Unewo here dambudziko rekuti kunyange kukuvara kudiki hakuregi kubuda ropa? (Factor VII Deficiency)

Dzimwe nguva unonzwa sekunge zvinotora nguva yakareba kuti kunyange ganda diki rimire kubuda ropa here? Kana kuti unongoshanduka kuita bhuruu wobva wakuvara? Pamwe pane chikonzero chiri shure kwezvinhu izvi chatisingazive. Ndizvo zvinoreva kushomeka kweFactor VII.

Chii chinonzi Factor VII Deficiency?

Zvichitaurwa zviri nyore, kushomeka kweFactor VII chirwere chemajini umo ropa rako risingagwambe zvakanaka, zvichireva kuti harimire kubuda ropa nekukurumidza. Kune mhando dzakasiyana dzemapuroteni muropa redu anonzi 'blood clotting factors' kana 'coagulation factors' . Factor VII ipuroteni imwe yakadaro. Mapuroteni ese aya anoshanda pamwe chete nemaplatelet ako kugadzira blood clot kuti amise kubuda ropa kana wakuvara. Maitiro aya akafanana nekuvaka madziro nezvidhinha nesimende. Saka, kana usina puroteni iyi inokosha inonzi Factor VII yakakwana mumuviri wako, kana kuti ikasashanda zvakanaka, zvinotora nguva yakareba kuti ropa rako rigwambe kupfuura zvakajairika. Ndokusaka uchikwenya nekubuda ropa zviri nyore kupfuura vamwe.

Mamwe mazita eizvi chirwere chaAlexander uye kushomeka kweproconvertin .

Kushomeka kwechinhu ichi cheVII kwakawanda sei?

Ichi chirwere chisingawanzoitiki . Pasi rose, chinokanganisa munhu mumwe chete pavanhu 500,000 . Kune zvirwere zvakawanda zveropa zvinogarwa nhaka izvo vanachiremba vanoti "zvisingawanzoitiki." Kushaikwa kweFactor VII ndechimwe chezvirwere izvi zvisingawanzoitiki.

Ndezvipi zviratidzo zveizvi?

Kushomeka kwechinhu chinonzi factor VII kunogona kukonzera kubuda ropa kwenguva refu uye mavanga ari nyore. Zviratidzo zvikuru zvinosanganisira:

• Matadza anobuda ropa.
• Kubuda ropa mumhino (epistaxis) , uye kuoma kumisa kubuda ropa mumhino.
• Kuvakadzi, kubuda ropa rakawanda panguva yekuenda kumwedzi (menorrhagia).
• Ropa muweti (hematuria) kana ropa mutsvina.
• Kubuda ropa nguva dzose mushure mekukuvara, kuvhiyiwa, kana kusununguka.
• Kubuda ropa mukati memuviri (semuenzaniso, mudumbu, munyama, mutsandanyama, mumapfundo).
• Kubuda ropa mukati memusoro kunogonawo kuitika. Izvi zvakakomba zvishoma.
• Kubuda ropa kamwe kamwe kunoitika kana kubuda ropa kamwe kamwe kukatanga pasina chikonzero chakajeka.

Kuoma kwezviratidzo izvi uye kuti zvinoonekwa riini zvinosiyana kubva pamunhu kusvika pamunhu. Semuenzaniso, mwana ane dambudziko guru reFactor VII anogona kuonekwa pakuzvarwa. Zvisinei, munhu ane zviratidzo zvisina kunyanya kuoma anogona kusaonekwa kusvika akura.

Chii chinokonzera kushomeka kweFactor VII?

Kunyangwe ichi chirwere chinogara nhaka , panewo nzira iyo chirwere ichi chinogona kuitika nayo muhupenyu hwese.

Kana wakazvarwa uine factor VII deficiency , zvinoreva kuti wakagara nhaka mutation, kana defect, muF7 gene yako. Iri gene rinotonga mashandiro anoita muviri wako factor VII. Kuti uve nefactor VII deficiency, unofanira kugara nhaka iyi gene mutation kubva kuvabereki vese vemuviri . Izvi zvinonzi autosomal recessive inheritance . Kana vabereki vese vaine iyi mutation, vane mukana we25% wekuva nemwana ane factor VII deficiency.

