Isifo seVon Willebrand

Isifo seVon Willebrand sesona sifo sixhaphakileyo esopha kwilifa.

Isifo seVon Willebrand sibangelwa kukusilela kwe-von Willebrand factor. I-Von Willebrand factor inceda iiplatelets zegazi zidibane kwaye zinamathele eludongeni lwemithambo yegazi, eyimfuneko ekunqandeni igazi. Kukho iintlobo ezininzi zesifo se-von Willebrand.

Imbali yosapho yokuphazamiseka kwegazi yeyona nto iphambili emngciphekweni.

Iimpawu zingabandakanya:

• Ukopha okungaqhelekanga kokuya exesheni
• Ukopha kweentsini
• Ukuthuthuzela
• Iimpumlo
• Ukudumba kolusu

Phawula: Uninzi lwabasetyhini abanesifo sokopha okunzima okanye ixesha elide abanaso isifo se-von Willebrand.

Isifo seVon Willebrand kunokuba nzima ukusixilonga. Amanqanaba asezantsi e-von Willebrand kunye nokopha akusoloko kuthetha ukuba unesifo se-von Willebrand.

Uvavanyo olunokwenziwa ukufumanisa esi sifo lubandakanya:

• Ixesha lokopha
• Ukuchwetheza igazi
• Inqanaba le-VIII yenqanaba
• Uhlalutyo lomsebenzi wePlatelet
• Ubalo lweplatelet
• Uvavanyo lweRistocetin cofactor
• Von Willebrand kuvavanyo lwezinto ezithile

Unyango lunokubandakanya i-DDAVP (desamino-8-arginine vasopressin). Liyeza ukunyusa inqanaba le-von Willebrand kunye nokunciphisa amathuba okopha.

Nangona kunjalo, i-DDAVP ayisebenzi kuzo zonke iintlobo zesifo se-von Willebrand. Uvavanyo kufuneka lwenziwe ukumisela ukuba loluphi uhlobo lwe-von Willebrand onayo. Ukuba uza kutyandwa, ugqirha wakho angakunika i-DDAVP ngaphambi kotyando ukubona ukuba amanqanaba akho e-von Willebrand ayonyuka na.

Iyeza i-Alphanate (into ebulala i-antihemophilic factor) yamkelwe ukunciphisa ukopha kubantu abanesifo ekufuneka betyandiwe okanye nayiphi na inkqubo engenayo.

Iplasma yegazi okanye into ethile VIII yamalungiselelo inokusetyenziselwa ukunciphisa ukopha.

Ukopha kunokuhla ngexesha lokukhulelwa. Abasetyhini abanayo le meko bahlala bengenalo ukopha kakhulu ngexesha lokubeleka.

Esi sifo sidluliselwa kwiintsapho. Iingcebiso ngemfuzo zinokunceda abo bafuna ukuba ngabazali baqonde umngcipheko wabantwana babo.

Ukopha kunokwenzeka emva kotyando okanye xa ukhuphe izinyo.

I-Aspirin kunye nezinye iziyobisi ezichasayo (i-NSAIDs) zinokuyenza mandundu le meko. Sukuthatha la mayeza ungakhange uqale uthethe nomboneleli wakho wezempilo.

Biza umnikezeli wakho ukuba ukuphuma kwegazi kwenzeka ngaphandle kwesizathu.

Ukuba unesifo se-von Willebrand kwaye ucwangciselwe uqhaqho okanye usengozini, qiniseka ukuba wena okanye usapho lwakho uxelela ababoneleli ngemeko yakho.

Ingxaki yokopha - von Willebrand

• Ukubumba kwegazi
• Amahlwili egazi

Umkhukula VH, uScott JP. Isifo seVon Willebrand. Ku: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, ii-eds. Incwadi kaNelson yeNcwadi yePediatrics. Umhla wama-21. IPhiladelphia, PA: Elsevier; 2020: isahluko 504.

UJames P, uRydz N. Ubume, ibhayoloji, kunye nemfuza ye-von Willebrand factor. Ku: Hoffman R, Benz EJ, Silberstein LE, et al, ii-eds. I-Hematology: Imigaqo esisiseko kunye nokuziqhelanisa. Umhla wesi-7. IPhiladelphia, PA: Elsevier; I-2018: isahluko 138.

INeff AT. Isifo seVon Willebrand kunye nokungaqheleki kokopha kweplatelet kunye nokusebenza kweethambo. Ku: IGoldman L, iSchafer AI, ii-eds. Amayeza eGoldman-Cecil. Ngomhla wama-26. IPhiladelphia, PA: Elsevier; 2020: isahluko 164.

Iingxaki zikaSamuels P. Hematologic zokukhulelwa. Ku: Landon MB, Galan HL, Jauniaux ERM okqhubekayo, eds. Ii-Obstetrics zikaGabbe: Ukukhulelwa okuqhelekileyo kunye neengxaki. Ngomhla we-8. IPhiladelphia, PA: Elsevier; 2021: isahluko 49.