Ðe nɔnɔme aɖe hã le ŋuwò si me ne ètsoe sue aɖe gɔ̃ hã la, ʋu medzudzɔna oa? (Nu VII ƒe Gbɔdzɔgbɔdzɔ) .

Ðe nèsena le ɖokuiwò me ɣeaɖewoɣi be exɔa ɣeyiɣi didi hafi lã sue aɖe gɔ̃ hã dzudzɔ ʋudodoa? Alo ɖeko nètrɔna zua blɔ eye nèxɔa abi? Ðewohĩ susu aɖe le megbe na nusiawo si ŋu míenya nu tsoe o. Emae nye nusi Factor VII ƒe anyimanɔmanɔ nye.

Nukae nye Nu VII ƒe Gbɔdzɔgbɔdzɔ?

Kpuie ko la, Factor VII ƒe anyimanɔmanɔ nye domenyiŋusẽfianu ƒe nɔnɔme si me wò ʋu metsi nyuie o, si fia be medzudzɔa ʋu si dona kabakaba o. Protein ƒomevi vovovowo le míaƒe ʋu me siwo woyɔna be ‘ʋu ƒe sisi ƒe nuwɔna’ alo ‘ʋu ƒe sisi ƒe nuwɔna’ . Factor VII nye protein mawo dometɔ ɖeka. Protein siawo katã wɔa dɔ aduadu kple wò ʋumenugbagbevi dzĩwo wɔa ʋu ƒe sisi be wòadzudzɔ ʋudodo ne èxɔ abi. Dɔ sia le abe gli tutu kple anyikpe kple siminti ene. Eyata ne protein vevi sia si woyɔna be Factor VII mele wò ŋutilã me o, alo ne mewɔa dɔ nyuie o la, exɔa ɣeyiɣi didi hafi wò ʋu natsi wu alesi wòle dzɔdzɔmee. Esia tae nèxɔa abi eye ʋu dona le ŋuwò bɔbɔe wu ame bubuwo ɖo.

Ŋkɔ bubu siwo wotsɔ na esiae nye Alexander ƒe dɔléle kple proconvertin ƒe anyimanɔmanɔ .

Aleke gbegbee factor VII ƒe anyimanɔmanɔ sia bɔe?

Le nyateƒe me la, esia nye nɔnɔme si mebɔ o . Le xexeame katã la, edzea ame abe ɖeka le 500,000 ɖesiaɖe me dzi . Ʋu ƒe sisi ƒe dɔléle geɖe li siwo ƒe dome wonyi siwo ɖɔktawo buna be "siwo mebɔ o." Factor VII ƒe anyimanɔmanɔ nye dɔléle siawo siwo mebɔ o dometɔ ɖeka.

Nukae nye esia ƒe dzesiwo?

Factor VII ƒe anyimanɔmanɔ ate ŋu ana ʋu nado le ame ŋu ɣeyiɣi didi eye wòaxɔ abi bɔbɔe. Dzesi veviawo dometɔ aɖewoe nye:

• Ʋu si le dodom le aɖuɖɔtoewo me.
• Ʋu si dona le ŋɔti me (epistaxis) , kple esesẽ be woatɔ te wo.
• Le nyɔnuwo gome la, ʋu si dona vevie le dzinukpɔkpɔ me (menorrhagia).
• Ʋu le aɖuɖɔ me (hematuria) alo ʋu le aɖuɖɔ me.
• Ʋu si nɔa dodom atraɖii le abixɔxɔ, amekoko, alo vidzidzi megbe.
• Ʋu si dona le ememe le ŋutilãa me (le kpɔɖeŋu me, le dɔgbo ƒe dɔwɔwɔ me, lãkusiwo, lãmekawo, ƒunukpeƒewo).
• Ʋu si dona le tagbɔ me hã ate ŋu adzɔ. Esia nye nusi ŋu nya ku ɖo vie wu.
• Ʋu si dona le eɖokui si dzɔna ne ʋudodo dze egɔme kpata le susu aɖeke manɔmee.

Dzesi siawo ƒe sesẽme kple ɣeyiɣi si wodzena la toa vovo tso ame ɖeka gbɔ yi bubu gbɔ. Le kpɔɖeŋu me, woate ŋu akpɔ vidzĩ si ŋu Factor VII ƒe dɔmawɔmawɔ nyuie le vevie le edziɣi. Gake ɖewohĩ womakpɔ dɔlélea le ame aɖe si ƒe dzesiwo mesẽ o ŋu o vaseɖe esime wòtsi.

