So wowɔ tebea bi nso a sɛ wotwa ade ketewaa bi mpo a, mogya nnyae? (Ade VII a Ɛho Hia) .

So ɛtɔ mmere bi a wote nka sɛ egye bere tenten ansa na wɔatwitwa ketewaa bi mpo ansa na mogya atɔ wo? Anaasɛ wodan bruu na woyɛ akuru kɛkɛ? Ebia ntease bi wɔ saa nneɛma yi akyi a yennim ho hwee. Ɛno ne nea Factor VII a enni hɔ no yɛ.

Dɛn ne Factor VII Deficiency?

Sɛ yɛbɛka no tiawa a, Factor VII a enni nipadua mu no yɛ awosu mu tebea a ɛma wo mogya ntumi nkɔ yiye, a ɛkyerɛ sɛ ennyae mogya a ɛretu ntɛmntɛm. Protein ahorow pii wɔ yɛn mogya mu a wɔfrɛ no ‘mogya a ɛma mogya tu’ anaa ‘mogya a ɛma mogya tu’ . Factor VII yɛ protein a ɛte saa no mu biako. Saa protein ahorow yi nyinaa ne wo mogya mu nkwammoaa a ɛma mogya tu no bom yɛ mogya a ɛbɛtɔ na ama mogya atɔ bere a woapira no. Saa adeyɛ yi te sɛ nea wɔde birikisi ne semɛnte resi ɔfasu. Enti, sɛ wunni protein a ɛho hia a wɔfrɛ no Factor VII yi a ɛdɔɔso wɔ wo nipadua mu, anaasɛ ɛnyɛ adwuma yiye a, egye bere tenten ansa na wo mogya no ayɛ mmerɛw sen sɛnea ɛte daa. Eyi nti na ɛnyɛ den sɛ wobɛpirapira na mogya atɔ wo sen afoforo no.

Edin afoforo a wɔde frɛ eyi ne Alexander yare ne proconvertin a enni nipadua mu .

Saa ade VII a enni hɔ yi abu so dɛn?

Eyi yɛ tebea a ɛntaa nsi ankasa . Wɔ wiase nyinaa no, ɛka nnipa bɛyɛ 500,000 biara mu biako . Mogya a ɛma mogya tu a wonya fi awo mu no pii wɔ hɔ a nnuruyɛfo bu no sɛ “ɛho yɛ na.” Factor VII a enni nipadua mu no yɛ nyarewa a wɔntaa nhu yi mu biako.

Dɛn ne eyi ho sɛnkyerɛnne?

Factor VII a enni nipadua no mu no betumi ama mogya atɔ no akyɛ na ayɛ mmerɛw sɛ ɔbɛpira. Nsɛnkyerɛnne atitiriw no bi ne:

• Mogya a ɛretu wɔ anom.
• Hwene mu mogya (epistaxis) , ne ɛyɛ den sɛ wobesiw ano.
• Wɔ mmea fam no, mogya a ɛtɔ kɛse bere a wɔrekɔ brayɛ (menorrhagia).
• Mogya a ɛwɔ nsu mu (hematuria) anaa mogya a ɛwɔ nsu mu.
• Mogya a ɛkɔ so tu wɔ opira, oprehyɛn, anaa awo akyi.
• Mogya a ɛba nipadua no mu (sɛ nhwɛso no, wɔ yafunu mu, ntini ahorow, ntini ahorow, nkwaa mu).
• Mogya a ɛba atiridiinini mu nso betumi aba. Eyi yɛ aniberesɛm kɛse kakra.
• Mogya a ɛba ara kwa ba bere a mogya fi ase mpofirim a ntease biara nnim no.

Ɛsono sɛnea sɛnkyerɛnne ahorow yi mu yɛ den ne bere a ɛda adi no gu ahorow wɔ nnipa biara mu. Sɛ nhwɛso no, wobetumi ahu akokoaa a Factor VII a enni no kɛse no wɔ n’awo mu. Nanso, ebia wɔrenhu obi a ɔwɔ yare no ho sɛnkyerɛnne a emu nyɛ den kosi sɛ obenyin.

Dɛn na ɛde Factor VII a enni hɔ ba?

