Ingabe nawe unesifo lapho ngisho nokusikeka okuncane kungayeki ukuphuma kwegazi? (Ukuntuleka Kwesici VII)

Ingabe ngezinye izikhathi uzizwa sengathi kuthatha isikhathi eside ngisho nokusikeka okuncane ukuyeka ukopha? Noma umane uphenduke ube luhlaza okwesibhakabhaka futhi ube nemihuzuko? Mhlawumbe kunesizathu salezi zinto esingazi ngaso. Yilokho ukuntuleka kwe-Factor VII.

Kuyini Ukuntuleka Kwe-Factor VII?

Kalula nje, ukuntuleka kwe-Factor VII kuyisimo sofuzo lapho igazi lakho lingavimbi kahle, okusho ukuthi aliyeki ukopha ngokushesha. Kunezinhlobo eziningana zamaprotheni egazini lethu ezibizwa ngokuthi 'izinto ezivimba igazi' noma 'izinto ezivimba igazi' . I-Factor VII ingenye yamaprotheni anjalo. Wonke lawa maprotheni asebenza ndawonye nama-platelet akho ukwakha i-blood clot ukuze kumiswe ukopha igazi uma ulimala. Le nqubo ifana nokwakha udonga ngezitini nosimende. Ngakho-ke, uma ungenayo eyanele le phrotheni ebalulekile ebizwa ngokuthi i-Factor VII emzimbeni wakho, noma uma ingasebenzi kahle, kuthatha isikhathi eside ukuba igazi lakho livimbe kunokujwayelekile. Yingakho ukhuhla futhi wopha igazi kalula kunabanye.

Amanye amagama alokhu yisifo sika-Alexander kanye nokuntuleka kwe-proconvertin .

Kuvame kangakanani lokhu kuntuleka kwe-factor VII?

Empeleni lesi yisimo esingavamile . Emhlabeni wonke, sithinta cishe umuntu oyedwa kwabangu-500,000 . Kunezinkinga eziningi zokwakheka kwegazi ezizuzwe njengefa odokotela abazibiza ngokuthi "ezingavamile." Ukuntuleka kwe-Factor VII kungenye yezifo ezivame kakhulu kulezi ezingavamile.

Ziyini izimpawu zalokhu?

Ukuntuleka kwe-Factor VII kungabangela ukopha isikhathi eside kanye nokulunywa kalula. Izimpawu eziyinhloko zifaka:

• Izinsini eziphuma igazi.
• Ukopha ekhaleni (epistaxis) , kanye nobunzima bokukuvimba.
• Kwabesifazane, ukopha okukhulu ngesikhathi sokuya esikhathini (i-menorrhagia).
• Igazi emchameni (i-hematuria) noma igazi esitulweni.
• Ukopha okuqhubekayo ngemva kokulimala, ukuhlinzwa, noma ukubeletha.
• Ukopha kwangaphakathi emzimbeni (isibonelo, ohlelweni lokugaya ukudla, izicubu, imisipha, amalunga).
• Ukopha ngaphakathi kobuchopho nakho kungenzeka. Lokhu kuyingozi kakhulu.
• Ukopha okuzenzekelayo kwenzeka lapho ukopha kuqala ngokuzumayo ngaphandle kwesizathu esibonakalayo.

Ubunzima balezi zimpawu nokuthi zivela nini kuyahlukahluka kuye ngomuntu nomuntu. Isibonelo, ingane enesifo esibi kakhulu se-Factor VII ingatholakala lapho izalwa. Kodwa-ke, umuntu onezimpawu ezincane angase angatholakali aze akhule.

Yini ebangela ukuntuleka kwe-Factor VII?

Nakuba lesi yisifo esizuzwa njengefa , kukhona nendlela lesi simo esingavela ngayo empilweni.

Uma uzalwa unesifo se-factor VII , kusho ukuthi uzuze i-mutation, noma i-defect, ku -F7 gene yakho. Lesi sakhi silawula indlela umzimba wakho odala ngayo i-factor VII. Ukuze uthuthukise ukuntuleka kwe-factor VII, kufanele uthole lesi shintsho sezakhi zofuzo kubazali bobabili bemvelo . Lokhu kubizwa ngokuthi i-autosomal recessive inheritance . Uma bobabili abazali benalesi shintsho, banethuba elingama-25% lokuba nomntwana onesifo se-factor VII.

