I-Von Willebrand Disease yiyona nto ixhaphakileyo yokufa kwegazi , echaphazela malunga ne-1% yabemi.
Yintoni iVon Willebrand factor?
I-Von Willebrand into yiprotheyini yegazi ebophezela kwi-VIII (i-coagulation factor). Xa inkalo yeVIII ingaboshwa kwi-Von Willebrand isifo, idibanisa kalula. I-Von Willebrand isifo sinceda iiplatelet ukuba zihambelane nakwiindawo zokulimala.
Ziziphi Iimpawu?
Ezinye izigulane azize zenze nayiphi na igazi eliphambili. I-Von Willebrand Disease idibene ne:
- Ukululaza
- Iipumphaza ezide
- Ukuhlanjululwa kwizinsini
- Ukuphuma ngexesha elide emva kokuphuma kwezinyo okanye ukulimala
- Igazi kwi-stool
- I-Hematuria (igazi kumchamo)
- I-Menorrhagia (ukuphuma kwexesha ngokugqithiseleyo)
- Ukuphuma kwegazi kunye kunye ne-soft tissue bleeding kungenzeka kwiifom ezinzima ezifana ne- hemophilia
Iintlobo zeVon Willebrand Disease
- Uhlobo 1: Olu luhlobo oluqhelekileyo, olwenzeka malunga nama-75% wezigulane. Kugqityiwe kwiintsapho kwi-autosomal fashion ebonisa ukuba umzali omnye kufuneka achaphazeleke ukuba adlule eso sifo. Oku kubangelwa kwinqanaba eliphantsi kweVon Willebrand. Ukuhlamba kunokuba ngumnxeba.
- Uhlobo lwesi-2: Uhlobo lwesi-2 luvela xa i-Von Willebrand ingasebenzi ngokuqhelekileyo.
- Uhlobo lwe-2A: Lwenziwe ngaphantsi kwefowuni. I-moderate ukuya kwi-moderation yegazi.
- Uhlobo lwe-2B: Unqamle kwi-autosomal fashion. I-moderate ukuya kwi-moderation yegazi. I-Thrombocytopenia (inani elisezantsi leplatelet) aliqhelekanga.
- Uhlobo lwe-2M: Uhlobo olungaqhelekanga ludlulile kwi-autosomal fowder.
- Uhlobo lwe-2N: Uhlobo olungaqhelekanga ludlulile kwi-autosomal fashion. Oku kuthetha isigulane esichaphazelekayo sifumana iikopi ezimbini zomguqulo oguquka, omnye kumzali ngamnye. Amazinga e-Factor VIII angaba phantsi kakhulu. Ukuhlamba kunokuba nzima kwaye kunokudideka nge-hemophilia A.
- Uhlobo lwe-3: Olu luhlobo olunqabileyo lwe-Von Willebrand Disease. Iyadlula kwi-autosomal fashion. Ukuhlamba kunokuba nzima. Izigulane ezinolu hlobo zinomlinganiselo ophantsi kakhulu okanye ezingekho kwiVon Willebrand. Oku kubangela ukusilela kwi Factor VIII kunye nokuphuma kwamanzi.
- Kufunyenwe: Le fomu yesifo seVon Willebrand ibangelwa ngenye into efana nomhlaza, ukuphazamiseka komzimba, ukukhubazeka kwemizimba (njengento ye-septric defect, aortic stenosis), amayeza okanye i-hypothyroidism.
I-Von Willebrand Disease Diagnised?
Okokuqala, ugqirha wakho kufuneka unqabise ukuba unesifo sokuphuma kwegazi esekelwe kwiimpawu ezingentla. Ukuba namanye amalungu entsapho abaneempawu ezifanayo ukwandisa ukusola kwi-Von Willebrand Disease, ngakumbi ukuba bonke abesilisa nabasetyhini bachaphazelekayo (ngokungafani ne- hemophilia echaphazela kakhulu amadoda).
I-Von Willebrand Disease ifunyaniswa ngokwenza iphaneli yomsebenzi wegazi ejongene nomlinganiselo we-Von Willebrand into egazini kunye nomsebenzi wayo (umsebenzi we-ristocetin cofactor). Ngenxa yokuba iintlobo ezininzi zeVon Willebrand Izifo zingabangela ukunciphisa i-Factor VIII, amanqanaba ale protheni yokuqhawula ayathunyelwa. I-Von Willebrand multimers, ejongene nesakhiwo se-Von WIllebrand kunye nendlela idilizwe ngayo, kubaluleke ngakumbi ekuhloleni iSifo sesi-2.
Ziyintoni Iingonyango zeVon Willebrand Disease?
Izigulane ezichaphazelekayo zingenakuze zifune unyango.
- I-DDAVP: I-DDAVP (ebizwa ngokuba yi-desmopressin) yi-hormone yokwenziwa komsebenzi eyenziwa nge-spray spray (okanye ngezinye ii-IV). Le hormone inceda umzimba ukukhulula i-Von Willebrand into egcinwe kwimithambo yegazi.
- I-Von Willebrand Factor replacement: Efana ne-substitution factor used in hemophilia, i-infonon ye-Von Willebrand into inokunikezelwa ukukhusela okanye ukuphatha ukuphuma kwamanzi. Ezi mveliso ziqulethe i-Factor VIII ngokunjalo.
- I-Antifibrinolytics: La mayeza (amagama e-brand u-Amicar noLysteda), anikezwa ngomlomo, anceda ukuzinzisa ukwakha i-clot. Ezi zinokubaluleka ngokukhethekileyo kwiimpumpo zamanzi, ukuphuma emlonyeni nokuphuma kwimihla.
- Ulwaphulo-mzimba: Kwiimfazana ezine-Von Willebrand Disease kunye nokuphuma kwexesha elinzima, ukukhulelwa kwe-hormonal ezifana neepilisi zokulawula ukuzalwa okanye izixhobo zangaphakathi zingaphantsi ukusetyenziswa.