Ɛwom sɛ eyi yɛ awosu mu yare titiriw de , nanso ɔkwan bi nso wɔ hɔ a saa tebea yi betumi afa so aba wɔ nkwa mu.

Sɛ wɔwoo wo a factor VII a enni mu , ɛkyerɛ sɛ woanya nkwaadɔm mu nsakrae, anaasɛ sintɔ bi wɔ wo F7 awosu mu abɔde mu . Saa awosu mu abɔde yi na ɛhwɛ sɛnea wo nipadua no yɛ factor VII no so. Sɛ wubenya factor VII a enni hɔ a, ɛsɛ sɛ wunya saa awosu mu nsakrae yi fi awofo a wɔwoo wo baanu no nyinaa hɔ . Wɔfrɛ eyi sɛ autosomal recessive inheritance . Sɛ awofo baanu no nyinaa wɔ saa mutation yi a, wɔwɔ hokwan bɛyɛ 25% sɛ wɔbɛwo abofra a factor VII a enni mu.

Factor VII deficiency that develops over time is called 'acquired factor VII deficiency' . Nneɛma pii wɔ hɔ a ebetumi de eyi aba:

• Mmɛn mu yare: Wo mmerɛbo na ɛyɛ Factor VII protein no. Sɛ wowɔ mmerɛbo mu yare a emu yɛ den a, ebia wo mmerɛbo rentumi nyɛ adwuma yi yiye.
• Vitamin K a enni nipadua no mu: Vitamin K ho hia ma nipadua no na ama ayɛ Factor VII. Sɛ woannya nea ɛdɔɔso a, wubetumi anya Factor VII a enni hɔ.
• Nnuru a ɛma mogya yɛ mmerɛw: Saa tebea yi nso betumi aba bere a wɔde nnuru a ɛma mogya yɛ mmerɛw te sɛ Warfarin (Coumadin®) redi dwuma no.
• Mogya mu nkwammoaa mu yare ne kokoram: Wɔabɔ amanneɛ sɛ Factor VII a enni nipadua no mu wɔ nnipa a wɔwɔ nyarewa te sɛ aplastic anemia ne leukemia mu .

Dɛn ne nsɛnnennen a ɛwɔ Factor VII a enni nipadua mu no mu?

Wɔ tebea horow a emu yɛ den mu no, Factor VII a enni nipadua no mu no betumi de akwahosan ho haw afoforo aba sɛ wɔansa a. Nhwɛsoɔ:

• Mogya a ɛtɔ kɔ nipadua mu: Sɛ mogya pii yera fi nipadua no mu a, wo mogya mu nkwammoaa kɔkɔɔ dodow betumi akɔ fam kɛse.
• Nkwaa a ɛsɛe: Sɛ mogya tu wɔ nkwaa bi mu a, ntini a ɛwɔ hɔ no betumi asɛe na nkwaa no rentumi nkotow anaasɛ ɛnteɛ yiye.
• Hematomas: Hematoma yɛ mogya a ɛboaboa ano wɔ wo honam ani. Sɛ hematoma kɛse bi ba a, ebetumi de nhyɛso aba akwaa a ɛbɛn hɔ no so na ama wɔanya yare no ho sɛnkyerɛnne a ɛyɛ yaw.

Nea ɛho hia sen biara no, sɛ wɔansa mogya a ɛretu denneennen a, ebetumi de ne nkwa ato asiane mu. Ɛno nti na ɛho hia sɛ wohwehwɛ nnuruyɛfo afotu sɛ mogya no annyae a.

Ɔkwan bɛn so na wohu Factor VII a enni nipadua no mu?

Wo duruyɛfo bebisa wo wo yare no ho sɛnkyerɛnne ne sɛ ebia obi wɔ w’abusua mu a ɔwɔ mogyatɔw ho abakɔsɛm anaa. Wɔde mogya mu nhwehwɛmu a ɛkyerɛ sɛnea wo mogya tu yiye na ɛkyerɛ Factor VII a enni hɔ.