Kushomeka kweFactor VII kunobuda nekufamba kwenguva kunonzi 'acquired factor VII deficiency' . Pane zvikonzero zvakawanda zvinogona kunge zviri:

• Chirwere chechiropa: Chiropa chako chinogadzira puroteni inonzi Factor VII. Kana uine chirwere chechiropa chakakomba, chiropa chako chingasakwanise kuita basa iri nemazvo.
• Kushaikwa kweVitamin K: Vitamin K inokosha kuti muviri ugadzire Factor VII. Kana ukasaiwana yakakwana, unogona kuva nekushomeka kweFactor VII.
• Mishonga inodzikisa ropa: Chirwere ichi chinogonawo kuitika kana uchishandisa mishonga inodzikisa ropa yakaita seWarfarin (Coumadin®) .
• Matambudziko emasero eropa nekenza: Kushomeka kwefactor VII kwakaonekwa muvanhu vane zvirwere zvakaita seaplastic anemia neleukemia .

Ndezvipi zvinetso zvinokonzerwa nekushaikwa kweFactor VII?

Muzviitiko zvakakomba, kushomeka kweFactor VII kunogona kukonzera mamwe matambudziko ehutano kana kukasarapwa. Semuenzaniso:

• Anemia: Kana ropa rakawanda rikabuda mumuviri, huwandu hwemasero matsvuku eropa hunogona kudzikira zvakanyanya.
• Kukuvara kwemajoini: Kana ropa richibuda mukati mejoini, nyama yacho inogona kupera uye joini inogona kusakwanisa kukotama kana kutwasuka zvakanaka.
• Hematomas: Hematoma muunganidzwa wemapundu eropa ari pasi peganda rako. Kana hematoma yakakura ikavapo, inogona kumanikidza nhengo dziri pedyo uye kukonzera zviratidzo zvinorwadza.

Chinonyanya kukosha ndechekuti kubuda ropa kwakanyanya kunogona kuisa hupenyu panjodzi kana kukasarapwa. Ndosaka zvichikosha kutsvaga rubatsiro rwechiremba kana kubuda ropa kukaramba kuchienderera mberi.

Kushomeka kweFactor VII kunoonekwa sei?

Chiremba wako achakubvunza nezvezviratidzo zvako uye kana paine munhu mumhuri yako ane nhoroondo yezvirwere zvekubuda ropa. Kushaikwa kwechinhu cheVII kunoonekwa nekuongororwa kweropa kunoongorora kuti ropa rako rakagwamba sei.

Miedzo iyi inosanganisira:

• Kuongororwa kwenguva yeprothrombin (PT/INR): Izvi zvinoyera kuti zvinotora nguva yakareba sei kuti ropa rigwambe zvichibva pakushanda kwechinhu chinogwamba ropa chinonzi prothrombin. Kana uine dambudziko refactor VII, zvinotora nguva yakareba kupfuura yakajairika kuti ropa rigwambe.
• Kuongororwa kwenguva yechikamu chethromboplastin (PTT): Izvi zvinoyera nguva inotora kuti ropa rigwambe zvichibva pane zvinoitwa nezvinhu zvinogwamba ropa kunze kweprothrombin. Mhedzisiro yekuedzwa uku inowanzova yakajairika kune munhu ane dambudziko refactor VII.
• Kuyedzwa kweFactor VII: Kuyedzwa uku kunoratidza mashandiro ari kuita puroteni yeFactor VII.

Kushomeka kwechinhu chinonzi factor VII kunorapwa sei?

Kurapa kunowanzo sanganisira kuwedzera factor VII inoshanda zvakanaka muropa rako. Ungangoshanda nachiremba ane ruzivo rwezvirwere zveropa, anonzi hematologist , uyo achasarudza kuti ndeapi marapirwo aunoda uye kuti uchaada kwenguva yakareba sei.

Kune nzira dzinoverengeka huru dzekurapa:

• Recombinant factor VIIa (NovoSeven®): Mushonga uyu wakatenderwa neUS Food and Drug Administration (FDA) kurapa kushomeka kwefactor VII. Unogadzirwa nemasayendisiti murabhoritari. Izvi zvinoreva kuti, kusiyana nedzimwe nzira dzekurapa dzinotsiva factor VII, haugadzirwe neropa rinobva kuvanhu.
• Prothrombin complex concentrates (PCC): Kurapa uku kune huwandu hwakawanda hwezvinhu zvina zvinogwamba ropa, kusanganisira factor VII, zvinotorwa kubva muropa rakapihwa.
• Plasma itsva yakaomeswa nechando (FFP): Unogonawo kupihwa plasma netsinga kuti ikubatsire kuvakazve zvitoro zvako zveFactor VII.

