I-Von Willebrand Disease iyisifo esivame kakhulu sokugaya igazi , esithinta cishe u-1% wabantu.
Yini iVon Willebrand factor?
I-Von Willebrand factor yi-protein yegazi ehlangene ne-VIII (i-coagulation factor). Uma i-factor VIII ingeyona i-Von Willebrand isifo, ihlehla kalula. Isifo se-Von Willebrand sisiza futhi amaplatelet ukunamathela kumasayithi okulimala.
Ziyini Izimpawu?
Ezinye iziguli azike zithole noma yikuphi ukuphapha okuphawulekayo. I-Von Willebrand Disease ihlotshaniswa ne:
- Ukulimala kalula
- Izinkinga ze-noseble eside
- Ukuhlwitha kusuka ezinsini
- Ukusuka isikhathi eside ngemva kokukhipha izinyo noma ukulimala
- Igazi esitokisini
- I-Hematuria (igazi emcinini)
- I-Menorrhagia (ukuphuma ngokweqile kokuya esikhathini)
- Ukuphuma kwegazi okuhlangene noma okulula kungase kwenzeke ngamafomu aqinile afana ne- hemophilia
Izinhlobo ze-Von Willebrand Disease
- Thayipha 1: Leli fomu elivame kakhulu, elivela cishe ezigulini ezingama-75%. Idluliselwa emindenini ngendlela ehamba phambili ye-autosomal okusho ukuthi umzali oyedwa kufanele adutshwe ukuze adlule lesi sifo. Lokhu kubangelwa ezingeni eliphansi kunezinga elijwayelekile le-Von Willebrand factor. Ukukhipha igazi kungaba nomnene kunzima.
- Thayipha 2: Uhlobo lwe-2 lwenzeka uma i-Von Willebrand factor ingasebenzi ngendlela evamile.
- Thayipha i-2A: Yehla ngendlela ye-autosomal evelele. Ukuphuza okukhulu ngokwezinga eliphansi.
- Thayipha i-2B: Yehle ngendlela ye-autosomal evelele. Ukuphuza okukhulu ngokwezinga eliphansi. I-Thrombocytopenia (isibalo esiphansi se-plateslet) asivamile.
- Thayipha i-2M: Uhlobo olungavamile luye lwadlula ngendlela ye-autosomal evelele.
- Thayipha i-2N: Uhlobo olungavamile olwedlule luye lwadlula ngendlela ye-autosomal ngokweqile. Lokhu kusho ukuthi isiguli esithintekile sithola amakhophi amabili wegesi eguquliwe, elilodwa kumzali ngamunye. Izinga lama-Factor VIII zingaba phansi kakhulu. Ukukhipha igazi kungaba nzima futhi kungahle kudideke nge-hemophilia A.
- Uhlobo lwe-3: Lolu hlobo olungajwayelekile lwe-Von Willebrand Disease. Idluliselwa ngendlela ye-autosomal evelele. Ukweqa kungaba nzima kakhulu. Iziguli ezinale hlobo zinenani eliphansi ngokweqile noma alikho i-Von Willebrand factor. Lokhu kubangela ukungabi khona kwe-Factor VIII nokukhishwa okuphawulekayo.
- Kutholakale: Leli fomu le-Von Willebrand isifo sibangelwa enye into efana nomdlavuza, ukuphazamiseka kwe-autoimmune, ukukhubazeka komzimba (njenge-ventricular septal defect, aortic stenosis), imithi noma i-hypothyroidism.
I-Von Willebrand Izifo Zitholakala Kanjani?
Okokuqala, udokotela wakho kumele asolise ukuthi unesifo sokuphuma kwegazi esekelwe ezimpawu ezingenhla. Ukuba namanye amalungu omndeni anezimpawu ezifanayo ukwandisa ukusola nge-Von Willebrand Disease, ikakhulukazi uma bobabili abesilisa nabesifazane besithintekile (ngokungafani ne- hemophilia ethinta kakhulu abesilisa).
I-Von Willebrand Disease itholakala ngokwenza iphaneli yomsebenzi wegazi elibukeka kokubili inani le-Von Willebrand factor egazini kanye nomsebenzi walo (umsebenzi we-ristocetin cofactor). Ngenxa yokuthi izinhlobo eziningana ze-Von Willebrand Izifo zingabangela ukunciphisa i-Factor VIII, amazinga ale phrotheni lokuqothula nawo athunyelwa. I-Von Willebrand multimers, ebuka isakhiwo se-Von WIllebrand factor nokuthi idilizwe kanjani, ibalulekile ngokukhethekile ekuhloleni uhlobo lweSifo sesi-2.
Yiziphi Izindlela Zokwelashwa Kwe-Von Willebrand Disease?
Iziguli ezithintekayo zingase zingadingi ukwelashwa.
- I-DDAVP: I-DDAVP (ebizwa ngokuthi i-desmopressin) iyinhlangano ye-hormone yokwenziwa eyenziwa nge-spray (noma nge-IV). Le hormone isiza umzimba ukuthi ukhulule i-Von Willebrand factor egcinwe emithanjeni yegazi.
- I-Von Willebrand Factor esikhundleni: Njengoba kufana nesimo esiphezulu esisetshenziselwa i-hemophilia, i-infusions ye-Von Willebrand factor inganikezwa ukuvimbela noma ukwelapha ukwelapha. Le mikhiqizo nayo iqukethe i-Factor VIII kanye.
- I-Antifibrinolytics: Le mithi (amagama omama u-Amicar noLysteda), ngokuvamile anikwa ngomlomo, asize ekuqiniseni ukwakheka kwe-clot. Lezi zingasiza ikakhulukazi nge-nosebleeds, ukuphuma komlomo nokuphuma esikhathini.
- Izinkinga zokubeletha: Kwabesifazane abanesifo se-Von Willebrand kanye nokukhulelwa kwesisu esinzima, ukukhulelwa kwe-hormonal njengamaphilisi okulawula ukubeletha noma amadivaysi e-intrauterine angasetshenziswa ukunciphisa / ukuyeka ukuphuma.