Ukuntuleka kwe-Factor VII okuthuthuka ngokuhamba kwesikhathi kubizwa ngokuthi 'ukuntuleka kwe-factor VII okutholakale' . Kunezimbangela eziningana ezingaba khona zalokhu:

• Isifo Sesibindi: Isibindi sakho senza iphrotheni i-Factor VII. Uma unesifo sesibindi esibi kakhulu, isibindi sakho singase singakwazi ukwenza lo msebenzi kahle.
• Ukuntuleka kwe-Vitamin K: I-Vitamin K ibalulekile emzimbeni ukuze wakhe i-Factor VII. Uma ungakutholi okwanele, ungaba nokuntuleka kwe-Factor VII.
• Imithi yokunciphisa igazi: Lesi simo singavela nalapho kusetshenziswa imithi yokunciphisa igazi efana ne -Warfarin (Coumadin®) .
• Izinkinga zamaseli egazi kanye nomdlavuza: Ukuntuleka kwe-Factor VII kubikwe kubantu abanezifo ezifana ne -aplastic anemia kanye ne-leukemia .

Yiziphi izinkinga zokuntuleka kwe-Factor VII?

Ezimweni ezimbi kakhulu, ukuntuleka kwe-Factor VII kungaholela kwezinye izinkinga zempilo uma kungelashwa. Isibonelo:

• I-anemia: Uma kulahleka igazi eliningi emzimbeni, inani lamaseli akho abomvu egazi lingehla kakhulu.
• Umonakalo wamalunga: Uma kukhona ukopha ngaphakathi kwamalunga, izicubu lapho zingase ziguge futhi amalunga angase angakwazi ukugoba noma ukuqondisa kahle.
• Ama-Hematoma: I-hematoma iqoqo lamahlule egazi ngaphansi kwesikhumba sakho. Uma kwakheka i-hematoma enkulu, ingafaka ingcindezi ezithweni eziseduze futhi ibangele izimpawu ezibuhlungu.

Okubaluleke kakhulu, ukopha okukhulu kungaba yingozi empilweni uma kungelashwa. Yingakho kubalulekile ukufuna iseluleko sezokwelapha uma ukopha kungayeki.

Kuhlonzwa kanjani ukuntuleka kwe-Factor VII?

Udokotela wakho uzokubuza ngezimpawu zakho nokuthi ngabe kukhona yini emndenini wakho onomlando wezinkinga zokopha. Ukuntuleka kwe-Factor VII kutholakala ngokuhlolwa kwegazi okuhlola ukuthi igazi lakho ligobile kangakanani.

Lezi zivivinyo zifaka:

• Ukuhlolwa kwesikhathi se-prothrombin (PT/INR): Lokhu kulinganisa ukuthi kuthatha isikhathi esingakanani ukuthi igazi lijiye ngokusekelwe emsebenzini we-blood clotting factor ebizwa ngokuthi i-prothrombin. Uma unesifo se-factor VII, kuthatha isikhathi eside kunokujwayelekile ukuthi igazi lijiye.
• Ukuhlolwa kwesikhathi se-thromboplastin esingaphelele (i-PTT): Lokhu kulinganisa isikhathi esithathwayo ukuze igazi lijiye ngokusekelwe emsebenzini wezinto ezijiya igazi ngaphandle kwe-prothrombin. Imiphumela yalolu vivinyo ivame ukuba yinto evamile kumuntu onesifo se-factor VII.
• Ukuhlolwa kwe-Factor VII: Lokhu kuhlolwa kukhombisa ukuthi iphrotheni ye-Factor VII isebenza kanjani.

Ukwelashwa kokuntuleka kwe-factor VII kunjani?

Ukwelashwa kuvame ukuhilela ukufaka i-factor VII esebenza kahle egazini lakho. Cishe uzosebenzisana nodokotela ochwepheshe bezinkinga zegazi, obizwa ngokuthi i-hematologist , ozonquma ukuthi yikuphi ukwelashwa okudingayo nokuthi uzoludinga isikhathi esingakanani.

Kunezindlela eziningana zokwelapha eziyinhloko:

• I-Recombinant factor VIIa (NovoSeven®): Lo muthi uvunyelwe yi-US Food and Drug Administration (FDA) ukwelapha ukuntuleka kwe-factor VII. Wenziwa ososayensi elabhorethri. Lokhu kusho ukuthi, ngokungafani nezinye izindlela zokwelapha ezifaka i-factor VII esikhundleni sazo, awenziwa ngegazi elinikelwe ngabantu.
• Ama-concentrates e-Prothrombin complex (PCC): Lokhu kwelashwa kuqukethe amazinga aphezulu ezici ezine zokujiya kwegazi, okuhlanganisa ne-factor VII, ezithathwe egazini elinikelwe.
• I-plasma eqandisiwe entsha (FFP): Unganikwa futhi i-plasma ngemithambo ukuze usize ekwakheni kabusha izitolo zakho ze-Factor VII.

