Von Willebrand cuta: menene menene, yadda za'a gano shi da kuma yadda ake yin magani

Wadatacce

• Babban bayyanar cututtuka
• Yaya ganewar asali
• Yadda ake yin maganin
• Jiyya a ciki
• Shin maganin ba shi da kyau ga jariri?

Cutar Von Willebrand ko VWD cuta ce ta kwayar halitta da gado ta halin raguwa ko rashi samar da sinadarin Von Willebrand (VWF), wanda ke da muhimmiyar rawa a cikin aikin narkewar jini. Dangane da kwaskwarimar, ana iya rarraba cutar von Willebrand zuwa manyan nau'ikan uku:

• Rubuta 1, wanda a cikinsa akwai raguwar sashi a cikin samarwar VWF;
• Rubuta 2, wanda abin da aka samar ba shi da aiki;
• Rubuta 3, wanda a cikinsa akwai cikakken rashi na Von Willebrand factor.

Wannan lamarin yana da mahimmanci don inganta mannewar platelet zuwa endothelium, ragewa da dakatar da zub da jini, kuma yana dauke da factor VIII na coagulation, wanda yake da mahimmanci don hana lalacewar platelet a cikin jini kuma ya zama dole don kunna factor X da ci gaba da cascade. don samar da faranti.

Wannan cutar ta gado ce kuma ta gado ce, wato, ana iya yada shi tsakanin tsararraki, duk da haka, ana iya samun sa a lokacin balagar sa lokacin da mutum ke da wani nau'in cutar kansa ko kansa, misali.

Cutar Von Willebrand ba ta da magani, amma sarrafawa, wanda dole ne a yi ta tsawon rayuwa bisa ga jagorancin likita, nau'in cuta da alamun da aka gabatar.

Babban bayyanar cututtuka

Alamun cutar Von Willebrand sun dogara da nau'in cutar, amma, mafi yawan abubuwan sun hada da:

• Yawan jini da tsawan lokaci daga hanci;
• Yawan zubar jini daga gumis;
• Zub da jini bayan yankewa;
• Jini a cikin kujeru ko fitsari;
• Yawaitar rauni a sassa daban-daban na jiki;
• Flowara yawan jinin haila.

Yawanci, waɗannan alamun sun fi tsanani ga marasa lafiya da cutar ta Wanobrand nau'in 3, saboda akwai rashi mai yawa na furotin da ke daidaita daskarewa.

Yaya ganewar asali

Ganewar cutar ta Von Willebrand ana yin ta ne ta hanyar gwaje-gwajen gwaje-gwaje inda ake binciken kasancewar VWF da cutar plasma factor VIII, ban da gwajin lokaci na zubar jini da kuma yawan keɓaɓɓun platelets. Yana da kyau a maimaita gwajin sau 2 zuwa 3 domin samun daidaiton ganewar cutar, tare da gujewa sakamakon karya.

Saboda cuta ce ta kwayar halitta, ana iya bada shawarar bada shawara kan kwayar halitta kafin ko lokacin daukar ciki don duba yiwuwar haihuwar jaririn da cutar.

Dangane da gwaje-gwajen gwaje-gwaje, ƙananan matakan ko rashi na VWF da factor VIII da tsawan aPTT yawanci ana gano su.

Yadda ake yin maganin

Yin magani don cutar ta Von Willebrand ana yin shi ne bisa ga jagorancin likitan jini da kuma amfani da antifibrinolytics, waɗanda ke da ikon sarrafa zub da jini daga murfin baka, hanci, zubar jini da kuma cikin hanyoyin haƙori, yawanci ana ba da shawarar.

Bugu da ƙari, ana iya nuna amfani da Desmopressin ko Aminocaproic acid don tsara coagulation, ban da mahimmin abin da ke cikin Von Willebrand.

A yayin jiyya, ana ba da shawarar cewa mutanen da ke fama da cutar ta Von Willebrand su guji yanayi mai haɗari, kamar yin wasan motsa jiki da yawa da shan aspirin da wasu magungunan da ba na steroidal ba, kamar Ibuprofen ko Diclofenac, ba tare da shawarar likita ba.

Jiyya a ciki

Mata masu cutar Von Willebrand na iya samun juna biyu na al'ada, ba tare da buƙatar magani ba, amma, ana iya yada cutar ga theira childrenansu, tunda cuta ce ta kwayar halitta.

A wadannan yanayin, maganin cutar a yayin daukar ciki ana yin sa ne kwanaki 2 zuwa 3 kacal kafin haihuwa tare da desmopressin, musamman idan haihuwa ta hanyar bangaren tiyata ne, kuma amfani da wannan magani don magance zubar jini da kiyaye rayuwar mace yana da mahimmanci. Yana da mahimmanci kuma ayi amfani da wannan maganin har zuwa kwanaki 15 bayan haihuwa, saboda matakan factor VIII da VWF sun sake raguwa, tare da haɗarin zubar jini bayan haihuwa.

Koyaya, wannan kulawa ba lallai bane koyaushe, musamman idan matakan VIII yawanci yawanci 40 IU / dl ne ko fiye. Wannan shine dalilin da ya sa yana da mahimmanci a sadu da lokaci-lokaci tare da likitan jini ko likitan mata don tabbatar da bukatar amfani da magunguna da kuma ko akwai haɗari ga mace da jaririn.

Shin maganin ba shi da kyau ga jariri?

Amfani da magunguna masu alaƙa da cutar ta Von Willebrand yayin ciki ba ya cutar da jariri, don haka hanya ce mai aminci. Duk da haka, ya zama dole ga jariri ya gudanar da gwajin kwayar halitta bayan haihuwa domin tabbatar da ko ba shi da cutar, kuma idan haka ne, don fara jinya.