Kodi Von Wilbrand Disease Ndi Chiyani?

Von Willebrand Disease ndi matenda ambiri omwe amachokera ku magazi , omwe amawononga pafupifupi 1% mwa anthu.

Kodi Von Willebrand ndi chiyani?

Von Willebrand chinthu ndi mapuloteni amagazi omwe amamanga ku VIII (coagulation factor). Pamene chinthu cha VIII sichiri chowopsa cha Von Willebrand matenda, chimatha mosavuta. Von Willebrand matenda amathandizanso mapulaneti kuti azigwirizana ndi malo ovulala.

Kodi Zizindikiro Ndi Ziti?

Odwala ena sakhala ndi magazi ambiri. Von Wilbrand Disease ikugwirizana ndi:

• Kulira kosavuta
• Mphuno ya nosebleeds
• Kutsekemera kuchokera ku chingamu
• Kuthamanga kwanthawi yaitali pambuyo poizoni kapena kuvulala
• Magazi mu mpando
• Hematuria (magazi mumtsuko)
• Menorrhagia (kutaya magazi kwambiri)
• Kuchetsa magazi kumodzi kumapangidwe koopsa m'mimba mofanana ndi hemophilia

Mitundu ya Von Willebrand Disease

• Mtundu Woyamba: Iyi ndiyo mawonekedwe ofala kwambiri, omwe amapezeka pafupifupi 75% mwa odwala. Zimadutsa m'mabanja mwa mafashoni omwe amachititsa kuti kholo limodzi lizikhudzidwa kuti lidutse matendawa. Izi zimachokera kufupi ndi yachiwiri ya Von Willebrand factor level. Kutsekemera kungakhale kofatsa mpaka koopsa.
• Mtundu 2: Mtundu 2 umapezeka pamene Von Willebrand chinthu sichigwira ntchito mwachizolowezi.
  • Lembani 2A: Anatsika mu mafashoni ambiri. Kuchepetsa magazi kwambiri.
  • Lembani 2B: Ndinadutsa mu mafashoni oposa autosomal. Kuchepetsa magazi kwambiri. Thrombocytopenia (yochepa platelet count) si yachilendo.
  • Pezani mtundu wa 2M: Mtundu wodabwitsa unadutsa mumayendedwe autosomal.
  • Mtundu wa 2N: Mtundu wodabwitsa unadutsa mu autosomal fashion fashion. Izi zikutanthauza kuti wodwalayo wodwalayo amalandira mitundu iwiri ya jini lotengera, yomwe imachokera kwa kholo lililonse. Zochitika Zachikhalidwe VIII zingakhale zochepa kwambiri. Kutsekemera kungakhale koopsa ndipo kungasokonezeke ndi hemophilia A.

• Mtundu wachitatu: Ichi ndi mtundu wamba wa Von Willebrand Disease. Zadutsa mu autosomal mafashoni kwambiri. Kutsekemera kungakhale kovuta. Odwala omwe ali ndi mtundu umenewu ali ndi ndalama zochepa kwambiri kapena alibe Von Willebrand factor. Izi zimapangitsa kuti Factor VIII ipeŵe ndi kutaya magazi kwakukulu.
• Kupezeka: Fomu iyi ya Von Willebrand matenda imayambanso ndi chinthu china monga khansa, matenda omwe amadzimva okha, matenda osokonezeka mtima (monga ventricular septal defect, aortic stenosis), mankhwala kapena hypothyroidism.

Kodi Von Wilbrand Disease Diagnosed?

Choyamba, dokotala wanu ayenera kukayikira kuti muli ndi matenda ochotsa magazi kuchokera ku zizindikiro zapamwamba. Kukhala ndi mamembala ena omwe ali ndi zizindikiro zofanana kumawonjezera kukayikira kwa Von Willebrand Disease, makamaka ngati amuna ndi akazi amakhudzidwa (mosiyana ndi hemophilia yomwe imakhudza kwambiri amuna).

Von Willebrand Disease amapezeka mwa kupanga ntchito ya magazi yomwe imayang'ana pa Von Willebrand chinthu m'magazi komanso ntchito yake (ntchito ya ristocetin cofactor). Chifukwa mitundu yambiri ya Von Willebrand Disease ikhoza kuchepetsa Factor VIII, mapuloteni otsekemerawa amatumizidwa. Von Willebrand multimers, yomwe imayang'ana momwe zinthu zilili ndi Von WIbrand factor ndi momwe zimathyoka, ndizofunika makamaka kuti mupeze matenda a mtundu wa 2.

Kodi Chithandizo cha Matenda a Won Wachilombo N'chiyani?

Odwala ochepetsedwa sangadye mankhwala.

• DDAVP: DDAVP (yomwe imatchedwanso demopressin) ndi hormone yokha yomwe imayendetsedwa kudzera pamphuno yamphongo (kapena nthawi zina kudzera mu IV). Hormone imathandiza thupi kutulutsa Von Willebrand chinthu chosungidwa m'mitsempha ya magazi.
• Von Willebrand Factor replacement: Mofananamo ndi malo omwe amagwiritsidwa ntchito mu hemophilia, infusions ya Von Willebrand chinthu chingaperekedwe pofuna kupewa kapena kutulutsa magazi. Zida zimenezi zili ndi Factor VIII komanso.

• Antifibrinolytics: Mankhwala awa (mayina a dzina la Amicar ndi Lysteda), omwe amaperekedwa pamlomo, amathandiza kuti zikhale zolimba. Izi zingakhale zothandiza makamaka pa nosebleeds, m'magazi m'magazi komanso m'magazi.
• Zochitika za kulera: Kwa amayi omwe ali ndi Von Willebrand Disease ndi kulemera kwa msambo wakuda, kuyamwa kwa mahomoni monga mapiritsi oletsa kubereka kapena intrauterine zingagwiritsidwe ntchito kuchepetsa / kusiya magazi.