Wilson nechirwere: zvinokonzera, zviratidzo, kurapa uye Diagnostics

chirwere Wilson kuti (hepatolenticular degeneration, Wilson chirwere - Konovalov) - isingawanzowanikwi nemagene chirwere icho chokuita vasingadzoreki mhangura muviri mumuviri uye pamwe chiropa uye nechepakati tsinga.

Kokutanga zviratidzo izvi zvakanyorwa muna 1883. Zvinoratidza chirwere dzakafanana zviratidzo multiple sclerosis, saka panguva iyo chirwere wakanzi "psevdosklerozom". Depth kudzidza dambudziko iri vachipa Chirungu wetsinga Samuel Wilson, uyo muna 1912 akarondedzera kiriniki inenge chose chirwere.

Wilson chirwere: Zvinokonzera

Sezvambotaurwa, chirwere ichi kuvabereki uye wabva mubereki mwana zvine autosomal recessive nenzira. Kukanganisa hwemhangura muviri nokuda mumwe mutation mumwe Gene riri refu ruoko chromosome namatatu. Hazvina Gene ichi inofananidzira ane puroteni chaicho muchimiro ATPase, riri basa chokufambisa yemhangura wayo kukumbanidzwa ceruloplasmin.

Zvikonzero kunotouraya vakadaro haasati ainyatsonzwisisa. Tinogona chete kutaura kuti chirwere Wilson chiri chirwere kakawanda pakati pamarudzi vanhuwo michato kwehama dzepedyo. Uyezve, chirwere ichi kakawanda chirwere pakati pavanhu uye vakomana, ane zera neeSultan kubva 15 kusvika 25 makore.

Wilson nehosha: huru zviratidzo

The chirwere anogona kuoneka nenzira dzakasiyana. Somugumisiro metabolic mhangura rinoungana mumuviri womunhu, nokudaro kukanganisa chiropa uye lenticular nuclei diencephalon.

In varwere, chirwere kungazviratidza kana chiropa kana chetsinga zviratidzo. Dzimwe nguva panewo marwadzo majoini, nefivha uye muviri pari. Kuunganidza mhangura pamusoro nguva kana kuruboshwe untreated kunogona kutungamirira cirrhosis. Mune zvimwe zviitiko, wokutanga richiratidzwa chetsinga zviratidzo - kuchinja maitiro uye kunetsa, uyewo chakamira pakunodengenyeka zvine. Zvinosuruvarisa kazhinji zviratidzo chirwere zvinoonekwa sezvinhu kuratidzwa pakuyaruka.

Untreated mhangura rinoungana mumuviri kunotungamirira zvinetso dzisingaverengeki, kusanganisira chirwere cheshuga, aneurysms, atherosclerosis, uye kusimba.

Wilson nehosha - Konovalova: kuongorora chirwere

Chirwere ichi vanofanira hazvo zvinosanganisira ropa kemisitiri. Panguva Murabhoritari kuidzwa ropa yokuenzanisira kubhadhara pfungwa padanho mhangura: ropa ponizhet sezvo zvose solute mamorekuru uchingounganidza munyama. Dzimwe nguva zvakakodzera uye chiropa biopsy. Kudzidza mimwe chiropa rinenge kuona kuwedzera uwandu hwemhangura.

Pane imwe inokosha chirwere pfungwa. Kana ukatarira meso varwere pamusoro cornea anogona kuona brownish zvindori - ndiwo chiratidzo inonzi Kaiser - Fleischer.

Wilson nehosha - Konovalova: kurapwa

Kusvika panguva chete inoshanda kurapwa kuprenil, pahwaro yakaitwa aivapo zvinodhaka zvose. fuma iyi kunoderedza siyana mhangura mumuviri uye anotora nayo pamusoro. Bvisai chirwere havagoni kuva nokusingaperi. Asi mushonga uyu zvinobvumira varwere kutungamirira hwandaiva, kushingaira upenyu.

Zvinosuruvarisa kana chirwere Wilson ari kuona yapera, kukuvara mumuviri, kunyanya kune huta zvinyama kare kusingarapiki. Saka zvinokosha nguva rokuongorora chirwere uye kutanga kurapwa.