Saa sɔhwɛ ahorow yi bi ne:

• Prothrombin bere (PT/INR) sɔhwɛ: Eyi gyina mogya mu ade a ɛma mogya tu a wɔfrɛ no prothrombin dwumadi so na ɛkyerɛ bere tenten a egye ansa na mogya atɔ. Sɛ wowɔ factor VII a enni wo mu a, egye bere tenten sen sɛnea ɛte daa ansa na mogya atɔ.
• Partial thromboplastin time (PTT) sɔhwɛ: Eyi gyina nneɛma a ɛma mogya tu a ɛnyɛ prothrombin dwumadi so susuw bere a egye ansa na mogya atɔ. Mpɛn pii no, nea efi sɔhwɛ yi mu ba no yɛ nea ɛfata wɔ obi a factor VII a enni no mu.
• Factor VII nhwehwɛmu: Saa sɔhwɛ yi kyerɛ sɛnea Factor VII protein no reyɛ adwuma.

Ɔkwan bɛn so na wɔsa factor VII a enni nipadua no mu?

Mpɛn pii no, nea ɛka ayaresa ho ne sɛ wode factor VII a ɛyɛ adwuma yiye bɛka wo mogya ho. Ɛda adi sɛ wo ne oduruyɛfo bi a ne ho akokwaw wɔ mogya mu nyarewa ho, a wɔfrɛ no mogya ho ɔbenfo , a obesi ayaresa a wuhia ne bere tenten a wubehia no ho gyinae.

Akwan atitiriw pii wɔ hɔ a wɔfa so sa yare:

• Recombinant factor VIIa (NovoSeven®): U.S. Aduan ne Nnuru Dwumadibea (FDA) apene so sɛ wɔmfa nsa factor VII a enni nipadua no mu. Nyansahufo na wɔyɛ no wɔ aduruyɛdan mu. Eyi kyerɛ sɛ, nea ɛnte sɛ ayaresa afoforo a wɔde si factor VII ananmu no, wɔmfa mogya a wɔde mae a efi nnipa hɔ na ɛyɛ.
• Prothrombin complex concentrates (PCC): Nneɛma anan a ɛma mogya tu, a factor VII ka ho, a wogye fi mogya a wɔde mae mu no dɔɔso wɔ saa ayaresa yi mu.
• Fresh frozen plasma (FFP): Wobetumi nso de plasma ama wo wɔ ntini mu na aboa ma wɔasan akyekye wo Factor VII sotɔɔ ahorow no.

Ayaresa afoforo:

• Nnuru a ɛko tia fibrinolytic: Nnuru yi yɛ adwuma denam mogya a ɛtwetwe a ɛba wo nipadua mu no a esiw ano no so. Wɔboa ma mogya mmoroso nsiw denam mogya a ɛtwetwe a ɛwɔ wo nipadua mu dedaw no a wɔbɔ ho ban no so. Nhwɛso ahorow bi ne nnuru a wɔfrɛ no aminocaproic acid ne tranexamic acid .
• Nnuru a wɔde siw awo ano:Nnuru ahorow bi a wɔde siw awo ano siw mogya a ɛbɛtɔ kɛse bere a obi rekɔ brayɛ no ano.
• Vitamin K a wɔde ma anaa nnuru a wɔde twitwiw nipadua no mu: Sɛ vitamin K a wunni na ɛde wo tebea no ba a, vitamin K pii a wubenya no betumi aboa wo nipadua no ma ayɛ factor VII pii.

Dɛn ne nsɛnnennen/nsunsuanso bɔne a ɛwɔ ayaresa no mu?

Ɛtɔ mmere bi a, ayaresa a ɛde factor VII ka mogya no ho no betumi de nsɛnnennen aba. Nanso, ɛho hia sɛ wokae sɛ wo duruyɛfo nim asiane ahorow yi na ɔreyɛ nneɛma bi de asiw ano.

