Isifo Wilson: izimbangela, izimpawu, zokwelapha nokuhlola

isifo Wilson (ukonakala hepatolenticular, uWilson syndrome - Konovalov) - a sifo esingavamile esidluliselwa ngofuzo okuyinto lihlotshaniswa kahle zethusi umzimba emzimbeni futhi kuhambisana isibindi isimiso sezinzwa esiyinhloko.

Ngokokuqala ngqá lezi zimpawu alotshwa 1883. Izenzo sifo iyefana izimpawu ka-multiple sclerosis, ngakho ngesikhathi lesi sifo siye sabizwa ngokuthi "psevdosklerozom". Ukulifunda ngijule le nkinga wahlanganyela wezinzwa oyiNgisi uSamuel uWilson, ongomunye ngo-1912 echazwe emtholampilo isifo cishe ngokuphelele.

isifo Wilson: Izimbangela

Njengoba kushiwo, lesi sifo zofuzo sidlulile kumzali siye enganeni ngendlela autosomal esikhubazayo. Ukwephula zethusi umzimba ngenxa ngokuguqula izakhi isakhi ise ingalo eside chromosome nantathu. Kuyinto yilesi sakhi buqonda ifomu amaprotheni ezithile ATPase, okuyinto unomthwalo ukuthuthwa yethusi yamiswa ku ceruloplasmin.

Izizathu ukuguquka kwezakhi zofuzo ezifana azikaqondakali ngokugcwele. Singathi nje ukuthi isifo Wilson sitholakale kaningi phakathi kwezizwe lapho imishado ezivamile kwezihlobo eziseduze. Ngaphezu kwalokho, lokhu sifo ngokuvamile kutholakala ukuthi uphethwe yini phakathi amadoda nabafana, ogama yobudala amabanga kusukela engu-15 kuya ku-25.

isifo Wilson: izimpawu eziyinhloko

Lesi sifo angase ukuzibonakalisa ngezindlela ezahlukene. Ngenxa yalokho zethusi umzimba aqoqana emzimbeni womuntu, ngaleyo ndlela eziphatha sesibindi nuclei we-lenticular wangakwesokunxele diencephalon.

In iziguli, lesi sifo ukuzibonakalisa noma isibindi noma izimpawu zokungasebenzi kahle kwemizwa. Ngezinye izikhathi kukhona ubuhlungu emalungeni, uphathwa umkhuhlane omubi nomzimba nobuhlungu bomzimba. Ukuthola zethusi ngokuhamba kwesikhathi uma iyekwa ingelashwa kungaholela isifo sokusha. Kwezinye izimo, kuqala wabonakaliswa izimpawu zokungasebenzi kahle kwemizwa - noshintsho ekuziphatheni kanye nesimo ngokomzwelo, kanye ukuzamazama. Ngeshwa, kaningi izimpawu zesifo zibonakala njengoba kubonakaliswa engenakugwenywa.

ithusi kungelashwa aqoqana emzimbeni okuholela iziyaluyalu eziningi kakhulu, kuhlanganise isifo sikashukela, aneurysms, isifo sokuqina kwemithambo yegazi, futhi yamathambo.

isifo Wilson - Konovalova: Ihlahlubo

Ukuxilongwa lesi sifo kufanele ngempela zihlanganisa igazi kwamakhemikhali. Phakathi laboratory ukuhlolwa isampula yegazi uyinaka ezingeni zethusi: yegazi ponizhet njengoba zonke molecule solute buthelela emisipheni. Ngesinye isikhathi kuyadingeka futhi isibindi biopsy. Esikhathini amasampula cwaningo kwezicubu isibindi ukunquma inani lelitfutfukile zethusi.

Kukhona elinye futhi iphuzu ezibalulekile zokuxilonga. Uma kubhekwa ngasohlangothini amehlo iziguli ku-cornea ungabona izindandatho sinombala - kungcono uphawu okubizwa ngokuthi Kaiser - Fleischer.

isifo Wilson - Konovalova: ukwelashwa

Kuze kube manje, ukwelashwa ngempumelelo kuphela kuprenil, ngesisekelo okwenziwé zonke izidakamizwa ekhona. Le into kunciphisa amazinga zethusi emzimbeni futhi kuthatha phezu. Lahla isifo akukwazi phakade. Kodwa lokhu yokwelapha ivumela iziguli ukuba ahole evamile, ukuphila okumatasa.

Ngeshwa, uma isifo Wilson itholwa Wakhumbula umonakalo emzimbeni, ikakhulukazi nezicubu sezinzwa engelapheki kakade. Sekuyisikhathi ke kubalulekile ukuxilonga izifo futhi baqala ukwelashwa.