Cutar Winebrand Vonbrand ita ce cutar ta zubar da jini ta kowa, wanda ya shafi kusan kashi 1% na yawan jama'a.
Menene Von Willebrand factor?
Von Willebrand factor shi ne furotin furotin da cewa bindiga zuwa factor VIII (wani factor coagulation). Lokacin da factor VIII ba a ɗaure shi zuwa cutar Von Willebrand ba, sai ya rushe sauƙi. Har ila yau, cutar Von Willebrand tana taimaka wa takalma don tallafawa shafuka.
Mene ne cututtuka?
Wasu marasa lafiya ba su taba yin jini ba. Von Willebrand cuta yana hade da:
- Ƙarfafawa mai sauki
- Tsayawa hanci
- Buga daga gums
- Zubar da zub da jini bayan hakocin hakori ko rauni
- Blood a cikin dutsen
- Hematuria (jini a cikin fitsari)
- Menorrhagia (matsanancin zubar jini)
- Ruwan haɗin gwiwa ko zub da jini mai laushi zai iya faruwa a manyan siffofin kama da hemophilia
Irin Von Willebrand cuta
- Nau'in 1: Wannan shi ne mafi yawan al'ada, yana faruwa a kusan 75% na marasa lafiya. An lasafta a cikin iyalai a cikin wata hanya mai mahimmanci wanda ke nufi kawai iyaye ɗaya ne kawai ya kamata a shawo kan cutar. Wannan yana haifar da ƙananan matakin matakin Von Willebrand. Tsai zai iya zama m zuwa tsanani.
- Nau'in 2: Rubutun 2 yana faruwa a lokacin da Von Willebrand factor ba ya aiki kullum.
- Rubutun 2A: Rushe a cikin ƙarancin kyauta. Hanyar da za a zubar da jini a matsakaici.
- Rubuta 2B: An sauka a cikin ƙarancin kyauta. Hanyar da za a zubar da jini a matsakaici. Thrombocytopenia (low count count) ba sababbin ba.
- Rubuta 2M: Wani nau'i wanda ba a sani ba ya sauka a cikin mafi kyawun hanzari.
- Rubuta 2N: Wani abu wanda ba a sani ba ya shige a cikin hanzari ba. Wannan yana nufin mutumin da ya kamu da cutar ya sami nau'i biyu na mahaifa, daya daga kowane iyaye. Factor VIII matakan iya zama musamman low. Ruwa zai iya zama mai tsanani kuma yana iya rikicewa da hemophilia A.
- Rubutun 3: Wannan nau'i ne irin na Von Willebrand. An sauke shi a cikin tsauri mafi rinjaye. Ruwa zai iya zama mai tsanani. Magunguna irin wannan suna da ƙananan adadi ko kuma ba a bin Von Willebrand factor. Hakanan hakan yana haifar da rashi a Factor VIII da kuma zub da jini.
- An samo: Wannan nau'i na Von Willebrand cutar ta haifar da wani abu kamar cutar ciwon daji, nakasar kamuwa da cuta, cututtuka na zuciya (kamar ventricular septal defect, aortic stenosis), magunguna ko hypothyroidism.
Yaya Yaya An gano Masifa Willebrand?
Da farko, likitanku dole ne ya kasance mai jin dadi cewa kuna da ciwon zubar da jini bisa ga alamomin bayyanar da ke sama. Samun wasu 'yan uwa tare da irin wannan alamar cutar yana ƙara damuwa ga cutar Von Willebrand, musamman idan maza da mata suna shafar (wanda ya bambanta da hemophilia wanda ya shafi maza).
Ana gano cutar ta Von Willebrand ta hanyar yin wani aikin jini wanda yake kallon duka nau'in Von Willebrand factor a cikin jini da kuma aikinsa (aiki na ristocetin cofactor). Saboda yawancin irin cutar Von Willebrand zai iya haifar da raguwa a cikin Factor VIII, an kuma aika da matakan wanzuwar furotin. Von Willebrand multimers, wanda yake duban tsarin Von WIllebrand factor da kuma yadda aka rushe, yana da mahimmanci musamman a bincikar cutar ta 2.
Menene Jiyya ga Von Willebrand cuta?
Magunguna masu rashin lafiya ba zasu buƙaci magani ba.
- DDAVP: DDAVP (wanda ake kira demopressin) wani hormone ne mai raɗaɗin da aka gudanar ta hanyar yaduwa ta hanci (ko lokaci-lokaci ta hanyar IV). Wannan hormone yana taimaka wa jiki ya saki Von Willebrand factor ajiya a cikin jini.
- Von Willebrand Factor maye gurbin: Kamar misalin factor amfani da hemophilia, infusions na Von Willebrand factor za a iya ba don hana ko bi da jini. Waɗannan samfurori sun hada da Factor VIII.
- Antifibrinolytics: Wadannan magunguna (sunayen suna Amicar da Lysteda), waɗanda aka ba da launi, sun taimaka wajen magance jini. Wadannan zasu iya zama da amfani sosai ga hanci, da zubar da bakin ciki da zubar jini.
- Magunguna: A cikin matan da ke fama da cutar Von Willebrand da zubar da jini mai tsanani, za a iya amfani da maganin rigakafi na haɗari irin na kwayoyin haihuwa ko na'urori na intrauterine don rage / dakatar da jini.