Von Willebrand Disease ndiyo inowanzobatwa nehutano hwekubuda ropa , inobata anenge 1% yevanhu.
Chii Chinonzi Von Willebrand factor?
Von Willebrand chimwe chinhu chironga cheropa chinosungiridza kuwedzera VIII (a coagulation factor). Kana chinetso VIII chisingarevi kuVon Willebrand chirwere, chinoputsika nyore nyore. Von Chirwere cheWillebrand chinobatsirawo maplatelet kunamatira kune nzvimbo dzekukuvara.
Ndezvipi Zviratidzo?
Vamwe varwere havana kumbobvira vaine chero chinhu chinokosha chinobuda ropa. Von Willebrand Disease inobatanidza ne:
- Zviri nyore kupwanya
- Nguva refu nosebleeds
- Kubvambura kubva kune gums
- Kubuda ropa kwenguva refu mushure mekugadzirisa zino kana kukuvara
- Ropa muzvigaro
- Hematuria (ropa mumurini)
- Menorrhagia (kunyanya kubuda kumwedzi kunopa ropa)
- Kubuda ropa kwakakwana kana kuchena kunokonzera ropa kunogona kuitika mumafomu akaoma akafanana nehemophilia
Zvinyorwa zveVon Willebrand Disease
- Tanga 1: Iyi ndiyo inowanzozivikanwa, inoitika inenge 75% yevarwere. Inopedzisira mumhuri mune mafashoni anozvigadzirisa akawanda anoreva kuti mubereki mumwe chete anofanira kuchinjwa kuti apfuure chirwere chacho. Izvi zvinokonzerwa nechezasi kune yakasikwa Von Willebrand factor level. Kusveta kunogona kuva nyoro kune yakaoma.
- Rudzi rwechipiri: Rudzi rwechipiri runoitika apo Von Willebrand factor haisi kushanda zvakanaka.
- Tora 2A: Yakapera mu autosomal yakawanda mafashoni. Kuenzanisa kusvika pakuenzanisa kubuda ropa kwakanyanya.
- Tora 2B: Yakapera mu autosomal yakawanda mafashoni. Kuenzanisa kusvika pakuenzanisa kubuda ropa kwakanyanya. Thrombocytopenia (low platelet count) haisi isiri.
- Tora 2M: Tsika isina kujairika inodarika mu autosomal yakakura mafashoni.
- Tora 2N: Tsika dzisina kujairika dzakapfuurira mu autosomal recess fashion. Izvi zvinoreva murwere anorwara anogamuchira makopi maviri e mutered gene, imwe kubva kumubereki mumwe nomumwe. Chiitiko cheVIII mazinga chinogona kunge chakanyanyisa. Kusveta kunogona kuva kwakanyanya uye kunogona kuvhiringidzwa ne hemophilia A.
- Rudzi rwechitatu: Ichi ndicho chisingawaniki cheVon Willebrand Disease. Icho chakapfuura muchitsime chakakosha chinotyisa. Kudya kunogona kuva kwakanyanya. Varwere vane rudzi urwu vane mukana wakawandisa kana kuti vasipo Von Willebrand factor. Izvi zvinokonzera kushayikwa mu Factor VIII uye kuwedzerwa kubuda ropa.
- Yakagadziriswa: Ichi chimiro cheVon Willebrand chirwere chinokonzerwa nechimwe chinhu chakafanana nekenza, autoimmune disorders, moyo wemanalies (kufanana ne ventricular septal defect, aortic stenosis), mishonga kana hypothyroidism.
Von Willebrand Disease Inoonekwa Sei?
Chokutanga, chiremba wako anofanira kunge achinyunyuta kuti une chirwere chekubuda ropa zvichienderana nezviratidzo zviri pamusoro. Kuva nedzimwe nhengo dzemhuri dzine zviratidzo zvakafanana zvinowedzera kusavhiringidzika kweVon Willebrand Disease, kunyanya kana varume nemadzimai vakagadziriswa (kusiyana neHemophilia inonyanya kuchinja varume).
Von Willebrand Disease inoratidzirwa nekuita mapurisa ebasa reropa rinotarisa zvose zviri zviviri zveVon Willebrand chinhu muropa pamwe chete nebasa rayo (ristocetin cofactor basa). Nokuti mhando dzakawanda dzeVon Willebrand Disease dzinogona kukonzera kuderedzwa kwe Factor VIII, mazinga eprotini inovhara inotumirwa zvakare. Von Willebrand multimers, iyo inotarisa chimiro cheVon WIllebrand factor uye kuti inoputsika sei, inonyanya kukosha pakuongorora Chirwere chechipiri.
Ndeapi Mishonga yeVon Willebrand Disease?
Vanyoro vanokanganisa varwere vangasazoda kurapwa.
- DDAVP: DDAVP (inonziwo demopressin) inonzi hormone inogadzirwa iyo inoshandiswa kuburikidza nepasip spray (kana pane dzimwe nguva kuburikidza ne IV). Iyi hormone inobatsira muviri kuti ubudise Von Willebrand chinhu chakachengetwa mumidziyo yeropa.
- Von Willebrand Factor replacement: Sezvakaita replacement factor inoshandiswa mu hemophilia, infusions yeVon Willebrand chinhu inogona kupiwa kudzivirira kana kurapa kubuda ropa. Izvozvi zvigadzirwawo zvine Factor VIII pamwe chete.
- Antifibrinolytics: Iyi mishonga (marita mazita Amicar naRysteda), anowanzopiwa mumuromo, anobatsira kugadzirisa maitiro ekugadzira. Izvi zvinogona kunyanya kukosha kumapapiro, kubuda mumuromo uye kumwedzi kunopa ropa.
- Zviratidzo zvepabonde: Muvakadzi vane Von Willebrand Disease uye kubuda kwemwedzi kunorema, zviratidzo zvekuberekwa kwehomoni zvakadai semishonga yekuberekwa kwepiritsi kana intrauterine zvinogona kushandiswa kuderedza / kurega kubuda ropa.