Ka nele X Factor X

ʻO ka hemahema ʻo Factor X (ten) kahi maʻi i hoʻokumu ʻia e ka nele o kahi protein i kapa ʻia he X ma ke koko. Alakaʻi ia i nā pilikia me ke kō ʻana o ke koko (coagulation).

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 ka coagulation, a i ʻole ke kāʻei ʻana, nā kumu. 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.

ʻO ka kumu X kahi ʻano coagulation like. Hoʻokumu pinepine ʻia ka hemahema X ma muli o kahi kīnā hoʻoilina i ka gen X factor. Kapa ʻia kēia ka nele X factor. ʻO ke kahe ʻana mai ka haʻahaʻa a ka paʻakikī ke kaukaʻi ʻana i ka nui o ka hemahema.

Ma muli o kekahi ʻano a i ʻole ka hoʻohana ʻana o kekahi mau lāʻau i ka hemahema o ka ʻoihana X. Kapa ʻia kēia ka loaʻa X loaʻa ʻole. Loaʻa ka hemahema X loaʻa ʻole. Hiki ke kumu ʻia e:

• Ka nele o ka wikamina K (hānau ʻia kekahi mau keiki hānau hou me ka nele o ka wikamina K)
• ʻO ke kūkulu ʻana i nā protein pono ʻole i loko o nā aʻa a me nā lālā (amyloidosis)
• Maʻi maʻi ʻeha nui
• Hoʻohana i nā lāʻau e pale ai i ka hoʻopili ʻana (anticoagulants e like me warfarin)

Hiki ke ʻike mua ʻia nā wahine me ka hemahema X ke loaʻa iā lākou ke kahe nui o ka menstrual a me ke kahe o ke koko ma hope o ka hānau ʻana. ʻIke mua ʻia ke ʻano i nā keikikāne hānau inā loaʻa ko lākou kahe i ʻoi aku ka lōʻihi ma mua o ka maʻamau ma hope o ke ʻoki poepoe ʻana.

Pākuʻi ʻia paha nā ʻōuli i kekahi o kēia mau mea:

• Kahe i loko o nā hono
• Kahe i loko o nā mākala
• Māmā maʻalahi
• Kahe nui ka haehae
• Ka kahe ʻana o ka membrane mucus
• Nā Nosebleeds e kū mālie ʻole
• Kahe ʻana ke kaula uila ma hope o ka hānau ʻana

ʻO nā hoʻāʻo e hana ʻia ana paha:

• ʻO ke kumu X assay
• Wā thromboplastin manawa (PTT)
• Ka manawa Prothrombin (PT)

Hiki ke kāohi ʻia ke kahe ʻana e ka loaʻa ʻana o ka infravenous (IV) infusions o ka plasma a i ʻole nā ​​concentrates o nā kumu hoʻopili. Inā hemahema ʻoe i ka wikamina K, e kuhikuhi ana kāu kauka i ka wikamina K na ʻoe e lawe ma ka waha, ma o nā injection i lalo o ka ʻili, a i ʻole ma o kahi aa (intravenously).

Inā loaʻa kēia maʻi kolohe iā ʻoe, e pono e:

• E haʻi aku i kāu poʻe mālama ola ma mua o kāu loaʻa ʻana i kekahi ʻano o ke kaʻina hana, e like me ka hana ʻoki ʻana a me nā hana niho.
• E haʻi i nā ʻohana o kou ʻohana no ka mea he maʻi like paha kā lākou akā ʻaʻole ʻike ia manawa.

Hiki i kēia mau kumuwaiwai ke hāʻawi i ka ʻike hou aku e pili ana i ka hemahema X.

• National Hemophilia Foundation: Nā Mea Kūpono ʻē aʻe - www.hemophilia.org/Bleeding-Disorder/Types-of-Bleeding-Disorders/Other-Factor-Deficiencies
• National Organization for Rare Disorder --rarediseases.org/rare-diseases/factor-x-deficiency
• NIH Genetics Home Reference - ghr.nlm.nih.gov/condition/factor-x-deficiency

Maikaʻi ka hopena inā akahai ke kūlana a loaʻa ʻole iā ʻoe ka lāʻau.

ʻO ka hoʻoilina hoʻoilina hoʻoilina X kahi ʻano o ke ola holoʻokoʻa.

ʻO ka nānā ʻana no ka loaʻa X loaʻa ʻole ka hilinaʻi ʻana i ke kumu. Inā kumu ʻia e ka maʻi akepau, pili ka hopena i ka maikaʻi e mālama ʻia ai kou maʻi ate. ʻO ka lawe ʻana i nā mea kōkua Kaminamina K e mālama pono ai i ka wikamina K. Inā hopena ʻia ka maʻi e amyloidosis, aia kekahi mau koho lapaʻau. Hiki i kāu kauka ke haʻi hou aku iā ʻoe.

Hiki ke hana i ke kahe nui a i ʻole ka nalowale ʻana o ke koko (hemorrhage). Hiki ke hoʻololi ʻia nā hono i nā maʻi koʻikoʻi mai nā kahe he nui.

E kiʻi i ke kōkua olakino kūpilikia inā loaʻa ʻole kahi wehewehe a make nui paha o ke koko.

ʻAʻohe pale e ʻike ʻia no ka hemahema o nā mea hoʻoilina hoʻoilina X. Ke loaʻa ʻole ka huaʻai K i ke kumu, hoʻohana i ka wikamina K hiki ke kōkua.

Ka nele o Stuart-Prower

• Hoʻomaʻamaʻa koko
• Huʻi koko

ʻO Gailani D, Wheeler AP, Neff AT. Nā hemahema coagulation ololi. I: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Nā Kumumanaʻo a me ka Hoʻomeheu. ʻEpekema 7 Piladelapia, PA: Elsevier; 2018: chap 137.

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.