Ezinye izindlela zokwelapha:

• Ama-antifibrinolytic agents: Le mithi isebenza ngokuvimba amahlule egazi akheka emzimbeni wakho ukuthi angancibiliki. Asiza ekuvimbeleni ukopha ngokweqile ngokuvikela amahlule egazi asevele esemzimbeni wakho. Izibonelo zifaka phakathi imithi ebizwa ngokuthi i-aminocaproic acid kanye ne-tranexamic acid .
• Amaphilisi okuvimbela inzalo:Amanye amaphilisi okuvimbela inzalo avimbela ukopha okukhulu ngesikhathi sokuya esikhathini.
• Izithasiselo noma imijovo ye-Vitamin K: Uma isimo sakho sibangelwa ukuntuleka kwe-vitamin K, ukuthola i-vitamin K eningi kungasiza umzimba wakho ukuthi wakhe i-factor VII eningi.

Yiziphi izinkinga/imiphumela emibi yokwelashwa?

Ukwelashwa okwengeza i-factor VII egazini ngezinye izikhathi kungabangela izinkinga. Kodwa-ke, kubalulekile ukukhumbula ukuthi udokotela wakho uyazi ngalezi zingozi futhi uthatha izinyathelo zokuzivimbela.

Izinkinga ezingaba khona zokwelashwa kwe-Factor VII zingafaka:

• Amahlule egazi ayingozi: Ukwengeza i-factor VII kungandisa ingozi yamahlule egazi angavimba imithambo yegazi. Udokotela wakho uzoqapha umthamo wemithi yakho ukuze anciphise le ngozi.
• Ukuchayeka ezifweni ezibangela izifo: Uma uthola igazi elinikelwe, kuhlale kunengozi yokuchayeka ezifweni eziyingozi (isib., amagciwane noma amabhaktheriya) egazini. Odokotela bahlola ngokucophelela imikhiqizo yegazi ukuze bavimbele lokhu.
• Ukusabela kokumpontshelwa igazi: Ungase ube nokusabela kokungezwani komzimba ne-plasma enikelwe.
• Ukwanda kwe-plasma: I-Factor VII ihlukaniswa ngokushesha kakhulu futhi isuswe egazini lakho. Ukuze uvimbele lokhu, ungadinga inani elikhulu le-plasma ukuze ugcine i-factor VII eyanele emzimbeni wakho. Lokhu kungabangela ukwanda kwe-plasma ohlelweni lwakho. Imiphumela yokwanda kwe-plasma ingafaka umfutho wegazi ophezulu kanye nokuvuvukala kwamaphaphu (i-pulmonary edema) .

Yini engingayilindela uma nginokuntuleka kwe-Factor VII?

Ukuntuleka kwe-Factor VII kuyisimo esihlala isikhathi eside . Nakuba kungekho ikhambi, ungasebenzisana nodokotela wakho ukuze usilawule.

Enye yezinselele ezinkulu ekunqumeni ukuthi lesi sifo sizoba sibi kangakanani ukuthi akunakwenzeka ukubikezela ubukhali bezimpawu ngokusekelwe ekutheni amazinga akho e-Factor VII aphansi kangakanani. Esikhundleni salokho, odokotela basebenza nabantu abanalesi simo sokuntuleka kwegazi ngokomuntu ngamunye. Umbono wakho uzoncika ezintweni ezihlukile kuwe, njengokuhlangenwe nakho kwakho, ukuthi yini ebangele ukuntuleka kwegazi, indlela osabela ngayo ekwelashweni, nokuthi ukopha kwakho kuvame ukuba kubi kangakanani.

Ngizinakekela kanjani?

Kubalulekile ukutshela bonke abahlinzeki bezempilo abakwelaphayo ngesimo sakho. Uma ingane yakho inokuntuleka kwe-factor VII, tshela noma ubani oyinakekelayo (kufaka phakathi othisha kanye nabasebenzi benkulisa).

