Isifo seVon Willebrand

Isifo iVon Willebrand yisona sifo esivame kakhulu ukopha esivela efeni.

Isifo iVon Willebrand sibangelwa ukuntuleka kwesici se-von Willebrand. Isici seVon Willebrand sisiza ama-platelet egazi ahlangane futhi anamathele odongeni lwemithambo yegazi, okudingekayo ekunqandeni igazi okujwayelekile. Kunezinhlobo eziningana zesifo se-von Willebrand.

Umlando womndeni wesifo sokopha yiyona nto eyingozi eyinhloko.

Izimpawu zingafaka:

• Ukopha okungavamile kokuya esikhathini
• Ukopha kwezinsini
• Ukulimaza
• Ukopha ngamakhala
• Ukuqhuma kwesikhumba

Qaphela: Iningi labesifazane abanesifo sokopha esindayo noma esinde isikhathi eside abanaso isifo se-von Willebrand.

Isifo seVon Willebrand kungaba nzima ukusithola. Amazinga we-Low von Willebrand factor kanye nokopha akusho ukuthi unesifo se-von Willebrand ngaso sonke isikhathi.

Ukuhlolwa okungenziwa ukuthola lesi sifo kufaka:

• Isikhathi sokopha
• Ukuthayipha kwegazi
• Izinga le-factor VIII
• Ukuhlaziywa komsebenzi wePlatelet
• Ukubalwa kweplatelet
• Ukuhlolwa kwe-Ristocetin cofactor
• IVon Willebrand factor izivivinyo ezithile

Ukwelashwa kungafaka i-DDAVP (desamino-8-arginine vasopressin). Kungumuthi wokukhulisa i-von Willebrand level level futhi unciphise namathuba wokopha.

Kodwa-ke, i-DDAVP ayisebenzi kuzo zonke izinhlobo zesifo se-von Willebrand. Ukuhlolwa kufanele kwenziwe ukuthola ukuthi hlobo luni lwe-von Willebrand onayo. Uma uzohlinzwa, udokotela wakho angakunika i-DDAVP ngaphambi kokuhlinzwa ukuze ubone ukuthi amazinga akho e-von Willebrand factor ayakhula yini.

Umuthi i-Alphanate (i-antihemophilic factor) uvunyelwe ukwehlisa ukopha kubantu abanalesi sifo okufanele bahlinzwe noma iyiphi enye inqubo ehlaselayo.

I-plasma yegazi noma amalungiselelo athile we-VIII nawo angasetshenziswa ukunciphisa ukopha.

Ukopha kungancipha ngesikhathi sokukhulelwa. Abesifazane abanalesi simo ngokuvamile abanalo ukopha ngokweqile ngesikhathi sokubeletha.

Lesi sifo sidluliselwa ngemindeni. Ukwelulekwa ngofuzo kungasiza abazoba ngabazali ukuba baqonde ubungozi bezingane zabo.

Ukopha kungahle kwenzeke ngemuva kokuhlinzwa noma lapho ukhishwe izinyo.

I-Aspirin nezinye izidakamizwa ezingezona ukuvuvukala (ama-NSAID) kungenza lesi simo sibe sibi kakhulu. Ungayiphuzi le mithi ngaphandle kokuqala ukhulume nomhlinzeki wakho wezokunakekelwa kwempilo.

Shayela umhlinzeki wakho uma ukopha kwenzeka ngaphandle kwesizathu.

Uma unesifo se-von Willebrand futhi uhlelelwe ukuhlinzwa noma usengozini, qiniseka ukuthi wena noma umndeni wakho utshela abahlinzeki ngesimo sakho.

Isifo sokopha - von Willebrand

• Ukwakheka kwegazi
• Amahlule egazi

Isikhukhula VH, Scott JP. Isifo seVon Willebrand. Ku: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Incwadi kaNelson Yezingane. Umhlaka 21. IPhiladelphia, PA: Elsevier; 2020: isahluko 504.

UJames P, uRydz N. Isakhiwo, ibhayoloji, kanye nezakhi zofuzo ze-von Willebrand factor. Ku: Hoffman R, Benz EJ, Silberstein LE, et al, ama-eds. I-Hematology: Izimiso Eziyisisekelo Nokuzijwayeza. Umhla wesi-7. IPhiladelphia, PA: Elsevier; 2018: isahluko 138.

I-Neff AT. Isifo se-Von Willebrand nokungajwayelekile kokopha kweplatelet nokusebenza kwemithambo yegazi. Ku: IGoldman L, iSchafer AI, ama-eds. Imithi yeGoldman-Cecil. Umhla ka-26. IPhiladelphia, PA: Elsevier; 2020: isahluko 164.

Izinkinga zikaSamuels P. Hematologic zokukhulelwa. Ku: Landon MB, Galan HL, Jauniaux ERM et al, eds. Ama-Obstetrics kaGabbe: Ukukhulelwa Okujwayelekile Nenkinga. Umhlaka 8. IPhiladelphia, PA: Elsevier; 2021: isahluko 49.