Loaʻa iā ʻoe kekahi maʻi kahi ʻoki liʻiliʻi ʻaʻole e pau ke kahe ʻana o ke koko? (Factor VII Deficiency)

Ke manaʻo nei ʻoe i kekahi manawa ua lōʻihi ka manawa no kahi ʻoki liʻiliʻi e hoʻōki ai i ke kahe ʻana o ke koko? A i ʻole e lilo wale ʻoe i polū a palapū? Malia paha he kumu ma hope o kēia mau mea a mākou i ʻike ʻole ai. ʻO ia ke ʻano o ka hemahema o Factor VII.

He aha ka hemahema o ka Factor VII?

I ka ʻōlelo maʻalahi, ʻo ka hemahema o Factor VII kahi maʻi genetic kahi i ʻole ai e paʻa pono kou koko, ʻo ia hoʻi, ʻaʻole ia e hoʻōki koke i ke kahe ʻana o ke koko. Aia kekahi mau ʻano protein i loko o ko kākou koko i kapa ʻia ʻo 'blood clotting factors' a i ʻole 'coagulation factors' . ʻO Factor VII kekahi o ia mau protein. Hana pū kēia mau protein a pau me kāu mau platelets e hana i kahi paʻa koko e hoʻōki i ke kahe ʻana o ke koko ke ʻeha ʻoe. Ua like kēia kaʻina hana me ke kūkulu ʻana i kahi pā me nā piliki a me ka sima. No laila, inā ʻaʻole lawa kēia protein koʻikoʻi i kapa ʻia ʻo Factor VII i loko o kou kino, a inā ʻaʻole ia e hana pono, ʻoi aku ka lōʻihi o ke koko e paʻa ai ma mua o ka maʻamau. ʻO ia ke kumu e ʻoi aku ai kou ʻeha a me ke kahe ʻana o ke koko ma mua o nā poʻe ʻē aʻe.

ʻO nā inoa ʻē aʻe no kēia ʻo ka maʻi o Alexander a me ka hemahema o ka proconvertin .

Pehea ka maʻamau o kēia hemahema factor VII?

He maʻi laha ʻole kēia. Ma ka honua holoʻokoʻa, hoʻopilikia ia i hoʻokahi i loko o 500,000 mau kānaka . Nui nā maʻi koko i hoʻoilina ʻia e nā kauka i kapa ʻia he "lahilahi." ʻO ka hemahema o Factor VII kekahi o nā maʻi laha ʻole.

He aha nā hōʻailona o kēia?

ʻO ka hemahema o Factor VII hiki ke hoʻoulu i ke kahe koko lōʻihi a me ka maʻalahi o ka ʻeha. ʻO nā hōʻailona nui:

• Nā ʻāʻī koko.
• Ke kahe koko o ka ihu (epistaxis) , a me ka pilikia e hoʻōki iā lākou.
• No nā wahine, ʻo ke kahe koko nui i ka wā menstruation (menorrhagia).
• Ke koko i loko o ka mimi (hematuria) a i ʻole ke koko i loko o ka ʻōpala.
• Ke kahe mau nei ke koko ma hope o kahi ʻeha, ʻoki kino, a hānau keiki paha.
• Ke kahe ʻana o ke koko i loko o ke kino (no ka laʻana, i loko o ka ʻōnaehana gastrointestinal, nā ʻiʻo, nā ʻiʻo, nā hono).
• Hiki nō hoʻi ke kahe koko ma loko o ke poʻo . He ʻoi aku ka koʻikoʻi o kēia.
• Hiki ke kahe koko koke ke hoʻomaka koke ke kahe koko me ke kumu ʻole.

ʻOkoʻa ke koʻikoʻi o kēia mau hōʻailona a me ka wā e ʻike ʻia ai mai kēlā kanaka a i kēia kanaka. No ka laʻana, hiki ke ʻike ʻia kahi pēpē me ka hemahema nui o Factor VII i ka wā hānau. Eia nō naʻe, ʻaʻole paha e ʻike ʻia kekahi me nā hōʻailona ʻoluʻolu a hiki i ko lākou wā ʻelemakule.