Nsɛnnennen a ebetumi aba wɔ Factor VII ayaresa mu no bi ne:

• Mogya a ɛtɔ a ɛyɛ hu: Sɛ wode factor VII ka ho a, ebetumi ama asiane a ɛwɔ hɔ sɛ mogya a ɛbɛtɔ a ebetumi asiw mogya ntini no ayɛ kɛse. Wo duruyɛfo bɛhwɛ nnuru dodow a wode di dwuma no so na ama asiane yi so atew.
• Ɔyare mmoawa a wɔde wɔn ho hyɛ mu: Sɛ wonya mogya a wɔde ama no a, asiane wɔ hɔ bere nyinaa sɛ wobenya ɔyare mmoawa a epira (sɛ nhwɛso no, mmoawa anaa mmoawa) wɔ mogya no mu. Nnuruyɛfo de ahwɛyiye hwehwɛ mogya mu nneɛma mu na wɔasiw eyi ano.
• Mogya a wɔde ma: Ebia wo ho yɛ wo hyew wɔ plasma a wɔde ama no ho.
• Plasma a ɛboro so: Factor VII no sɛe ntɛmntɛm dodo na woyi fi wo mogya mu. Sɛnea ɛbɛyɛ na woasiw eyi ano no, ebia wubehia plasma pii na ama factor VII a ɛdɔɔso akɔ so atra wo nipadua mu. Eyi betumi ama plasma a ɛwɔ wo nipadua mu no akɔ soro. Nea ebetumi afi mogya mu duru a ɛboro so mu aba no bi ne mogya mmoroso ne ahurututu a ɛho ahonhon (ahurututu mu akisikuru) .

Sɛ mewɔ Factor VII a enni hɔ a, dɛn na metumi ahwɛ kwan?

Factor VII a enni nipadua mu no yɛ tebea a ɛkɔ so wɔ nkwa nna nyinaa mu . Ɛwom sɛ aduru biara nni hɔ de, nanso wubetumi ne wo duruyɛfo ayɛ adwuma de adi ho dwuma.

Nsɛnnennen akɛse biako a ɛwɔ sɛnea yare no mu bɛyɛ den a wobɛkyerɛ mu ne sɛ wontumi nnyina sɛnea wo Factor VII dodow no sua so nkyerɛ sɛnea sɛnkyerɛnne ahorow no mu yɛ den. Mmom no, nnuruyɛfo ne nnipa a wɔwɔ saa sintɔ yi yɛ adwuma ankorankoro. W’adwene begyina nneɛma a ɛyɛ soronko ma wo so, te sɛ osuahu a wunyae, nea ɛde sintɔ no bae, sɛnea woyɛ w’ade wɔ ayaresa ho, ne sɛnea wo mogya taa mu yɛ den no so.

Mɛyɛ dɛn ahwɛ me ho?

Ɛho hia sɛ woka wo tebea no ho asɛm kyerɛ akwahosan ho adwumayɛfo a wɔsa wo yare nyinaa. Sɛ wo ba no nni factor VII a, ka kyerɛ obiara a ɔhwɛ no (a akyerɛkyerɛfo ne adwumayɛfo a wɔhwɛ no da biara ka ho).

Bisa wo duruyɛfo no nneɛma a wubetumi ayɛ de ahwɛ wo ho da biara da. Ebia ɔbɛkamfo nneɛma te sɛ:

• Kwati dwumadi ahorow a asiane kɛse wom:Ebia wobɛpɛ sɛ wokwati dwumadi ahorow a ɛma asiane a ɛwɔ hɔ sɛ wubepira no yɛ kɛse (e.g., agumadi a wɔde di nkitaho).
• Kwati nnuru bi: Ebia ebehia sɛ wotwe wo ho fi nnuru a ɛma wo mogya yɛ mmerɛw (e.g., aspirin , NSAIDs , SSRI nnuru a ekum adwenemhaw ). Ebia wobɛpɛ nso sɛ wokwati nnuru a wɔde ka aduan ho a mpataa mu ngo, vitamin E, ne turmeric wom, efisɛ eyinom nso betumi ama mogya a ɛbɛtɔ wo no ayɛ kɛse.
• Fa w’ayaresa nhyehyɛe no bi sie: Ɛho hia sɛ wode nkrataa a efi wo mogya ho oduruyɛfo hɔ a ɛfa sɛnea ɛsɛ sɛ akwahosan ho adwumayɛfo sa wo yare bere a asɛm bi asi ntɛm no sie.
• Nya awosu ho afotu anaa nhwehwɛmu: Ansa na wobɛbɔ mmɔden sɛ wobɛwo akokoaa no, wubetumi ne awosu ho ɔfotufo bi akasa de ahwɛ asiane a ɛwɔ hɔ sɛ wo ba no benya factor VII a enni awo mu.