Nukae hea Factor VII ƒe anyimanɔmanɔ vɛ?

Togbɔ be domenyiŋusẽfianu ƒe dɔléle koŋue nye esia hã la , mɔ aɖe hã li si dzi nɔnɔme sia ate ŋu ato adzɔ le agbe me.

Ne wodzi wò kple factor VII ƒe anyimanɔmanɔ , efia be ènyi domenyiŋusẽfianu ƒe tɔtrɔ, alo nusi gblẽ le wò F7 domenyiŋusẽfianu ŋu ƒe dome. Domenyiŋusẽfianu siae kpɔa alesi wò ŋutilã wɔa factor VII dzi. Be factor VII ƒe anyimanɔmanɔ nadze ŋuwò la, ele be nànyi domenyiŋusẽfianu ƒe tɔtrɔ sia ƒe dome tso dzila ŋutɔŋutɔ eveawo siaa gbɔ . Woyɔa esia be autosomal recessive inheritance . Ne dzila eveawo siaa ƒe tɔtrɔ sia le wo ŋu la, mɔnukpɔkpɔ anɔ wo si be woadzi vi si me factor VII ƒe anyimanɔmanɔ le.

Woyɔa Factor VII ƒe anyimanɔmanɔ si dona le ɣeyiɣi aɖe megbe be ‘acquired factor VII deficiency’ . Nu geɖe li siwo ate ŋu ahe esia vɛ:

• Aklãdɔ: Wò aklãe wɔa Factor VII protein. Ne aklãdɔ sesẽ aɖe le ŋuwò la, ɖewohĩ wò aklã mate ŋu awɔ dɔ sia nyuie o.
• Vitamin K ƒe anyimanɔmanɔ: Vitamin K le vevie na ŋutilãa be wòawɔ Factor VII. Ne mèkpɔe wòsu o la, àte ŋu axɔ Factor VII ƒe gbɔdzɔgbɔdzɔ.
• Atike siwo nana ʋu mekɔna o: Nɔnɔme sia ate ŋu ado mo ɖa hã ne wozã atike siwo nana ʋu mekɔna o abe Warfarin (Coumadin®) ene .
• Ʋumenugbagbeviɣidɔ kple kansa: Woka nya ta be Nu VII ƒe anyimanɔmanɔ le amesiwo ŋu dɔlélewo abe aplastic anemia kple ʋumenugbagbeviɣidɔ ene le ŋu .

Nukawoe nye kuxi siwo dona tso Factor VII ƒe anyimanɔmanɔ me?

Le nɔnɔme sesẽwo me la, Factor VII ƒe anyimanɔmanɔ ate ŋu ahe lãmesẽkuxi bubuwo vɛ ne womedae o. Le kpɔɖeŋu me:

• Ʋusɔgbɔdɔ: Ne ʋu geɖe bu le ŋutilãa me la, wò ʋumenugbagbevi dzĩwo ƒe xexlẽme ate ŋu aɖiɖi ŋutɔ.
• Ƒunukpeƒewo ƒe gbegblẽ: Ne ʋu le dodom le ƒunukpeƒe aɖe me la, lãkusi si le afima ate ŋu avuvu eye ƒunukpeƒea mate ŋu abɔbɔ alo aɖɔ ɖo nyuie o.
• Ʋumenugbagbeviɣidɔ: Ʋumenugbagbeviɣidɔ nye ʋu si ƒo ƒu ɖe wò ŋutigbalẽ te. Ne ʋumenugbagbeviɣidɔ gã aɖe do la, ate ŋu aƒoe ɖe ŋutinu siwo te ɖe wo ŋu nu eye wòahe vevesese ƒe dzesiwo vɛ.

Vevietɔ wu la, ʋu si dona vevie ate ŋu ade ame ƒe agbe afɔku me ne womewɔ atike nɛ o. Esia tae wòle vevie be nàdi atikewɔlawo ƒe aɖaŋuɖoɖo ne ʋua medzudzɔ o.

Aleke wokpɔa Factor VII ƒe anyimanɔmanɔ dzea sii?

Wò ɖɔkta abia wò tso wò dzesiwo kple nenye be ʋu ƒe dodo ƒe dɔléle le wò ƒomea me dometɔ aɖe ŋu hã. Wokpɔa Factor VII ƒe anyimanɔmanɔ to ʋudodokpɔ siwo me wokpɔa alesi wò ʋu ƒoa xlãe nyuie me.