Mamwe marapirwo:

• Mishonga inorwisa fibrinolytic: Mishonga iyi inoshanda nekumisa magwamba eropa anoumbwa mumuviri wako kuti asanyungudike. Inobatsira kudzivirira kubuda ropa rakawanda nekudzivirira magwamba eropa ari mumuviri wako. Mienzaniso inosanganisira mishonga inonzi aminocaproic acid uye tranexamic acid .
• Mapiritsi ekudzivirira pamuviri:Mamwe marudzi emapiritsi ekudzivirira pamuviri anodzivirira kubuda ropa rakawanda panguva yekuenda kumwedzi.
• Mishonga yekuwedzera kana majekiseni eVitamin K: Kana chirwere chako chichikonzerwa nekushaikwa kweVitamin K, kuwana Vitamin K yakawanda kunogona kubatsira muviri wako kugadzira factor VII yakawanda.

Ndezvipi zvinetso/migumisiro yekurapwa?

Kurapa kunowedzera factor VII muropa dzimwe nguva kunogona kukonzera matambudziko. Zvisinei, zvakakosha kuyeuka kuti chiremba wako anoziva nezvenjodzi idzi uye ari kutora matanho ekudzivirira.

Matambudziko angangoitika ekurapa kweFactor VII anogona kusanganisira:

• Kugwamba kweropa kunokuvadza: Kuwedzera factor VII kunogona kuwedzera njodzi yekugwamba kweropa kunogona kuvhara mitsipa yeropa. Chiremba wako achatarisa mushonga wako kuti aderedze njodzi iyi.
• Kusangana nezvirwere: Pakugamuchira ropa rinobva kune mumwe munhu, pane mukana wekuwana zvirwere zvinokuvadza (semuenzaniso, mavhairasi kana mabhakitiriya) muropa. Vanachiremba vanoongorora zvinhu zveropa nokungwarira kuti vadzivise izvi.
• Maitiro ekuisirwa ropa: Ungave uine allergy kana ukapihwa plasma.
• Kuwanda kweplasma: Factor VII inopwanyika nekukurumidza uye inobviswa muropa rako. Kuti udzivise izvi, ungangoda plasma yakawanda kuti uchengetedze factor VII yakakwana mumuviri wako. Izvi zvinogona kukonzera kuwedzera kweplasma mumuviri wako. Migumisiro yekuwanda kweplasma inogona kusanganisira BP yakakwira uye kuzvimba kwemapapu (pulmonary edema) .

Chii chandingatarisira kana ndine kushaya Factor VII?

Kushaikwa kwechirwere cheFactor VII chirwere chinogara kwenguva refu . Kunyangwe pasina mushonga, unogona kushanda nachiremba wako kuti uzvigadzirise.

Chimwe chezvinhu zvikuru pakuona kuti chirwere ichi chakakomba sei ndechekuti hazvigoneke kufanotaura kuoma kwezviratidzo zvichibva pakuti Factor VII levels yako yakaderera zvakadii. Pane kudaro, vanachiremba vanoshanda nevanhu vane dambudziko iri vari vega vega. Maonero ako achaenderana nezvinhu zvakasiyana newe, zvakaita sezvakaitika kwauri, chakakonzera kushomeka kwechirwere, maitiro aunoita kana warapwa, uye kuti kubuda ropa kwako kunowanzova kwakanyanya sei.

Ndinozvitarisira sei?

Zvakakosha kuudza vese vanokupa rubatsiro rwehutano nezvechirwere chako. Kana mwana wako aine dambudziko rekushaya zvinhu zvinokwana factor VII, taurira chero ani zvake anomutarisira (kusanganisira vadzidzisi nevashandi vekurera vana).

