Mate von Willebrand o

Angiohemophilia, ranei, i roto i nga kupu atu, mate von Willebrand o (tuhituhia hoki e pā ana ki a ia "te purapura atrombotsitopenicheskaya") - he mate toto āhuatanga e te hekenga i roto i upoo- toto me te riro i roto i te autosomal tino ngoikore ranei automno tikanga. puta ana tēnei māuiuitanga ki te auautanga o te 1: 1000. Ko te tümanako ora e kore e o te iwi mate iti, engari i reira he tonu he tūponotanga nui o te whakawhanake i te toto ora-te whakawehi.

Ko te take o tenei mate ko te kore o te taonga ngā (von Willebrand take) i roto i roto i te taiepa o te oko, me te ki te mahi rite te stabilizer me te taraka o te tauwehe VIII o te pūnaha toto coagulation (antihemophilic globulin A ranei). Ka rite ki te hua o te adhesion mōtepe ohorere ki te taiepa o oko toto, ratou agglutination ki tetahi ki tetahi, nui haere heke toto kaute pūtau me te coagulation wā toto.

mate von Willebrand o He maha ngā momo.

Momo 1 āhuatanga e te maha iti o te tauwehe von Willebrand ki hanganga matū tonu. puta te reira i roto i āhua 75% o tūroro ki te tātaritanga. tauira tino Autosomal o tupu.

Momo 2. pā te huringa te hanganga mokowā o tauwehe von Willebrand i roto i nohanga toto noa. Hauora kitea i roto i te 5-15% o tūroro. Whakaratohia e rave rahi momoroto (A, B, M, N). Ka taea e kainga rere rua autosomal tikanga ngoikore tino, me te autosomal.

Momo 3. Ko te uaua tino puka rere. āhuatanga i te reira e te taumata tino iti o te take i roto i te wē vWF (iti iho i te 10% o noa). Pāngia iti iho i te 5% o tūroro. puta tupu i roto i te tikanga ngoikore autosomal.

momo mōtepe. Nā ki irakētanga e kua riro i roto i te momo autosomal-tino, i reira te mea he takahi mōtepe receptor hanganga matatini, here ki te tauwehe von Willebrand.

Ko te momo riro. whakatika te reira i roto i te akoranga o te ora rite ki te hua o ngā tukanga autoimmune, āhuatanga e te ahua o autoantibodies i roto i te wē toto von Willebrand Factor.

Nā ki te meka i reira he he momo multiform o mate von Willebrand, kia kia tino kanorau te pikitia haumanu. Hemorrhagic syndrome kia rerekē i ngā puka ngawari ki te predominance o nasal me gingival toto ki puka kino tupu ki uterine taimaha, me te toto Gastro-intestinal. I roto i te tamariki, e taea te whakapae te mate o te toto noa i te ihu me te maru auau i runga i te kiri. Mai te tuku i te mate e kore e piri ki te pūira sex, te tūponotanga o te patu i te taua hoki tane e rua. Women, Heoi, e tika ana ki ngā āhuatanga ture, me te whaiaroaro o te rauropi, he uaua ki te aro ki te mate. E mea pinepine te whakawhanake haaparuparu menorrhagia, he tūponotanga tiketike o te kulukia toto. Tenei e taea e arahi ki te putanga o te anemia pou-hemorrhagic. He he ano hoki te gemoartrozov tupono iti, e kore arahi ki maumaui nui o te nekeneke mahi.

Me pēhea te ki te tātari mate von Willebrand?

Ko te tukanga tika ngāwari tenei. Tātaritanga o te mate puta i runga i te pūtake o maha tauira mauhaa, whakatau i te āhua o oko toto; tātari toto, e tāutu ana i te taumata o te mahi o von Willebrand take, tere toto coagulation, te aroaro ka taea o autoantibodies i roto i te wē toto. He mea tika ki te karanga ki te tohutohu iranga ki te whakatau i te āhua o te tupu, me te mōrea o te whakawhanake i te mate i roto i nga whakatupuranga o muri.

Ka taea te tukinotia mate a Willebrand i te pūtake o ngā tikanga motuhake, me te kore-motuhake. A, no te whakamahi huarahi nonspecific mīhini toto mutu, haemostatics rohe, waikawa aminocaproic (inhibits te mahi o te pūnaha toto fibrinolytic) Dicynonum (hakahaka thrombogenesis). A, no te taea te whakamahi therapy tauwhāiti kripretsipitatov e roto i coagulant kaihoko me etahi āhuatanga upoo-.

He reira taea ki te ārai mate von Willebrand?

Ka rite ki e āraihia mahi ngawari tūtohutia, e kore e kawe te mōrea o te whara, me te rangahau i mua i hapu he tamaiti o te rakau whakapapa o te whānau, he te mau melo o e kawe o te mate.