Dodokpɔ siawo dometɔ aɖewoe nye:

• Prothrombin time (PT/INR) dodokpɔ: Esia nɔa te ɖe ʋu ƒe sisi ƒe nusi woyɔna be prothrombin ƒe dɔwɔwɔ dzi dzidzea ɣeyiɣi didi si wòxɔna hafi ʋu natsi. Ne factor VII ƒe anyimanɔmanɔ le ŋuwò la, exɔa ɣeyiɣi didi wu alesi wòle be ʋu natsi.
• Partial thromboplastin time (PTT) dodokpɔ: Esia dzidzea ɣeyiɣi si wòxɔna hafi ʋu natsi le ʋu ƒe sisi ƒe nusiwo menye prothrombin o ƒe dɔwɔwɔ nu. Zi geɖe la, dodokpɔ sia me tsonu sɔna le amesi ŋu factor VII ƒe dɔmawɔmawɔ nyuie le gome.
• Factor VII dodokpɔ: Dodokpɔ sia ɖe alesi Factor VII protein la le dɔ wɔme fia.

Aleke wokpɔa Factor VII ƒe anyimanɔmanɔ gbɔe?

Zi geɖe la, atikewɔwɔ bia be woatsɔ factor VII si wɔa dɔ nyuie akpe ɖe wò ʋu ŋu. Anɔ eme be àwɔ dɔ kple ɖɔkta si bi ɖe ʋudɔlélewo me, si woyɔna be ʋuŋutinunyala , si atso nya me le atike si nèhiã kple ɣeyiɣi didi si nàhiãe ŋu.

Atikewɔwɔmɔnu vevi geɖewo li:

• Recombinant factor VIIa (NovoSeven®): United States ƒe Nuɖuɖu Kple Atike Ŋuti Dɔwɔƒe (FDA) da asi ɖe atike sia dzi be woatsɔ ada factor VII ƒe anyimanɔmanɔ. Dzɔdzɔmeŋutinunyalawoe wɔe le nudokpɔƒe. Esia fia be, to vovo na atike bubu siwo wozãna tsɔ ɖɔa li factor VII la, wometsɔa ʋu si amewo na la wɔe o.
• Prothrombin complex concentrates (PCC): Nu ene siwo naa ʋumenugbagbeviɣidɔ, siwo dome factor VII hã le, siwo woɖe tso ʋu si wona me la sɔ gbɔ ɖe atikewɔwɔ sia me.
• Fresh frozen plasma (FFP): Woate ŋu ana plasma hã wò to ʋuka me be wòakpe ɖe ŋuwò nàgbugbɔ wò Factor VII fiasewo atu.

Atikewɔwɔ bubuwo:

• Atike siwo tsia fibrinolytic nu: Atike siawo wɔa dɔ to ʋuka siwo ƒona le wò ŋutilã me la nu tsotso me. Wokpena ɖe ame ŋu be ʋu nagado le eme fũ o to ʋuka siwo le wò ŋutilã me xoxo la takpɔkpɔ me. Kpɔɖeŋu aɖewoe nye atike siwo woyɔna be aminocaproic acid kple tranexamic acid .
• Vidzidzinutsitikewo:Vidzidzitike ƒomevi aɖewo xea mɔ na ʋu si dona le ame ŋu vevie le dzinukpɔkpɔ me.
• Vitamin K kpeɖeŋutɔ alo atike dodo: Ne vitamin K ƒe anyimanɔmanɔ gbɔe wò dɔlélea tso la, vitamin K geɖe xɔxɔ ate ŋu akpe ɖe wò ŋutilã ŋu wòawɔ factor VII geɖe wu.

Nukawoe nye atikewɔwɔa ƒe kuxiwo/nugbegblẽ le ame ŋu?

Dɔdamɔnu siwo tsɔa factor VII kpena ɖe ʋua ŋu ate ŋu ahe kuxiwo vɛ ɣeaɖewoɣi. Gake ele vevie be nàɖo ŋku edzi be wò ɖɔkta nya afɔku siawo eye wòle afɔ aɖewo ɖem be yeaxe mɔ ɖe wo nu.

