Von Willebrand chirwere: chii icho, nzira yekuzviziva uye marapirwo anoitwa

Zvemukati

• Zviratidzo zvikuru
• Sei kuongororwa
• Kurapwa kunoitwa sei
• Kurapa mune pamuviri
• Kurapa kwakaipa kumwana here?

Chirwere cheVon Willebrand kana VWD chirwere chemajini uye chinogarwa nhaka chinoratidzwa nekuderera kana kusavapo kwekugadzirwa kweVon Willebrand factor (VWF), iyo ine basa rakakosha mukugadzirisa mashandiro. Zvinoenderana nekugadziriswa, chirwere chaVil Willebrand chinogona kuiswa muzvikamu zvitatu zvikuru.

• Tora 1, umo mune kuderera zvishoma mukugadzirwa kweVWF;
• Tora 2, umo chinhu chakaburitswa chisiri kushanda;
• Tora 3, umo mune kukwana kwakakwana kweVon Willebrand factor.

Ichi chinhu chakakosha kusimudzira kunamatira kweplatelet kune endothelium, kudzikira uye kumisa kubuda kweropa, uye iri kutakura chinhu VIII chekugwamba, izvo zvakakosha kudzivirira kuora kweplatelet muplasma uye zvinodikanwa kuti ishandiswe yechinhu X uye kuenderera kweiyo cascade. kugadzira platelet plug.

Ichi chirwere chiri chemajini uye chinogarwa nhaka, ndiko kuti, chinogona kupfuudzwa pakati pezvizvarwa, zvisinei, icho chinogona zvakare kuwanikwa mukati mehukuru kana munhu aine rumwe rudzi rwechirwere chemuviri kana gomarara, semuenzaniso.

Chirwere chaVon Willebrand hachina mushonga, asi kuzvidzora, kunofanirwa kuitwa muhupenyu hwese zvinoenderana nachiremba, mhando yechirwere nezviratidzo zvinoratidzwa.

Zviratidzo zvikuru

Zviratidzo zvechirwere cheVon Willebrand zvinoenderana nerudzi rwechirwere, zvisinei, chinonyanya kusanganisira:

• Kakawanda uye kwenguva refu kubuda ropa kubva mumhino;
• Kudzokorora kubuda ropa kubva pamazino;
• Kuwedzera kubuda ropa mushure mekucheka;
• Ropa muchigaro kana weti;
• Kakawanda kupwanya zvikamu zvakasiyana zvemuviri;
• Kuwedzera kuenda kumwedzi.

Kazhinji, izvi zviratidzo zvinonyanya kuomarara kune varwere vane von Willebrand rudzi rwechitatu chirwere, sezvo paine kushomeka kukuru kweprotein inodzora kugwamba.

Sei kuongororwa

Kuongororwa kwechirwere cheVon Willebrand kunoitwa kuburikidza nemuyedzo wevarabhoritari umo kuvapo kweVWF uye plasma factor VIII kunoongororwa, kuwedzera pakuyerera kweropa nguva uye kuwanda kwemaplatelet. Zvakajairika kuti bvunzo dzidzokororwe kaviri kana katatu kuitira kuti uwane kunyatsoongorora chirwere, uchidzivirira mhedzisiro-isina mhedzisiro.

Nekuti icho chirwere chemajini, kurairwa kwemajini usati kana panguva yekuzvitakura kunogona kukurudzirwa kuti utarise njodzi yemwana kuzvarwa aine chirwere ichi.

Zvichienderana nemuyedzo wevarabhoritari, mashoma masosi kana kusavapo kweVWF uye factor VIII uye kwenguva refu aPTT zvinowanzoonekwa.

Kurapwa kunoitwa sei

Kurapa kwechirwere cheVon Willebrand kunoitwa zvinoenderana nekutungamira kwehematologist uye kushandiswa kweantifibrinolytics, ayo anokwanisa kudzora kubuda ropa kubva mumuromo mucosa, mhino, kubuda ropa uye nenzira dzemazino, inowanzo kurudzirwa.

Uye zvakare, kushandiswa kweDesmopressin kana Aminocaproic acid kugadzirisa coagulation kunogona kuratidzwa, mukuwedzera kune Von Willebrand factor concentrate.

Panguva yekurapwa, zvinorayirwa kuti vanhu vane chirwere cheVon Willebrand vanodzivirira mamiriro ezvinhu ane njodzi, senge tsika yemitambo yakanyanyisa uye kunwa kweaspirini uye nezvimwe zvisiri-steroidal zvinorwisa-kupisa zvinodhaka, senge Ibuprofen kana Diclofenac, pasina zano rekurapa.

Kurapa mune pamuviri

Vakadzi vane chirwere cheVon Willebrand vanogona kuve nepamuviri zvakajairika, pasina kudiwa kwemishonga, zvisinei, chirwere ichi chinogona kupfuudzwa kuvana vavo, nekuti chiri chirwere chemajini.

Muzviitiko izvi, kurapwa kwechirwere panguva yekuzvitakura kunoitwa chete mazuva maviri kana matatu usati wasununguka ne desmopressin, kunyanya kana kuendesa kuri nechikamu chekusiya, uye kushandiswa kwemushonga uyu kudzora kubuda kweropa uye kuchengetedza hupenyu hwemukadzi kwakakosha. Izvo zvakakoshawo kuti uyu mushonga ushandiswe kusvika kumazuva gumi neshanu mushure mekusununguka, sezvo nhanho dzechinhu VIII neVWF dzichiderera zvakare, zvine njodzi yekubuda ropa mushure mekusununguka.

Nekudaro, uku kuchengetwa hakugare kuchidikanwa, kunyanya kana factor VIII nhanho kazhinji iri 40 IU / dl kana zvimwe. Ndosaka zvakakosha kuti uve nehukama nguva nenguva nachiremba wezvematambudziko kana vezvekubereka kuti vaone kukosha kwekushandisa mishonga uye kana paine njodzi kune vese mukadzi nemwana.

Kurapa kwakaipa kumwana here?

Iko kushandiswa kwemishonga inoenderana nechirwere cheVon Willebrand panguva yekuzvitakura hakukuvadze mwana, uye nekudaro inzira yakachengeteka. Nekudaro, zvakafanira kuti mucheche aite genetiki ongororo mushure mekuzvarwa kuitira kuti aone kana ane chirwere kana kwete, uye kana zvirizvo, atange kurapwa.