He aha ke kumu o ka hemahema o ka Factor VII?

ʻOiai he maʻi hoʻoilina kēia, aia kekahi ala e hiki ai i kēia ʻano ke hiki mai i ka wā o ke ola.

Inā hānau ʻia ʻoe me ka hemahema o ka factor VII , ʻo ia hoʻi ua hoʻoilina ʻoe i kahi mutation, a i ʻole kīnā, i loko o kāu gene F7 . Hoʻomalu kēia gene i ke ʻano o ka hana ʻana o kou kino i ka factor VII. No ka hoʻomohala ʻana i ka hemahema o ka factor VII, pono ʻoe e hoʻoilina i kēia mutation gene mai nā mākua olaola ʻelua . Ua kapa ʻia kēia he autosomal recessive inheritance . Inā loaʻa i nā mākua ʻelua kēia mutation, loaʻa iā lākou ma kahi o 25% ka manawa e loaʻa ai kahi keiki me ka hemahema o ka factor VII.

ʻO ka hemahema o Factor VII e ulu ana i ka hala ʻana o ka manawa ua kapa ʻia ʻo 'acquired factor VII deficiency' . Aia kekahi mau kumu kūpono no kēia:

• Maʻi akepaʻa: Hana kou akepaʻa i ka protein Factor VII. Inā loaʻa iā ʻoe ka maʻi akepaʻa koʻikoʻi, ʻaʻole paha e hiki i kou akepaʻa ke hana pono i kēia hana.
• Ka nele o ka wikamina K: He mea nui ka wikamina K no ke kino e hana i ka Factor VII. Inā ʻaʻole lawa ka nui o ia mea, hiki iā ʻoe ke loaʻa ka hemahema o ka Factor VII.
• Nā lāʻau hoʻomāmā koko: Hiki ke loaʻa kēia ʻano i ka wā e hoʻohana ai i nā lāʻau hoʻomāmā koko e like me Warfarin (Coumadin®) .
• Nā maʻi o ke koko a me ke kanesa: Ua hōʻike ʻia ka hemahema o Factor VII i nā poʻe me nā maʻi e like me ka anemia aplastic a me ka leukemia .

He aha nā pilikia o ka hemahema o Factor VII?

I nā hihia koʻikoʻi, hiki i ka hemahema o Factor VII ke alakaʻi i nā pilikia olakino ʻē aʻe inā ʻaʻole mālama ʻia. Eia kekahi laʻana:

• Anemia: Ke nalowale nui ke koko mai ke kino, hiki ke hāʻule nui ka helu o kāu mau cell koko ʻulaʻula.
• Ka pōʻino o nā hono: Ke kahe ke koko i loko o kahi hono, hiki ke mae nā ʻiʻo ma laila a ʻaʻole hiki i ke hono ke kūlou a pololei paha.
• Hematomas: ʻO ka hematoma kahi hōʻiliʻili o nā ʻōpū koko ma lalo o kou ʻili. Inā kū mai kahi hematoma nui, hiki iā ia ke kau i ke kaomi ma luna o nā ʻōpū kokoke a hoʻoulu i nā hōʻailona ʻeha.

ʻO ka mea nui loa, hiki i ke kahe koko nui ke hoʻoweliweli i ke ola inā ʻaʻole mālama ʻia. No laila he mea nui e ʻimi i ke aʻo lapaʻau inā ʻaʻole e pau ke kahe koko.

Pehea e ʻike ʻia ai ka hemahema o Factor VII?

E nīnau aku kāu kauka iā ʻoe e pili ana i kou mau hōʻailona a inā paha he moʻolelo ko kekahi o kou ʻohana no nā maʻi kahe koko. Hoʻomaopopo ʻia ka hemahema o Factor VII me nā hoʻāʻo koko e loiloi ai i ka maikaʻi o ke kāpili ʻana o kou koko.