Kuxi siwo ate ŋu ado tso Factor VII ƒe atikewɔwɔ me ate ŋu anye:

• Ʋu ƒe sisi si gblẽa nu le ame ŋu: Ne wotsɔ factor VII kpe ɖe eŋu la, ate ŋu ana ʋu ƒe sisi si ate ŋu axe ʋukawo nadzi ɖe edzi. Wò ɖɔkta alé ŋku ɖe wò atike ƒe agbɔsɔsɔme ŋu be wòaɖe afɔku sia dzi akpɔtɔ.
• Dɔlékuiwo ƒe amedzidzedze: Ne wole ʋu si wotsɔ na ame xɔm la, afɔku nɔa eme ɣesiaɣi be dɔlékui siwo gblẽa nu le ame ŋu (e.g., dɔlékuiwo alo dɔlékuiwo) ate ŋu anɔ ʋua me. Ðɔktawo léa ŋku ɖe ʋumenu siwo wozãna le ʋu me ŋu nyuie be woaxe mɔ ɖe esia nu.
• Dodo na ame: Ðewohĩ ʋumenugbagbevi dzĩ si wotsɔ na wò la ƒe nulɔdɔ anɔ ŋuwò.
• Plasma ƒe agba si gbɔ eme: Nu VII gblẽna kabakaba akpa eye woɖenɛ le wò ʋu me. Be nàxe mɔ ɖe esia nu la, ɖewohĩ ahiã be nàzã ʋumenugbagbeviɣidɔ gbogbo aɖe be factor VII si sɔ nanɔ wò ŋutilã me. Esia ate ŋu ana ʋumenugbagbeviɣi nadzi ɖe edzi le wò lãmenugbagbeviwo me. Nusiwo ʋumenugbagbeviɣi ƒe agbɔsɔsɔ fũ ate ŋu agblẽ le ame ŋu ate ŋu anye ʋusɔgbɔdɔ kple lãkusiwo ƒe vuvu (aklã me ʋuʋu) .

Nukae mate ŋu akpɔ mɔ na ne Factor VII ƒe gbɔdzɔgbɔdzɔ le ŋunye?

Nu VII ƒe anyimanɔmanɔ nye nɔnɔme si nɔa ame ƒe agbemeŋkekewo katã me . Togbɔ be atike aɖeke meli o hã la, àte ŋu awɔ dɔ kple wò ɖɔkta akpɔ egbɔ.

Kuxi gãtɔ siwo le dɔlélea ƒe sesẽme nyanya me dometɔ ɖekae nye be womate ŋu agblɔ dzesiawo ƒe sesẽme ɖi le alesi wò Factor VII ƒe agbɔsɔsɔ le sue nu o. Ke boŋ ɖɔktawo wɔa dɔ kple amesiwo ŋu gbɔdzɔgbɔdzɔ sia le ɖekaɖeka. Wò nukpɔsusu anɔ te ɖe nu siwo le etɔxɛ na wò dzi, abe wò nuteƒekpɔkpɔ, nusi he gbɔdzɔgbɔdzɔa vɛ, alesi nèwɔa nu ɖe ​​atikewɔwɔ ŋui, kple alesi ʋu si dona le ŋuwò nu sẽna zi geɖe dzi.

Aleke malé be na ɖokuinye?

Ele vevie be nàgblɔ wò nɔnɔmea na lãmesẽdɔwɔla siwo katã daa dɔ na wò. Ne factor VII ƒe dɔmawɔmawɔ nyuie le viwò ŋu la, gblɔe na amesiame si kpɔa edzi (nufialawo kple ŋkeke ɖeka dzikpɔlawo hã le eme).

Bia wò ɖɔkta tso nu siwo nàte ŋu awɔ atsɔ alé be na ɖokuiwò gbesiagbe ŋu. Ate ŋu akafu nusiwo le abe:

• Ƒo asa na dɔwɔna siwo me afɔku le ŋutɔ:Àte ŋu aƒo asa na nuwɔna siwo naa abixɔxɔ ƒe afɔkua dzina ɖe edzi (e.g., kadodo kple amewo ƒe kamedefefewo).
• Ƒo asa na atike aɖewo: Ðewohĩ ahiã be nànɔ adzɔge tso atike siwo nana wò ʋu naɖiɖi (e.g., aspirin , NSAIDs , SSRI antidepressants ). Àdi hã be yeaƒo asa na nuɖuɖu siwo me tɔmelãmi, vitamin E, kple turmeric le, elabena esiawo hã ate ŋu ana ʋu nado ɖe edzi.
• Dzra wò atikewɔwɔ ƒe ɖoɖoa ƒe kɔpi ɖo: Ele vevie be nàdzra nuŋlɔɖi siwo tso wò ʋuŋutinunyala gbɔ ku ɖe alesi wòle be lãmesẽdɔwɔlawo nawɔ atike na wò ne nɔnɔme kpata aɖe do mo ɖa ŋu.
• Na domenyiŋusẽfianu ŋuti aɖaŋuɖoɖo alo dodokpɔ: Hafi nàdze agbagba adzi vi la, àte ŋu aƒo nu kple domenyiŋusẽfianu ŋuti aɖaŋuɖola aɖe atsɔ akpɔ afɔku si le viwòa ŋu be wòanyi factor VII ƒe anyimanɔmanɔ ƒe dome.

