Isifo von Willebrand kaThixo

Angiohemophilia, okanye, ngamanye amazwi, isifo von Willebrand kaThixo (kwakubhaliwe malunga "atrombotsitopenicheskaya purpura" yakhe) - a igazi esibonakala ukuncipha igazi ekunqandeni kwaye ilifa ngendlela autosomal lubalaseleyo okanye sofuzo indlela automno disorder. Le ngezifo kwenzeka nge frequency 1: 1000. Iminyaka yokuphila abantu abagulayo akuxhomekekanga lifinyele, kodwa kusoloko kukho amathuba ophezulu yokuphuhlisa ezophayo esisongela ubomi bakho.

Unobangela esi sifo kukunqongophala kwezinto ethile (von Willebrand factor) equlethwe eludongeni umkhumbi ukukhonza somfanekiso kunye njengomthuthi of factor VIII (antihemophilic globulin okanye A) kwinkqubo coagulation igazi. Ngenxa yoko zokuphazamiseka zegazi adhesion eludongeni kwemithambo yegazi, agglutination lwabo omnye komnye, kunciphise cell izibalo kunye coagulation kwandiswe ixesha owophayo.

isifo von Willebrand iye iindidi eziliqela.

Type 1 ebalasele inani elingonelanga von Willebrand factor kunye nesakhiwo yemichiza ingaguqukanga. Senzeka nama-75% ezigulane kunye uxilongo. indlela Autosomal lubalaseleyo zelifa.

Uhlobo 2. tshintsho luchaphazela kubume lwendawo von Willebrand into ngokwesiqhelo lamanani egazini. Ngokwezonyango wazibona 5-15% kwizigulane. Ngaphandle iintlobo zeHPV zingonobangela eziliqela (A, B, M, N). Ilifa unako flow zombini autosomal ngendlela lubalaseleyo kunye autosomal sofuzo.

Uhlobo 3. Le fom linzima emathunjini omhlaba. Oku lubonakala ngokuba inqanaba elisezantsi kakhulu into kwi plasma vWF (ngaphantsi kwe-10% of eziqhelekileyo). Kwafunyaniswa ukuba ngaphantsi kwe-5% kwizigulane. Ilifa kwenzeka ngendlela sofuzo autosomal.

uhlobo zegazi. Ngenxa zofuzo lilifa kwi uhlobo autosomal rhoqo, kukho lunyhasho zegazi receptor isakhiwo entsonkothileyo, nangokuzikhanyeza von Willebrand into.

Uhlobo ezifunyenweyo. Oku kuvela ngethuba lobomi ngenxa yeenkqubo samathambo, luphawulwa imbonakalo autoantibodies kwi plasma yegazi von Willebrand Factor.

Ngenxa yokuba kukho iindidi multiform lwesifo von Willebrand, umfanekiso lwezonyango inokuba zahlukahlukene kakhulu. Sophele syndrome ingahluka iifomu eziphakathi ngakumbi ubukho bezinto ngeempumlo kunye gingival ukopha iintlobo kakhulu zisenzeka kunye zesibeleko enzima kunye gastro-emathunjini ukopha. Ebantwaneni, esi sifo orhanelwa ukopha ezizenzekelayo ukusuka impumlo kunye rhoqo ukugruzuka apha esikhumbeni. Ekubeni njengelifa sifo hayi ngqo kwi zofuzo ngesondo, amathuba betha efanayo nobudala. Abafazi, Noko ke, ngenxa yokuba iimpawu siseko kunye nokomzimba kumzimba, esi sifo kunzima ukuzinyamezela. Amaxesha amaninzi ukuphuhlisa menorrhagia isiba, amathuba eliphezulu Kukudandatheka legazi. Oku kungakhokelela ukuvela igazi post-ukopha. Kukho kwakhona ithuba gemoartrozov encinane, musa kungakhokelela yanyhashwa umsebenzi motor.

Indlela ukuxilonga isifo von Willebrand?

Le yinkqubo noko lula. Lokufunyaniswa kwesifo lwenzeka ngokusekelwe iisampulu ezininzi negalelo, nokumisela imeko imithambo yegazi; Uhlalutyo Blood, echaza inqanaba umsebenzi von Willebrand into, isantya coagulation igazi, ubukho kunokwenzeka of autoantibodies kwi plasma yegazi. Kuyimfuneko ukuba ndibhenele eli ngeengcebiso ngezemvelo kucacisa uhlobo ilifa kunye nengozi yokuba esi sifo kwizizukulwana ezilandelayo.

isifo Willebrand xa inganyangwa ngokusekelwe iindlela ezithile kunye non-ngqo. Xa indlela nonspecific isebenzisa ngoomatshini yokumisa ukopha, haemostatics zasekuhlaleni, aminocaproic acid (abangaphangeliyo umsebenzi kwinkqubo yegazi fibrinolytic) Dicynonum (wandisa thrombogenesis). Xa therapy ethile zingasetyenziswa kripretsipitatov eziqulathe coagulating arhente kunye neemeko ezithile amahlwili.

Ngaba kunokwenzeka ukuba ukuthintela isifo von Willebrand?

Njengoko amanyathelo othintelo ziyacetyiswa umthambo nokuthantamisa, musa ingozi yokwenzakala, kwakunye nophando phambi kokuba ekhulelwe umntwana umthi ngokwemilibo yabo usapho, amalungu ezo abathwali sifo.