Hemopilia

ʻO Hemophilia e pili ana i kahi hui o nā maʻi kahe o ke kahe a lōʻihi ka lōʻihi o ke kō ʻana o ke koko.

ʻElua ʻano hemophilia:

• Hemophilia A (hemophilia kahiko, a i ʻole hemahema ma ke ʻano VIII)
• Hemophilia B (maʻi Kalikimaka, a i ʻole hemahema IX)

Ke kahe nei ʻoe i ke kahe, hana ʻia kahi hopena o nā hopena i loko o ke kino e kōkua ai i ke kōkō ʻana o ke koko. Kapa ʻia kēia hana ke cascade coagulation. Pili ia i nā proteins kūikawā i kapa ʻia ʻo ka coagulation, a i ʻole nā ​​mea hoʻopili. Loaʻa paha iā ʻoe kahi manawa kiʻekiʻe o ke kahe ʻana o ke kahe inā e nalowale ana hoʻokahi a ʻoi paha o kēia mau kumu a hana ʻole paha e like me ka mea e pono ai.

Hoʻokumu ʻia ʻo Hemophilia e ka nele o ke kumu ʻōpala VIII a i ʻole IX i loko o ke koko. I ka hapanui o nā hihia, hala ʻia ka hemophilia e nā ʻohana (hoʻoilina). ʻO ka hapanui o ka manawa, hoʻoili ʻia i nā keiki kāne.

ʻO ka hōʻailona nui o ka hemophilia ke kahe ʻana o ke kahe. ʻAʻole ʻike ʻia nā hihia akahai a hiki i ka wā ma hope o ke ola, ma hope o ke kahe nui ʻana ma hope o ke kaha ʻana a i ʻole kahi ʻeha.

I nā hihia maikaʻi loa, kū i ke kahe ke kumu ʻole. Hiki ke kahe i loko o nā wahi āpau a maʻa mau ke kahe ʻana i nā hono.

ʻO ka hapanui pinepine, ʻike ʻia ka hemophilia ma hope o ka loaʻa ʻana o kahi kanaka i ka hanana kahe koko ʻole. Hiki ke hōʻoia ʻia e kahi hoʻokolohua koko i hana ʻia e ʻike i ka pilikia, inā loaʻa i nā lālā ʻohana ʻē aʻe ke ʻano.

ʻO ka lapaʻau maʻamau ka mea e puku i ka nalowale ʻana o ke kumu paʻa no ke koko ma o ka aa (infraions intravenous).

Pono e mālama ʻia i ka wā ʻoki kino inā loaʻa ʻoe i kēia maʻi koko. No laila, e haʻi pono aku i kāu kauka maʻi he maʻi maʻi kāu.

He mea nui nō hoʻi e kaʻana like i ka ʻike e pili ana i kāu maʻi me nā ʻohana pili koko ma muli o ka hopena o lākou.

ʻO ka hui ʻana i kahi hui kākoʻo kahi e puʻunaue ai nā lālā i nā pilikia maʻamau hiki ke hōʻoluʻolu i ke koʻikoʻi o kahi maʻi wā lōʻihi (mau).

Hiki i ka hapanui o ka poʻe me ka hemophilia ke hana i nā hana maʻamau. Akā ke kahe nei kekahi poʻe i nā hono, kahi e kaupalena ai i kā lākou hana.

Hiki i kekahi poʻe liʻiliʻi me ka hemophilia ke make i ke kahe nui.

Hemophilia A; Hemophilia kahiko; Hewa ʻole ʻo Factor VIII; Hemophilia B; Maʻi Kalikimaka; Ka hemahema Factor IX; Maʻi maʻi heʻe - hemophilia

• Huʻi koko

Carcao M, Moorehead P, Lillicrap D. Hemophilia A a me B. In: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Nā Kumumanaʻo a me ka Hoʻomeheu. ʻEpekema 7 Piladelapia, PA: Elsevier; 2018: chap 135.

Halelu JE. Hemostasis a me ka coagulation koko. I: Hall JE, hoʻoponopono. ʻO Guyton a me Hall Textbook of Medical Physiology. 13th ed. Piladelapia, PA: Elsevier; 2016: chap 37.

ʻO Ragni MV. Nā maʻi hemorrhagic: deficiencies factor coagulation. I: Goldman L, Schafer AI, eds. ʻO ka lāʻau lapaʻau Goldman-Cecil. 25th hua. Piladelapia, PA: Elsevier Saunders; 2016: chap 174.