Zvirwere zvinowanzoitika nekuda kwechimiro chinoonekwa sevashoma, asi kwenguva refu munhu ari kurarama, anowanzomuka mukurapa.
Congenital pathologies imwe yezvinonetsa zvikuru mukurapa, nokuti zvirokwazvo pamberi pechiremba inova basa rokunyengedza zvisikwa nekugadzirisa kukanganisa kwayo.
Chirwere cheWilson-Konovalov chakatsanangurwa muna 1912 neChirungu chechirwere chechirwere Samuel Wilson, uyo akacherechedza zviratidzo zvakawanda zve hepato-cerebral dystonia, cirrhosis yechiropa uye akazvibatanidza pasi pezita rokuti "progressive lenticular degeneration."
Chirevo chechirwere ichi chiri mukuti muviri unowedzera huwandu hwakawanda hwemhangura, kureva, mumatunhu ayo anokosha zvikuru - uropi uye chiropa.
Mune chiyero chinowanikwa, mhangura inobatanidzwa mukugadzirwa kwemagetsi emagetsi, mapfupa, kugadzirwa kwegorgen uye pigment ye melanin. Asi kana kushandiswa kwemhangura kubviswa kwemhangura (uye ichi ndicho chinetso chechirwere chechirwere), chinogona kuisa upenyu pangozi. Kazhinji, mhangura inodyiwa nezvokudya uye yakanyanyiswa ne bile, mukuumbwa kwacho chiropa chinoshingaira. Kana chirwere ichi chisina kutarisirwa, ipapo chirevo hachina kunaka.
Mikana yekuvandudzwa kwechirwere cheWilson-Konovalov
Kunze kwezana remakore zviuru zana, vanachiremba vanoona hutachiwana hutatu chete. Iyo inoparidzirwa zvinyorwa zvepamusoro, izvo zvinoreva kuti mukana wekukura kwayo uri kune avo vanhu vane vabereki vose vane mutant ATP7B geni mumutambo wechi13 wekromosome. Genetics inofungidzira kuti iyi geni inenge inenge 0.6% yevanhu venyika. Muboka rinokosha rekutengesa vana vanozvarwa muukama hwepedyo.
Zviratidzo zvehosha yaWilson-Konovalov
Chirwere ichi chinogona kuzviratidza muhucheche kana kuyaruka nenzira yehutachiona hwehutachiona uye kusakundika kwechiropa.
Vanachiremba vanosiyanisa zvitatu zvechirwere ichi:
- chirwere chinokanganisa chiropa chete;
- kushungurudzika kunobata chete basa repakati yenheyo;
- ane maitiro akavhenganiswa, pane zviratidzo zvemiviri miviri.
Uyewo mune chirwere pane zvikamu zviviri, ichi rudzi rwekudzivirira nguva yehutachiona hwaWilson-Konovalov:
- rakadarika - makore mashanu;
- nzvimbo yechipatara zviratidzo.
Kune marudzi maviri ezvirwere:
- acute - muna 25% kamwe-kamwe kune jaundice, kutonhora kunowedzera, kusakondwa kweropa;
- chisingaperi - kuratidzika kunonoka kwechirwere, icho chinoitika mumakore 8-16.
Kana zvirwere zvinoputika zvichiitika, zviratidzo zvinotevera zvinoitika:
- manzi eropa;
- kuneta uye kusimba;
- kuderedza kudya uye kushanda simba;
- chigaro chisina kugadzikana uye kubvisa;
- kurwadziwa kunotyisa muropa;
- itching;
- kuwedzera kwekushisa kwemuviri;
- jaundice ;
- edema;
- kubuda ropa mukati uye mumatumbo.
Muchiitiko chekutyora kwepakati penheyo system, zviratidzo zvinotevera zvinoitika:
- kuratidzwa kwezviratidzo nezera remakore makumi maviri;
- "Masky chiso";
- copious salivation;
- dambudziko rekutaura uye kuwirirana kwemasangano;
- kudengenyeka;
- athetosis;
- hutsinye - hutsinye uye hurumende.
Pakati pezviratidzo zvakasiyana-siyana zvechirwere - kuumbwa kwemhete yakasvibirira pamucheto wekonoa weziso.
Zvinetso zvechirwere cheWilson-Konovalov
Migumisiro yechirwere cheWilson-Konovalov mukushayikwa kwehutano yakakura. Pane kukanganisa munhengo dzakawanda uye maitiro:
- ganda - rakawedzera mavara, zvikwata zvebhuruu pamisungo;
- itsvo - mabwe muitsvo , kurira kwemakumbo;
- mwoyo - rhythm kushungurudza;
- manotsi - akabatwa mu20-50% emhosva;
- mapfupa - fragility yavo inowedzera, kuputika kunowanzoitika;
- endocrine system - kunonoka mukukurudzirana zvepabonde, varume vanogona kuva nekuwedzera mumaronda emakumbo, vakadzi vane dambudziko nekutakura kwe fetus.
Kuongororwa kwechirwere cheWilson-Konovalov
Nzira dzinotevera dzinoshandiswa pakuongororwa:
- kurongedza nemwenje unopinza;
- kutsunga kwezinga re ceruloplasmin;
- kutsunga kwemhangura mumusi wega wega uye serum.
Kurapa kwechirwere cheWilson-Konovalov
Kurapa kunosanganisira zvose mishonga uye miviri yekudya:
- kudya neWilson-Konovalov chirwere-tafura nhamba ye5 uye kukwana kwemhangura kusvika 1 mg pazuva;
- chiremba Kuprenil;
- Uniatio;
- vitamini B6.