Bere bɛn na ɛsɛ sɛ mekɔ me duruyɛfo nkyɛn?

Sɛ wo anaa wo ba hu sɛnkyerɛnne biara a ɛkyerɛ sɛ mogya retu no daa anaasɛ ɔtaa yɛ bruu a, kɔ wo duruyɛfo nkyɛn. Mogya a ɛba hwene mu ne bruu a ɛtɔ mmere bi a ɛyɛ ade a ɛfata. Nanso sɛ ɛba sen sɛnea ɛtaa ba a, ebetumi ayɛ mogya ho haw ho sɛnkyerɛnne.

Bere bɛn na ɛsɛ sɛ mekɔ Emergency Treatment Unit (ETU) ?

Sɛ mogya a ɛretu wo no de w’akwahosan to asiane mu a, kɔ baabi a wɔhwɛ wɔn a wɔwɔ ayaresa a egye ntɛmpɛ mu. Hwehwɛ nsɛnkyerɛnne te sɛ eyinom:

• Hwene mu mogya a ɛtra hɔ dɔnhwerew biako anaa nea ɛboro saa.
• Fa pads anaa tampons bɛboro abien a wɔde gu nsu mu dɔnhwerew biara nnɔnhwerew abien anaa nea ɛboro saa (ma mmea).

Nsɛm bɛn na ɛsɛ sɛ mibisa me duruyɛfo?

Nsɛmmisa bi a wubetumi abisa wo duruyɛfo ni:

• So Factor VII a minni me no fi awosu mu anaasɛ ɛbaa akyiri yi?
• Ayaresa bɛn na mihia?
• Bere tenten ahe na ɛsɛ sɛ meyɛ ayaresa?
• Nneɛma bɔne bɛn na ɛsɛ sɛ mehwɛ bere a wɔreyɛ ayaresa no?
• Nsakrae bɛn na ɛsɛ sɛ meyɛ wɔ asetra mu na ama matumi adi me tebea no ho dwuma?

So Factor VII a enni nipadua no mu ne ‘hemophilia’ yɛ pɛ?

Factor VII a enni nipadua mu ne hemophilia yɛ mogya mu nyarewa abien a ɛfa ho, nanso ɛsono emu biara, a wonya fi awo mu. Factor VII a enni nipadua mu no fi protein Factor VII (7) a enni nipadua no mu, bere a hemophilia A , hemophilia a abu so sen biara no fi protein Factor VIII a enni nipadua no mu (8).

Hemophilia A abu so sen factor VII a enni nipadua no mu, na ɛka nnipa bɛyɛ 100,000 biara mu biako. Ɛtaa ba mmarima nso so kɛse .

Nanso nea ɛho hia sen biara ne sɛ, saa tebea abien yi nyinaa betumi ama mogya atu dodo, enti ɛsɛ sɛ wodi ho dwuma yiye denam oduruyɛfo mmoa so.

Nneɛma a ɛho hia sen biara a ɛsɛ sɛ yɛkae

Ɔkwan biara nni hɔ a wɔbɛfa so asiw awo a wɔde mogya a ɛma mogya tu mu yare factor VII a enni hɔ no ano. Nanso ɛno nkyerɛ sɛ wo (anaa wo ba) ntumi mfa saa tebea yi ntra ase a ahoɔden wom na ɛyɛ nnam. Wo mogya ho ɔyaresafo betumi aboa wo ma woasiw mogya a ɛbɛtɔ ano na woadi ho dwuma. Obetumi nso aboa wo ma woayɛ nhyehyɛe a wode bɛyɛ nea sɛ mogya tu wo dodo a. Mma ɛnhaw wo, wubetumi de tebea yi atra ase yiye.

` Factor VII Deficiency, Mogya a Ɛtwetwe, Mogya a Ɛtu, Awosu mu Nyarewa, Alexander Yare, Mogya Ho Ɔbenfo, Mogya Nyarewa