Von Willebrand chirwere

Angiohemophilia, kana, mune mamwe mazwi, chirwere of Willebrand raMwari (uyewo akanyorwa nezvake wake "atrombotsitopenicheskaya purpura") - ropa chirwere dzinoratidzwa kuderera kweropa uye nhaka ive autosomal rave kana recessive nenzira automno. zvokurwara ichi kunoitika ane kakawanda 1: 1000. Hunotarisirwa upenyu vanhu vanorwara haina kunoderedza asi pane nguva dzose refu ndingangokuvara kuva ropa upenyu pangozi.

Chinokonzera chirwere ichi ndicho kushaya imwe pfuma (of Willebrand chinhu) huri parusvingo mudziyo uye kuti ave stabilizer uye transporter kuti chinhu VIII (antihemophilic globulin, kana A) ropa coagulation hurongwa. Somugumisiro anetseka platelet adhesion pamadziro nemidziyo ropa, ravo agglutination vakataurirana, akaderedza eropa Counts uye coagulation kwakawedzera ropa nguva.

chirwere Von Willebrand rine mhando dzakawanda.

Type 1 iri ichazivikanwa noku kukwana uwandu of Willebrand chinoita pamwe kuchinja namakemikari mamiriro. It unowanikwa anenge 75% varwere nechirwere. Autosomal chikuru mufananidzo nhaka.

Type 2. Kuchinja zvazvinoita spatial maumbirwo of Willebrand chinhu chakaipa ropa kuwanda. Akaora kuchengetwa 5-15% wevarwere. Kunopiwa dzakawanda subtypes (A, B, M, N). Nhaka anogona yerera zvose autosomal rave uye autosomal recessive netsika.

Type 3. yakaomesesa runoyerera chimiro. It dzinoratidzwa pasi kwazvo pamwero chinhu yeplasma vWF (asingasviki 10% kubva dzose). Chirwere asingasviki 5% wevarwere. Nhaka kunoitika munzvimbo autosomal recessive nenzira.

Platelet mhando. Nokuda kunotouraya kuti vari nhaka munzvimbo autosomal-hune mhando, pane kutyora platelet Kanotokonya yakaoma yakarongeka, ndichisunga kuna of Willebrand chikonzero.

The vawana mhando. It unovapo mukufamba upenyu semubairo autoimmune urongwa, dzinoratidzwa chimiro autoantibodies vari plasma of Willebrand Factor.

Nokuda kuti pane multiform mhando of Willebrand chirwere, ari kurapwa mufananidzo angava siyana chaizvo. Hemorrhagic chirwere dzinosiyana nyoro siyana ane predominance pamusoro nasal uye gingival ropa kuti zvikuru siyana kuitika pamwe rinorema uterine uye gastro-intestinal kubuda ropa. In vana, chirwere chinogona vaifungidzirwa vaingoerekana ropa kubva mhino uye kakawanda kupwanywa paganda. Sezvo nhaka chirwere haisi vakanamatira zvepabonde okutodza, kuti ndingangokuvara kurova saizvozvo aroorane. Women, zvisinei, nokuda kweBumbiro uye vasiri zvechadenga muviri, chirwere zvakaoma kuti kushivirira. Kazhinji kuva inoremadza menorrhagia, yakakwirira ndingangokuvara postpartum ropa. Izvi zvingaita kuti kwaita post-Hemorrhagic kupererwa neropa. Panewo diki mukana gemoartrozov, haanamati zvakakomba kutyorwa motokari chaitika.

How rokuongorora of Willebrand chirwere?

Uyu kumbonyanya nyore kwacho. Chirwere chirwere kunoitika pachishandiswa vakawanda paku ivhu, kuziva mamiriro midziyo ropa; Blood pachisara iro rinozivisa padanho basa of Willebrand chinhu, ropa coagulation kumhanya, pangava kuvapo autoantibodies muropa plasma. Zvakakodzera kukumbira kune majini mazano kuona chimiro nhaka uye pangozi yokuita chirwere mu tevera nezvizvarwa.

chirwere Willebrand anogona kurapwa pachishandiswa chaiyo uye zvisiri Nzira dzinoshandiswa. Kana nonspecific kusvika anoshandisa zvokuimba vanorega ropa, dzemunharaunda haemostatics, aminocaproic asidhi (kuchititadzisa basa fibrinolytic ropa hurongwa) Dicynonum (boosts thrombogenesis). Kana chaiwo mushonga inogona kushandiswa kripretsipitatov ane coagulating mumiririri uye vamwe pakugwamba zvinhu.

Zvinoita kudzivirira of Willebrand chirwere?

Sezvo matanho vari varumbidzwa munyoro muviri, musatakura njodzi yokukuvadzwa, uyewo kutsvakurudza usati yokubereka mwana mazita muti mhuri, nhengo vari vatakuri chirwere.