ʻO kēia mau hoʻokolohua:

• Hoʻāʻo manawa Prothrombin (PT/INR): Ke ana kēia i ka lōʻihi o ke koko e kulu ai ma muli o ka hana o kahi mea kulu koko i kapa ʻia ʻo prothrombin. Inā loaʻa iā ʻoe ka hemahema o ka factor VII, ʻoi aku ka lōʻihi o ke koko e kulu ai ma mua o ka maʻamau.
• Hoʻāʻo manawa thromboplastin hapa (PTT): Ke ana kēia i ka manawa e pono ai ke koko e hoʻopili ma muli o ka hana o nā mea hana clotting ʻē aʻe ma mua o ka prothrombin. ʻO ka maʻamau, he maʻamau nā hopena o kēia hoʻāʻo i kahi kanaka me ka hemahema o ka factor VII.
• Hoʻāʻo Factor VII: Hōʻike kēia hoʻāʻo pehea e hana ai ka protein Factor VII.

Pehea e mālama ʻia ai ka hemahema o ka factor VII?

ʻO ka maʻamau, ʻo ka hoʻohui ʻana i ka factor VII e hana pono ana i kou koko ka mea maʻi. E hana pū paha ʻoe me ke kauka loea i nā maʻi koko, i kapa ʻia he hematologist , nāna e hoʻoholo i ka lāʻau lapaʻau āu e pono ai a me ka lōʻihi o kou pono ʻana.

Aia kekahi mau ʻano lapaʻau nui:

• ʻO ka Recombinant factor VIIa (NovoSeven®): Ua ʻāpono ʻia kēia lāʻau lapaʻau e ka US Food and Drug Administration (FDA) e mālama i ka hemahema o ka factor VII. Hana ʻia ia e nā kānaka ʻepekema ma kahi keʻena hoʻokolohua. ʻO ke ʻano kēia, ʻaʻole i hana ʻia mai ke koko i hāʻawi ʻia mai nā kānaka e like me nā lāʻau lapaʻau pani factor VII ʻē aʻe.
• Nā ʻāpana paʻakikī Prothrombin (PCC): Loaʻa i kēia lāʻau lapaʻau nā ʻāpana kiʻekiʻe o ʻehā mau mea hana clotting, me ka factor VII, i lawe ʻia mai ke koko i hāʻawi ʻia.
• Plasma hau hou (FFP): Hiki ke hāʻawi ʻia iā ʻoe ka plasma ma ke ʻano intravenous e kōkua i ke kūkulu hou ʻana i kāu mau waihona Factor VII.

Nā lāʻau lapaʻau ʻē aʻe:

• Nā mea antifibrinolytic: Hana kēia mau lāʻau lapaʻau ma ke kāohi ʻana i nā ʻūpā koko e hana ʻia ana i loko o kou kino mai ka hoʻoheheʻe ʻana. Kōkua lākou i ka pale ʻana i ke kahe koko nui ma ka pale ʻana i nā ʻūpā koko i loko o kou kino. ʻO nā laʻana e komo pū me nā lāʻau lapaʻau i kapa ʻia ʻo aminocaproic acid a me ka tranexamic acid .
• Nā lāʻau hoʻomalu hānau:Pale aku kekahi ʻano lāʻau hoʻomalu hānau i ke kahe koko nui i ka wā o ka menstruation.
• Nā mea hoʻohui a i ʻole nā ​​​​inikini Vitamin K: Inā loaʻa kou maʻi i ka nele o ka vitamin K, ʻo ka loaʻa ʻana o ka vitamin K hou aku e hiki ke kōkua i kou kino e hana i nā mea hou aku VII.

He aha nā pilikia/nā hopena ʻaoʻao o ka lāʻau lapaʻau?

ʻO nā lāʻau lapaʻau e hoʻohui i ka factor VII i ke koko hiki ke hana i nā pilikia i kekahi manawa. Eia nō naʻe, he mea nui e hoʻomanaʻo ua ʻike kāu kauka i kēia mau pilikia a ke hana nei i nā hana e pale aku ai iā lākou.

