Von Willebrand ta cuta

Angiohemophilia, ko, a cikin wasu kalmomi, von Willebrand ta cuta (kuma rubuta game da ita "atrombotsitopenicheskaya purpura") - a jini cuta halin da karu a jini clotting da aka gada a wani autosomal rinjaye ko recessive hanya automno. Wannan Pathology na faruwa a tare da wani mita na 1: 1000. A tsawon rayuwar da marasa lafiya da aka ba rage, amma akwai da yaushe wani babban Yiwuwar tasowa a rai barazana zub da jini.

A sabili da wannan cuta ne rashin wani musamman abu (von Willebrand factor) kunshe a bango na jirgin ruwa kuma su bauta a matsayin stabilizer da transporter na factor Sabunta (antihemophilic globulin ko A) na jini coagulation tsarin. A sakamakon gaji da damuwa da platelet mannewa a kan garun jini, su agglutination da juna, rage jini cell kirga kuma coagulation ya karu zub da jini lokaci.

Von Willebrand ta cuta yana da dama iri.

Type 1 ne halin da wani kasa yawan von Willebrand factor tare da canzawa sinadaran tsarin. Yana auku a kimanin 75% na marasa lafiya da cutar. Autosomal rinjaye juna na gādo.

Type 2. The canje-canje shafi cikin sarari tsarin von Willebrand factor a al'ada jini yawa. Asibiti lura a 5-15% da marasa lafiya. Bayar da dama subtypes (A, B, M, N). Gādo iya gudãna daga ƙarƙashinsu biyu autosomal rinjaye da kuma autosomal recessive hanya.

Type 3. A mafi wuya qoramu form. Yana halin da sosai low matakin na factor a jini vWF (kasa da kashi 10% na al'ada). Bincikar lafiya kasa da 5% da marasa lafiya. Gādo auku a wani autosomal recessive hanya.

Platelet irin. Saboda maye gurbi cewa an gaji a wani autosomal-rinjaye type, akwai wani take hakkin platelet tsoka mai amsa sigina hadaddun tsarin, dauri ga von Willebrand factor.

A samu irin. Yana taso a cikin shakka daga rayuwa a sakamakon autoimmune matakai, halin da bayyanar autoantibodies a cikin jini jini von Willebrand Factor.

Saboda gaskiya cewa akwai wani a sassan ayyukan iri von Willebrand cuta, da na asibiti hoto iya zama sosai bambancin. Hemorrhagic ciwo iya bambanta daga m siffofin da predominance na hanci da gingival zub da jini mai tsanani siffofin da ke faruwa tare da nauyi igiyar ciki, kuma gastro-hanji zub da jini. A yara, cutar za a iya da ake zargi da maras wata-wata na jini daga hanci da m bruising a kan fata. Tun da gadon da cutar ba a bin da jima'i chromosomes, yiwuwar samun bugawa guda duka biyu mata da maza. Mata, duk da haka, saboda tsarin mulki da kuma physiological halaye da kwayoyin, cutar ne mafi wuyar jure. Sau da yawa ci gaba debilitating menorrhagia, wani babban yiwuwar zubar hemorrhage. Wannan zai sa zuwa fitowan da post-hemorrhagic anemia. Akwai kuma kananan damar gemoartrozov, ba su kai ga tsanani take hakki na mota aiki.

Yadda za a gane asali von Willebrand cuta?

Wannan shi ne fairly sauki tsari. Ganewar asali da cutar na faruwa a kan tushen da yawa instrumental samfurori, tabbatas da jihar jini. Jini analysis, wadda tayi matakin aiki na von Willebrand factor, jini coagulation gudun, da zai yiwu gaban autoantibodies a cikin jini jini. Wajibi ne a shigar da kara a kayyade shawara domin sanin yanayin gado da kuma hadarin bunkasa cutar a bãyanta.

Willebrand ta cutar za a iya bi a kan tushen da takamaiman da kuma wadanda ba takamaiman hanyoyin. Lokacin da nonspecific m yana amfani da inji hana zub da jini, gida haemostatics, aminocaproic acid (damuarn na ayyuka na fibrinolytic jini tsarin) Dicynonum (boosts thrombogenesis). Lokacin da takamaiman far za a iya amfani da kripretsipitatov wanda dauke da coagulating wakili da kuma wasu clotting dalilai.

Shin yana yiwuwa a hana von Willebrand cuta?

Kamar yadda m matakan da ake bada shawarar m motsa jiki, ba da wani sashe da hadarin rauni, kazalika da gudanar da bincike kafin conceiving wani yaro na asali itace na iyali, membobin wanda suke dauke da kwayar cutar.