Matenda a Von Willebrand

Angiohemophilia, kapena, mwa kuyankhula kwina, matenda a Willebrand (omwe amatchedwanso "atrombocytopenic purpura") ndi matenda a magazi omwe amadziwika ndi kuchepa kwa kutaya magazi ndipo amachokera ku autosomal kapena autonomic recessive type. Matendawa amapezeka pafupipafupi 1: 1000. Kuyembekeza kwa moyo kwa anthu odwala sikungachepetsedwe, koma nthawi zonse nthawi zambiri amakhala ndi mwayi waukulu wopha magazi.

Choyambitsa matendawa ndi kusowa kwa chinthu china (vWF) chomwe chili mu khoma lachitetezo ndikukhala ngati stabilizer ndi chithandizo cha factor VIII (kapena anti-hemophilic globulin A) ya magazi. Chotsatira chake, mapuloletti amamatira ku khoma la mitsempha ya magazi, kuphatikizapo wina ndi mzake, kugawanika kwa maselo a magazi kumachepetsa ndipo nthawi yamagazi imakula.

Matenda a Von Willebrand ali ndi mitundu ingapo.

Mtundu 1. Umadziwika ndi mphamvu ya von Willebrand yosasinthika. Amapezeka pafupifupi 75% odwala omwe ali ndi matendawa. Mtundu wodziwika kwambiri wa cholowa.

Mtundu 2. Zosintha zimakhudza malo opangidwa ndi von Willebrand factor pa ndondomeko yoyenera m'magazi. Amagwiritsidwa ntchito pang'onopang'ono mu 5-15% mwa odwala. Pali zigawo zingapo (A, B, M, N). Cholowa chikhoza kuchitika zonse mu autosomal kwambiri komanso autosomal mtundu wambiri.

Mtundu 3. Njira yovuta kwambiri. Wodziwika ndi wotsika kwambiri wa von Willebrand chinthu m'magazi a m'magazi (osachepera 10% mwa chizolowezi). Amadziwika osachepera 5% mwa odwala. Cholowa chimapitirira molingana ndi autosomal recessive mtundu.

Mtundu wa Thrombocyte. Chifukwa cha kusintha kwa zinthu zomwe zimatengera mtundu wa autosomal, pali kusokonezeka kwa kapangidwe kake kakang'ono ka mapuloleteni, omwe amamangidwa ndi a von Willebrand factor.

Mtundu woyenera. Zimayambira pa moyo chifukwa cha njira zomwe zimadziwika ndi maonekedwe a m'magazi a m'magazi a autoantibodies kwa von Willebrand factor.

Chifukwa chakuti pali mitundu yosiyanasiyana ya matenda a von Willebrand, chithunzi chachipatala chingakhale chosiyana kwambiri. Matenda a Hemorrhagic amatha kukhala ndi mitundu yofewa yomwe imakhala ndi mafinya ambirimbiri omwe amapezeka m'mimba ndi mazira akuluakulu omwe amabwera chifukwa cha uterine komanso m'mimba. Ali mwana, matendawa amatha kukayikira mwazidzidzidzi kuchokera m'mphuno komanso kuphulika khungu nthawi zambiri. Popeza cholowa cha matendawa sichigwirizana ndi ma chromosomes opatsirana pogonana, mwayi wokondana pakati pa oimira awiriwa ndi chimodzimodzi. Komabe, amayi, chifukwa cha maonekedwe a thupi ndi matupi a thupi, amakhala odwala matendawa. Kawirikawiri zimakhala zofooketsa mimba, mwayi wa kutuluka magazi pambuyo pake. Izi zingachititse kuti zikhale zochitika m'thupi mwachangu. Palinso kachilombo kakang'ono ka haemoarthritis, zomwe sizimayambitsa kuwonongeka kwakukulu kwa magalimoto.

Kodi mungapeze bwanji matenda a von Willebrand?

Iyi ndi njira yovuta kwambiri. Kuzindikira kwa matendawa kumachokera ku mayesero ambiri a zida zomwe zimapangitsa mitsempha ya mthupi; Kuyezetsa magazi komwe kumasonyeza kuchuluka kwa ntchito ya von Willebrand factor, mlingo wamagazi, kutuluka kwa maantiantibodies m'magazi a magazi. Ndikofunika kuti upange uphungu wokhudzana ndi majini kuti udziwe momwe alili cholowa komanso chiopsezo chotenga matendawa m'mibadwo yotsatira.

Matenda a Von Willebrand amatha kuchiritsidwa mothandizidwa ndi njira zopanda ntchito komanso zenizeni. Ndi njira yosavomerezeka, kuyimitsa makina okhetsa magazi, magulu a hemostatics, aminocaproic acid (imaletsa ntchito ya fibrinolytic dongosolo la magazi), dicinone (kumawonjezera thrombogenesis). Ndi mankhwala enieni, ndizotheka kugwiritsa ntchito crypticipates, omwe ali ndi othandizira othandizira ndi zinthu zina zogwirizanitsa.

Kodi n'zotheka kupewa matenda a von Willebrand?

Monga njira yowonetsetsa kuti ntchito yowala ikhale yopanda kuvulaza, komanso kafukufuku asanatenge mwana wa banja, omwe mamembala ake amanyamula matendawa.