Isifo von Willebrand sika

Angiohemophilia, noma, ngamanye amazwi, isifo von Willebrand sika (ngokubhala ngaye "atrombotsitopenicheskaya purpura") - ukuphazamiseka igazi libhekene ukwehla igazi ekunqandeni kanye ezuzwa i autosomal evelele enginayo noma esikhubazayo ngendlela automno. Lokhu zokugula kwenzeka nge imvamisa 1: 1000. Isilinganiso sobude bokuphila kwabantu ogulayo akuyona encishisiwe, kodwa kukhona njalo awokuthi ukwakha ukopha okuyisimo esisongela ukuphila.

Imbangela yalesi sifo ukungabi into ethile (von Willebrand isici) eziqukethwe odongeni bomkhumbi nokusebenza njengomeluleki isimame futhi Transporter ka-factor VIII (antihemophilic globulin noma A) kohlelo igazi coagulation. Ngenxa siyabhubha platelet adhesion odongeni imithambo yegazi, agglutination babo omunye nomunye, encishisiwe lwengqamuzana yegazi ukubalwa coagulation anda ukopha isikhathi.

isifo Von Willebrand sika has izinhlobo eziningana.

Uhlobo 1 libhekene anenombolo enganele von Willebrand isici nge ingashintshiwe chemical isakhiwo. Senzeka cishe 75% kweziguli uphethwe. Autosomal iphethini evelele ifa.

Uhlobo 2. Izinguquko kuthinte isakhiwo kwezendawo von Willebrand isici in lokuhlala evamile igazi. Emtholampilo ebonwe 5-15% eziguli. Kuncike subtypes eziningana (A, B, M, N). Ifa kungaba ageleze kokubili autosomal ephezulu kanye autosomal ngendlela esikhubazayo.

Uhlobo 3. nzima kakhulu ifomu olandelanayo. It elinesimo yizinga eliphansi kakhulu isizathu esenza plasma vWF (ngaphansi kuka-10% evamile). Uma sisheshe satholakala esingaphansi 5% weziguli ezine. Ifa yilesi sifo autosomal ngendlela esikhubazayo.

uhlobo Platelet. Ngenxa ukuguquka kwezakhi zofuzo ukuthi zizuzwe e uhlobo autosomal-evelele, kukhona ukwephulwa platelet yokwamukela isakhiwo eziyinkimbinkimbi, sesibopho ukuthobisa von Willebrand isici.

Uhlobo kuluthola. It ephakama ukuphila ngenxa izinqubo sokuhlasela kwamasosha omzimba, libhekene ukubukeka autoantibodies e oketshezini olungenambala lwegazi von Willebrand Factor.

Ngenxa yokuthi kukhona izinhlobo multiform ka von Willebrand isifo, isithombe emtholampilo kungenzeka zahlukahlukene kakhulu. Oluhambisana syndrome zingahluka kusuka amafomu omnene nge predominance ka acinene futhi gingival ukopha kumafomu ezinzima ezenzeka nge lesizalo esindayo futhi ukopha gastro-emathunjini. Ezinganeni, isifo kungenziwa esolwa ukopha nokuguqulwa emakhaleni nasebusweni nokulimala njalo esikhumbeni. Njengoba lesi sifo njengefa akuyona banamathela chromosome ubulili, ematfuba ukushaya okufanayo zabo bobubili ubulili. Abesifazane, Nokho, ngenxa izici zomthethosisekelo kanye bokuphila umzimba, lesi sifo singumbulali nzima ukubekezelela. Ngokuvamile ukuthuthukisa okuthena amandla menorrhagia, awokuthi kwangemva kokubeletha ukopha. Lokhu kungaholela ukuvela anaemia post-oluhambisana. Kukhona ithuba gemoartrozov encane, akuvezi nokwephula motor umsebenzi.

Indlela ukuxilonga von Willebrand isifo?

Lena inqubo kahle kulula. Ukuxilongwa sifo kwenzeka ngesisekelo amasampula eziningi wezinsimbi, ekutholeni isimo imithambo yegazi; Igazi ukuhlaziywa, olukukhombayo ezingeni umsebenzi von Willebrand isici, isivinini igazi coagulation, khona okungenzeka autoantibodies e oketshezini olungenambala lwegazi. Kuyadingeka ukuba banxuse Ukwelulekwa zofuzo ukucacisa uhlobo ifa kanye nengozi yokuba nesifo lesi sifo subsequent zukulwane.

isifo Willebrand sika zingelashwa ngesisekelo izindlela eziqondile futhi okungezona ethize. Lapho indlela nonspecific isebenzisa mechanical stop ukopha, haemostatics zendawo, aminocaproic acid (kuvimbela umsebenzi we fibrinolytic uhlelo igazi) Dicynonum (kuqinisa thrombogenesis). Lapho ukwelashwa ethize ingasetshenziswa kripretsipitatov aqukethe coagulating ejenti kanye nezici ezithile ekunqandeni.

Kungenzeka yini ukuvimbela von Willebrand isifo?

Njengoba izinyathelo zokuvimbela yanconywa umsebenzi mnene, ningathwali ubungozi bokulimala kanye nocwaningo phambi lokukhulelwa ingane kwesihlahla lozalo umndeni, amalungu okuyizinto abathwala lesi sifo.