i hōʻino ʻia. ʻO ke transfusion koko kahi ʻoki o ke koko mai kahi mea hāʻawi.

Inā ʻoi aku ka nui o nā ʻōuli o ka maʻi, hiki i kāu kauka ke koi aku i kahi splenectomy (ka hemo ʻana o ka iwi maha). Ke kōkua nei i ka hemo ʻana o ka spleen e ʻokiʻoki i ka helu o nā RBC e luku ʻia ana. ʻOiai me ka spleen i hoʻoneʻe ʻia, mau paha nā ʻōuli o ka maʻi. ʻO ka nūhou maikaʻi e hōʻemi loa ka mālama ʻana i kāu mau hōʻailona a hoʻomaikaʻi i kou ola.