Matenda Wilson: zimayambitsa, zizindikiro, chithandizo diagnostics

matenda Wilson (hepatolenticular alibe, Wilson syndrome - Konovalov) - ochepa chabe majini matenda amene amagwirizana ndi kagayidwe chakhungu mkuwa thupi limodzi ndi chiwindi ndi dongosolo chapakati mantha.

Kwa nthawi yoyamba zizindikiro analembedwa 1883. Kuwonekera kwa matenda ofanana ndi zizindikiro za ofoola ziwalo, kotero pa nthawi matenda umatchedwa "psevdosklerozom". Mozama vutoli chinkhoswe English katswiriyu Samuel Wilson, yemwe 1912 anafotokoza chipatala pafupifupi kwathunthu matenda.

matenda Wilson: Zoyambitsa

Monga tanenera, matenda ndi majini ndi wadutsa kuchokera kholo mwana mu autosomal unsembe recessive. Kulakwila kagayidwe mkuwa chifukwa kusintha kwa khalidwe ili mu dzanja yaitali khromozomu khumi ndi chitatu. Ndi jini izi encodes mapuloteni mawonekedwe enieni ATPase, amene ali ndi udindo kwa zoyendera zamkuwa ndi kukhazikitsidwa kwake mu ceruloplasmin.

Chifukwa masinthidwe amenewa bwino. Tikhonza kunena kuti matenda Wilson amawapeza zambiri pakati pa mitundu ina kumene mabanja wamba pakati pa achibale. Komanso, matenda awa zambiri anapezeka pakati pa amuna ndi anyamata, amene zaka ranges kuchokera zaka 15 mpaka 25.

matenda Wilson: zizindikiro zazikulu

matenda kungaonekere m'njira zosiyanasiyana. Chifukwa cha mkuwa kagayidwe kachakudya amasonkhana mu thupi la munthu, motero okhudza chiwindi ndi mtima lenticular diencephalon.

Odwala matendawa angasonyeze kapena chiwindi kapena zizindikiro ubongo. Nthawi zina palinso ululu malo, malungo ndi zopweteka thupi. Kudzikundikira mkuwa pa nthawi ngati sanalandire chithandizo kungayambitse matenda enaake. Nthawi zina, woyamba anasonyeza zizindikiro ubongo - kusintha khalidwe ndi boma maganizo, komanso inagwedezeka. Mwatsoka, nthawi zambiri matenda a zimawoneka ngati maonekedwe a unyamata.

mkuwa Popanda kuchitapo kanthu amasonkhana thupi yopita ku matenda ambiri kuphatikizapo matenda a shuga, aneurysms, atherosclerosis, ndi rickets.

matenda Wilson - Konovalova: Matendawa

Matenda a matenda ayenera monga umagwirira magazi. Pa zasayansi chiyesedwe cha chitsanzo magazi kusamalira kwambiri mlingo wa mkuwa: mwazi ponizhet monga mamolekyulu onse solute kudziunjikira mu zimakhala ndi. Nthawi zina m'pofunika ndi chiwindi biopsy. Mu zitsanzo kuphunzira minofu chiwindi kudziwa bwino kuchuluka kwa mkuwa.

Pali china chofunika matenda mfundo. Kuwaona kwa maso a odwala pa diso aone mphete brownish - ndi chizindikiro cha otchedwa Kaiser - Fleischer.

matenda Wilson - Konovalova: chithandizo

Chibwenzi yekha mankhwala ndi kuprenil, pa maziko a amene anapanga mankhwala onse alipo. mankhwala kuchepetsa kuchuluka kwa mkuwa thupi ndipo akutenga izo. Tayani matenda sangakhale muyaya. Koma mankhwala amenewa kumathandiza odwala kutsogolera munthu wabwinobwino, achangu.

Mwatsoka, ngati matenda Wilson ali wapezeka mochedwa, kuwonongeka kwa thupi, makamaka wamanjenje minofu kale sizingasinthe. Choncho n'kofunika nthawi matenda ndi kuyamba chithandizo.