Bvunza chiremba wako nezvezvinhu zvaunogona kuita kuti uzvitarisire zuva nezuva. Anogona kukukurudzira zvinhu zvakaita se:

• Dzivisa mabasa ane njodzi huru:Ungangoda kudzivisa mitambo inowedzera njodzi yekukuvara (semuenzaniso, mitambo yekubata munhu).
• Dzivisa mimwe mishonga: Ungangoda kurega kushandisa mishonga inoderedza ropa rako (semuenzaniso, aspirin , NSAIDs , SSRI antidepressants ). Ungangodawo kudzivisa mishonga ine mafuta ehove, vhitamini E, uye turmeric, sezvo izvi zvinogonawo kuwedzera kubuda ropa.
• Chengeta kopi yechirongwa chako chekurapwa: Zvakakosha kuchengeta magwaro kubva kuna chiremba wako weropa pamusoro pekuti unofanira kurapwa sei nenyanzvi dzehutano kana paine emergency.
• Wana mazano kana kuongororwa kwemajini: Usati waedza kuva nemwana, unogona kutaura nenyanzvi yezvemajini kuti uongorore njodzi yemwana wako yekuwana chirwere chekushaya factor VII.

Ndinofanira kuona chiremba wangu rini?

Kana iwe kana mwana wako mukaona zviratidzo zvekubuda ropa nguva dzose kana kuti kusviba kweropa nguva nenguva, onai chiremba wenyu. Kubuda ropa mumhino uye kusviba kweropa dzimwe nguva zvakajairika. Asi kana zvikaitika kupfuura zvakajairika, zvinogona kunge zviri chiratidzo chedambudziko reropa.

Ndinofanira kuenda kuEmergency Treatment Unit (ETU) riini?

Kana kubuda ropa kwako kuchikanganisa hutano hwako, enda kuchipatara che emergency. Tsvaga zviratidzo zvakaita seizvi:

• Kubuda ropa mumhino kunotora awa imwe kana kupfuura.
• Kunyorovesa mapedhi kana matamponi anopfuura maviri paawa kwemaawa maviri kana kupfuura (kuvakadzi).

Mibvunzo ipi yandinofanira kubvunza chiremba wangu?

Heano mimwe mibvunzo yaunogona kubvunza chiremba wako:

• Kushaya kwangu kweFactor VII kwakagara nhaka here kana kuti kwakazotanga pave paya?
• Rudzii rwekurapwa rwandinoda?
• Ndichatora nguva yakareba sei ndichirapwa?
• Ndezvipi zvimwe zvinokanganisa zvandinofanira kuziva panguva yekurapwa?
• Ndezvipi zvinhu zvandinofanira kuchinja muhupenyu hwangu kuti ndirarame nechirwere changu?

Kushomeka kweFactor VII kwakafanana here ne'hemophilia'?

Kushaikwa kweFactor VII nehemophilia zvirwere zviviri zvine hukama, asi zvakasiyana, zveropa. Kushaikwa kweFactor VII kunokonzerwa nekushaikwa kwepuroteni Factor VII (7), nepo hemophilia A , rudzi rwakajairika rwehemophilia, inokonzerwa nekushaikwa kwepuroteni Factor VIII (8).

Hemophilia A inowanikwa zvakanyanya kupfuura kushomeka kwefactor VII, ichibata munhu mumwe chete pavanhu 100,000. Inowanikwawo zvakanyanya kuvarume .

Asi chinonyanya kukosha ndechekuti matambudziko ese ari maviri anogona kukonzera kubuda ropa rakawanda, saka anofanira kurapwa zvakanaka nerubatsiro rwachiremba.

Zvinhu zvinonyanya kukosha zvekurangarira

Hapana nzira yekudzivirira kuzvarwa uine dambudziko rekuvhara ropa (blood clotting disorder VII deficiency). Asi izvi hazvirevi kuti iwe (kana mwana wako) haugone kurarama hupenyu hwakanaka uye hune hutano uine chirwere ichi. Chiremba wako weropa anogona kukubatsira kudzivirira uye kudzora kubuda ropa. Iye anogonawo kukubatsira kugadzira hurongwa hwezvaunofanira kuita kana ukarasa ropa rakawanda. Usazvidya moyo, unogona kurarama zvakanaka nechirwere ichi.

Kushaikwa kweFactor VII, Kuvhara Ropa, Kubuda ropa, Zvirwere Zvenhaka, Chirwere chaAlexander, Nyanzvi yeHematologist, Zvirwere zveRopa