ʻO nā pilikia e hiki mai ana o ka lāʻau Factor VII e komo pū me:

• Nā ʻūpā koko ʻino: ʻO ka hoʻohui ʻana i ka factor VII e hoʻonui paha i ka pilikia o nā ʻūpā koko e hiki ke ālai i nā kīʻaha koko. E nānā kāu kauka i kāu nui o ka lāʻau lapaʻau e hōʻemi i kēia pilikia.
• Ka ʻike ʻia ʻana i nā pathogens: I ka wā e loaʻa ai ke koko i hāʻawi ʻia, aia mau ka pilikia o ka ʻike ʻia ʻana i nā pathogens ʻino (e like me nā virus a i ʻole bacteria) i loko o ke koko. Nānā pono nā kauka i nā huahana koko e pale aku i kēia.
• Nā hopena hoʻoili koko: Loaʻa paha iā ʻoe kahi hopena maʻi i ka plasma i hāʻawi ʻia.
• ʻO ka nui o ka plasma: Ua wāwahi koke ʻia ka Factor VII a lawe ʻia mai kou koko. I mea e pale ai i kēia, pono paha ʻoe i ka nui o ka plasma e mālama i ka nui o ka factor VII i loko o kou kino. Hiki i kēia ke hoʻonui i ka plasma i loko o kāu ʻōnaehana. Hiki i nā hopena o ka nui o ka plasma ke komo pū me ke koko kiʻekiʻe a me ka pehu ʻana o nā māmā (pulmonary edema) .

He aha kaʻu e manaʻo ai inā loaʻa iaʻu ka hemahema o Factor VII?

He maʻi ia no ke ola holoʻokoʻa ka hemahema o Factor VII. ʻOiai ʻaʻohe lāʻau lapaʻau, hiki iā ʻoe ke hana pū me kāu kauka e hoʻokele iā ia.

ʻO kekahi o nā pilikia nui i ka hoʻoholo ʻana i ke koʻikoʻi o ka maʻi, ʻo ia ka hiki ʻole ke wānana i ke koʻikoʻi o nā hōʻailona ma muli o ka haʻahaʻa o kāu mau pae Factor VII. Akā, hana pū nā kauka me nā poʻe me kēia hemahema ma ke ʻano pākahi. E hilinaʻi kou manaʻo i nā mea kūikawā iā ʻoe, e like me kou ʻike, ke kumu o ka hemahema, pehea ʻoe e pane ai i ka lāʻau lapaʻau, a me ke koʻikoʻi o kou kahe koko.

Pehea wau e mālama ai iaʻu iho?

He mea nui e haʻi aku i nā mea lawelawe olakino āpau e mālama iā ʻoe e pili ana i kou kūlana. Inā loaʻa i kāu keiki ka hemahema o ka factor VII, e haʻi aku i kekahi mea nāna e mālama iā ia (me nā kumu a me nā limahana mālama keiki).

E nīnau i kāu kauka e pili ana i nā mea hiki iā ʻoe ke hana e mālama iā ʻoe iho i kēlā me kēia lā. Hiki iā ia ke paipai i nā mea e like me:

• E pale i nā hana pilikia nui:Makemake paha ʻoe e pale i nā hana e hoʻonui ai i ka pilikia o ka hōʻeha (e laʻa, nā haʻuki pili).
• E pale i kekahi mau lāʻau lapaʻau: Pono paha ʻoe e noho mamao mai nā lāʻau lapaʻau e hoʻomāmā i kou koko (e laʻa, aspirin , NSAID , SSRI antidepressants ). Hiki paha iā ʻoe ke pale i nā mea hoʻohui i loaʻa ka ʻaila iʻa, ka wikamina E, a me ka turmeric, no ka mea hiki i kēia mau mea ke hoʻonui i ke kahe ʻana o ke koko.
• E mālama i kope o kāu hoʻolālā lapaʻau: He mea nui e mālama i nā palapala mai kāu hematologist e pili ana i ke ʻano e mālama ʻia ai ʻoe e nā loea olakino i ka wā o kahi pilikia.
• E kiʻi i ka ʻōlelo aʻoaʻo genetic a i ʻole ka hoʻāʻo ʻana: Ma mua o ka hoʻāʻo ʻana e loaʻa kahi pēpē, hiki iā ʻoe ke kamaʻilio me kahi kākāʻōlelo genetic e loiloi i ka pilikia o kāu keiki e hoʻoilina i ka hemahema factor VII.