Buza udokotela wakho ngezinto ongazenza ukuze uzinakekele nsuku zonke. Angase akuncome izinto ezifana nalezi:

• Gwema imisebenzi eyingozi kakhulu:Ungase ufune ukugwema imisebenzi eyandisa ingozi yokulimala (isb., imidlalo yokuxhumana).
• Gwema imithi ethile: Kungase kudingeke ukuthi uhlale kude nemithi enciphisa igazi lakho (isib. i-aspirin , ama-NSAID , ama-antidepressant e-SSRI ). Ungase futhi ufune ukugwema izithako eziqukethe uwoyela wezinhlanzi, i-vitamin E, kanye ne-turmeric, njengoba lokhu kungandisa ukopha.
• Gcina ikhophi yohlelo lwakho lokwelashwa: Kubalulekile ukugcina amadokhumenti avela kudokotela wakho wegazi mayelana nendlela okufanele uphathwe ngayo ochwepheshe bezempilo uma kwenzeka isimo esiphuthumayo.
• Thola ukwelulekwa noma ukuhlolwa kwezakhi zofuzo: Ngaphambi kokuba uzame ukuba nomntwana, ungakhuluma nomeluleki wezakhi zofuzo ukuze ahlole ingozi yengane yakho yokuthola ukuntuleka kwe-factor VII.

Kufanele ngimbone nini udokotela wami?

Uma wena noma ingane yakho nibona noma yiziphi izimpawu zokopha okuqhubekayo noma ukuba luhlaza okwesibhakabhaka okuvamile, bona udokotela wakho. Ukopha ekhaleni kanye nokuba luhlaza okwesibhakabhaka ngezikhathi ezithile kuyinto evamile. Kodwa uma kwenzeka ngaphezu kokuvamile, kungaba uphawu lwenkinga yegazi.

Kufanele ngiye nini e-Emergency Treatment Unit (ETU) ?

Uma ukopha kwakho kusongela impilo yakho, iya egumbini lezimo eziphuthumayo. Bheka izimpawu ezinjengalezi:

• Ukopha ekhaleni okuhlala ihora noma ngaphezulu.
• Ukumanzisa ama-pad noma ama-tampon angaphezu kwamabili ngehora amahora amabili noma ngaphezulu (kwabesifazane).

Yimiphi imibuzo okufanele ngiyibuze udokotela wami?

Nazi ezinye imibuzo ongayibuza udokotela wakho:

• Ingabe ukuntuleka kwami ​​kwe-Factor VII kuyifa noma kwavela kamuva?
• Hlobo luni lokwelashwa engiludingayo?
• Kuzodingeka ngenze ukwelashwa isikhathi esingakanani?
• Yimiphi imiphumela emibi okufanele ngiyiqaphele ngesikhathi sokwelashwa?
• Yiziphi izinguquko zendlela yokuphila okudingeka ngizenze ukuze ngiphathe isimo sami?

Ingabe ukushoda kwe-Factor VII kuyafana 'ne-hemophilia'?

Ukuntuleka kwe-Factor VII kanye ne-hemophilia kuyizifo ezimbili ezihlobene, kodwa ezihlukene, ezizuzwe njengefa. Ukuntuleka kwe-Factor VII kubangelwa ukuntuleka kweprotheni i-Factor VII (7), kuyilapho i-hemophilia A , uhlobo oluvame kakhulu lwe-hemophilia, kubangelwa ukuntuleka kweprotheni i-Factor VIII (8).

I-Hemophilia A ivame kakhulu kunokuntuleka kwe-factor VII, ithinta cishe umuntu oyedwa kubantu abayi-100,000. Ivame kakhulu nakwabesilisa .

Kodwa into ebaluleke kakhulu ukuthi zombili lezi zimo zingabangela ukopha ngokweqile, ngakho-ke zidinga ukuphathwa kahle ngosizo lukadokotela.

Izinto ezibaluleke kakhulu okufanele uzikhumbule

Ayikho indlela yokuvimbela ukuzalwa unesifo sokuwa kwegazi esibizwa ngokuthi yi-blood clotting disorder VII deficiency. Kodwa lokho akusho ukuthi wena (noma ingane yakho) ngeke niphile impilo enempilo nesebenzayo ngalesi simo. Udokotela wakho wegazi angakusiza ukuvimbela nokuphatha ukopha. Angakusiza futhi ukuthi wakhe uhlelo lokuthi wenzeni uma wopha kakhulu. Ungakhathazeki, ungaphila ngempumelelo nalesi simo.

Ukuntuleka Kwesici VII, Ukuvinjelwa Kwegazi, Ukopha, Izifo Ezizuzwayo, Isifo Sika-Alexander, Isazi Segazi, Izifo Zegazi