Ɣekaɣie wòle be makpɔ nye ɖɔkta?

Ne wò alo viwòa de dzesi dzesi aɖewo be ʋu le dodom atraɖii alo blɔ nɔa eŋu enuenu la, yi ɖɔkta gbɔ. Ʋu si dona le ŋɔti me kple blɔ ɣeaɖewoɣi sɔ. Gake ne wodzɔ wu alesi wònɔna ɖaa la, ate ŋu anye ʋukuxi aɖe ƒe dzesi.

Ɣekaɣie wòle be mayi Atikewɔwɔ Kpata ƒe Dɔwɔƒe (ETU) ?

Ne wò ʋu si le dodom nye afɔku na wò lãmesẽ la, yi xɔ si me wowɔa dɔ kpata le. Di dzesiwo abe esiawo ene:

• Ʋu si dona le ŋɔti me si nɔa anyi gaƒoƒo ɖeka alo esi wu nenema.
• Tsidede pad alo tampon siwo wu eve le gaƒoƒo ɖeka me gaƒoƒo eve alo esi wu nenema (na nyɔnuwo).

Nya kawoe wòle be mabia nye ɖɔkta?

Nya aɖewo siwo nàte ŋu abia wò ɖɔktae nye esi:

• Ðe nye Factor VII ƒe anyimanɔmanɔ nye domenyinu loo alo ɖe wòva dzɔ emegbea?
• Atikewɔwɔ ka ƒomevie mehiã?
• Ɣeyiɣi didi kae wòahiã be mawɔ atike nam?
• Nu gbegblẽ kawoe wòle be manya nu tsoe le atikewɔwɔ me?
• Agbenɔnɔ ƒe tɔtrɔ kawoe wòle be mawɔ be makpɔ nye nɔnɔmea gbɔ?

Ðe Factor VII ƒe anyimanɔmanɔ sɔ kple ‘ʋumenugbagbeviɣidɔ’?

Factor VII ƒe anyimanɔmanɔ kple ʋusɔgbɔdɔ nye ʋumenugbagbeviɣidɔ eve siwo do ƒome, gake woto vovo, siwo ƒe dome wonyi. Protein Factor VII (7) ƒe anyimanɔmanɔ gbɔe Factor VII ƒe anyimanɔmanɔ tsona, gake hemophilia A , si nye ʋusɔgbɔdɔ ƒomevi si bɔ wu, ya tso protein Factor VIII ƒe anyimanɔmanɔ gbɔ (8).

Ʋusɔgbɔdɔ A bɔ wu factor VII ƒe anyimanɔmanɔ, eye wòdzea ame abe ɖeka le 100,000 ɖesiaɖe me dzi. Ebɔ ɖe ŋutsuwo hã me wu .

Gake nusi le vevie wue nye be nɔnɔme siawo evea siaa ate ŋu ana ʋu nado fũu akpa, eyata ehiã be woakpɔ wo gbɔ nyuie to ɖɔkta ƒe kpekpeɖeŋu me.

Nu vevitɔ kekeake siwo dzi wòle be woaɖo ŋkui

Mɔ aɖeke meli si dzi woato axe mɔ ɖe vidzidzi kple ʋu ƒe sisi ƒe dɔléle si nye factor VII ƒe anyimanɔmanɔ nu o. Gake ema mefia be wò (alo viwòa) màte ŋu anɔ agbe si me lãmesẽ le, si me dzo le kple nɔnɔme sia o. Wò ʋuŋutinunyala ate ŋu akpe ɖe ŋuwò nàxe mɔ ɖe ʋudodo nu ahakpɔ egbɔ. Ate ŋu akpe ɖe ŋuwò hã be nàwɔ ɖoɖo ɖe nusi nàwɔ ne ʋu le dodom le ŋuwò fũu akpa ŋu. Mègatsi dzi o, àte ŋu anɔ agbe dzidzedzetɔe kple nɔnɔme sia.

` Factor VII Deficiency, Ʋu ƒe Ʋuʋu, Ʋudodo, Domenyinu, Alexander ƒe Dɔléle, Ʋuŋutinunyala, Ʋudɔlélewo