Āhea wau e ʻike ai i kaʻu kauka?

Inā ʻike ʻoe a i ʻole kāu keiki i kekahi mau hōʻailona o ke kahe koko mau a i ʻole ka uliuli pinepine, e ʻike i kāu kauka. He mea maʻamau ka kahe ʻana o ka ihu a me ka uliuli i kekahi manawa. Akā inā ʻoi aku ka nui o ka hana ʻana ma mua o ka mea maʻamau, he hōʻailona paha ia o ka pilikia koko.

Āhea wau e hele ai i ka Emergency Treatment Unit (ETU) ?

Inā he mea hoʻoweliweli kou kahe ʻana o ke koko i kou olakino, e hele i ka lumi pilikia. E nānā i nā hōʻailona e like me kēia:

• Ke kahe ʻana o ke koko ihu no hoʻokahi hola a ʻoi paha.
• Ka hoʻomaʻū ʻana ma mua o ʻelua mau pads a i ʻole nā ​​tampons i kēlā me kēia hola no ʻelua hola a ʻoi aku paha (no nā wahine).

He aha nā nīnau e pono iaʻu e nīnau aku i kaʻu kauka?

Eia kekahi mau nīnau hiki iā ʻoe ke nīnau i kāu kauka:

• He maʻi hoʻoilina anei koʻu hemahema Factor VII a i ʻole ua ulu aʻe ma hope?
• He aha ke ʻano o ka lāʻau lapaʻau e pono ai iaʻu?
• ʻEhia ka lōʻihi e pono ai iaʻu e mālama ʻia?
• He aha nā hopena ʻaoʻao e pono iaʻu e makaʻala i ka wā o ka mālama ʻana?
• He aha nā hoʻololi o ke ʻano o ka nohona e pono ai iaʻu e hana e mālama ai i koʻu kūlana?

Ua like anei ka hemahema o Factor VII me ka 'hemophilia'?

ʻO ka hemahema o Factor VII a me ka hemophilia he mau maʻi koko hoʻoilina pili, akā kaʻawale. Hoʻokumu ʻia ka hemahema o Factor VII e ka hemahema o ka protein Factor VII (7), ʻoiai ʻo ka hemophilia A , ke ʻano maʻamau o ka hemophilia, i hoʻokumu ʻia e ka hemahema o ka protein Factor VIII (8).

ʻOi aku ka maʻamau o ka Hemophilia A ma mua o ka hemahema o ka factor VII, e hoʻopilikia ana ma kahi o hoʻokahi i loko o 100,000 mau kānaka. ʻOi aku ka maʻamau i nā kāne .

Akā ʻo ka mea nui loa, hiki i kēia mau kūlana ʻelua ke hoʻoulu i ke kahe koko nui, no laila pono e mālama pono ʻia me ke kōkua o ke kauka.

Nā mea nui loa e hoʻomanaʻo

ʻAʻohe ala e pale aku ai i ka hānau ʻia ʻana me ka hemahema o ka maʻi clotting koko factor VII. Akā, ʻaʻole ia manaʻo ʻaʻole hiki iā ʻoe (a i ʻole kāu pēpē) ke ola i kahi ola olakino a ikaika me kēia maʻi. Hiki i kāu hematologist ke kōkua iā ʻoe e pale a hoʻokele i ke kahe ʻana o ke koko. Hiki iā ia ke kōkua iā ʻoe e hoʻomohala i kahi hoʻolālā no ka mea e hana ai inā nui kou kahe ʻana o ke koko. Mai hopohopo, hiki iā ʻoe ke ola me ka holomua me kēia maʻi.

ʻO ka hemahema o ka Factor VII, ke kāpili ʻana o ke koko, ke kahe koko ʻana, nā maʻi hoʻoilina, ka maʻi o Alexander, ke kauka koko, nā maʻi koko