Contents
Daya daga cikin mafi halayyar ãyõyin cutar ne Pathological tarawa na jan karfe a yankin na daban-daban gabobin, nama lalacewa, musamman hanta, matsaloli na juyayi tsarin, canje-canje a cikin iris na ido.
Menene cutar Wilson-Konovalov
Kalmar Wilson-Konovalov ta cuta ne na gado Pathology. Yana faruwa ne lokacin da iyaye suka ba da wata cuta mai lahani (ATP7B) ga ɗansu. Yanayin yana nufin autosomal recessive pathologies, wato, yana faruwa idan kowane iyaye yana ɗauke da irin wannan kwayar halitta a cikin sel kuma yaron ya gaji duka kwayoyin halitta a lokaci daya - daga uwa da uba.
Wannan ɓataccen ƙwayar ƙwayar cuta yana ba da umarni don haɗin furotin wanda ke tsara musanya da jigilar jan ƙarfe a cikin jiki. Tare da lahaninsa, jan ƙarfe yana taruwa a cikin hanta, yana mai da hankali a cikin ganglia na jijiyoyi, kuma yana shiga cikin iris na ido. Pathology ba kowa ba ne, wani lokacin yana da wuyar ganewa, musamman idan babu irin waɗannan marasa lafiya a cikin iyali.
Sanadin cutar Wilson-Konovalov a cikin manya
Makullin tsari a cikin wannan ilimin cututtuka shine gado na rashin lahani daga iyaye. Yana kan chromosome na 13 kuma yana daidaita metabolism na jan karfe.
A matsakaita, jikin manya ya ƙunshi kusan 50-70 MG na jan karfe kuma yana buƙatar ba fiye da MG 2 na sinadarin kowace rana, wanda ke fitowa daga abinci.
Yawancin microelement (95%) ana canjawa wuri tare da haɗin gwiwa tare da furotin na plasma, ceruloplasmin. Hanta yana samuwa kullum, kuma kusan 5% na jan karfe ne kawai ake jigilar su tare da albumin.
Ana buƙatar Copper don shiga cikin matakai na rayuwa, ciki har da masu oxidative. Idan cutar Wilson ta tasowa, fitar da shi yana damuwa, maida hankali a cikin plasma yana ƙaruwa, daga nan ya yada zuwa kyallen takarda. Babban tarin tagulla yana faruwa a cikin kwakwalwa, a cikin yankin iris, cikin hanta, da kuma cikin koda. Yawan wuce haddi na microelement yana da tasiri mai guba.
Alamun cutar Wilson-Konovalov a cikin manya
Abubuwan da za a iya bayyana sun bambanta sosai. Mafi sau da yawa, hanta yana shan wahala (kimanin 40 - 50% na lokuta), kuma a wasu lokuta, ana iya lura da cututtuka na jijiyoyi da matsalolin tunani. Tare da lalacewa ga tsarin mai juyayi da hangen nesa, alamar alama ta bayyana - bayyanar zoben Kaiser-Fleischer (yana faruwa ne saboda ƙaddamar da jan karfe a cikin iris tare da ƙayyadaddun launin ruwan kasa).
A cikin nau'in ciwon ciki na cutar, bayyanar cututtuka yawanci suna bayyana kusa da shekaru 40. Mahimman siffofi sun haɗa da:
- cirrhosis na hanta;
- na kullum ko fulminant (fulminant) hepatitis.
A cikin ƙuruciya, bambance-bambancen cutar mai tsauri-arrhythmohyperkinetic na faruwa sau da yawa. Yana farawa da taurin kai (ƙuƙwalwa, rashin bin ƙa'ida) na tsokoki, rashin lafiyar fuska, matsalar magana, matsalolin yin motsi da ke buƙatar ƙwarewar motsa jiki, da raguwar hankali. Ciwon yana ci gaba a hankali, tare da lokuta masu tsanani da kuma gafara.
Bambancin girgiza cutar Wilson yawanci yana faruwa tsakanin shekaru 10 zuwa 30 zuwa 35. Ana iya samun bayyanar cututtuka irin su rawar jiki, raguwar motsi, jinkirin magana, tashin hankali, matsalolin tunani.
Mafi ƙarancin nau'in cutar shine cututtukan extrapyramidal-cortical. Ya yi kama da kowane nau'i, ban da haka za a sami rikice-rikice masu rikitarwa, matsalolin tunani mai tsanani, rikice-rikice na motsi.
Jiyya na cutar Wilson-Konovalov a cikin manya
Binciken farko yana da mahimmanci don ingantaccen magani. Wannan ba koyaushe ba ne mai sauƙi, musamman a cikin yanayi inda babu alamun bayyanar cututtuka da cututtukan iris tare da bayyanar zobe. Mafi sau da yawa, marasa lafiya suna zuwa wurin likitan neurologist, likitan gastroenterologist, ko kuma likitan ido ya gano matsalar.
kanikancin
Idan muna magana ne game da bayyanar cututtuka na ido, likita ya fara nazarin yanayin idanu tare da fitilun tsage don tabbatar da kasancewar zoben Kaiser-Fleischer.
Ana nuna alƙawarin gwaje-gwajen biochemical na jini da fitsari, wanda zai nuna ƙarar abun ciki na jan ƙarfe a cikin fitsari da raguwar ƙwayar ceruloplasmin a cikin jini na jini.
CT ko MRI za su nuna matakan atrophic a cikin kwakwalwa da cerebellum, lalacewa ga ƙananan ƙwayoyin basal.
Bugu da kari, ana yin shawarwari tare da masanin ilimin halittar dan adam da gwaje-gwajen kwayoyin halitta da yawa wadanda ke gano nakasassu.
Magungunan zamani
Babban hanyar maganin wannan cuta shine nada magungunan thiol, musamman unithiol ko D-penicillamine, cuprenil. Ana ɗaukar magungunan na dogon lokaci, likita ya zaɓi mafi kyawun kashi, wanda zai guje wa illa.
Bugu da ƙari, likita na iya amfani da kwayoyi daga ƙungiyar neuroleptics, tare da rigidity na tsoka - levodopa ko carbidopa.
A lokuta masu tsanani, ana nuna dashen hanta da kuma maganin rigakafi. Yana yiwuwa a yi amfani da biohemoperfusion tare da keɓance abubuwan rayuwa na salon salula na saifa tare da hanta.
Bugu da ƙari, wajibi ne a bi tsarin abinci ban da abincin da ke dauke da adadi mai yawa na jan karfe.
Rigakafin cutar Wilson-Konovalov a cikin manya a gida
"Don rigakafin cututtuka," in ji shi. Likitan neurologist Valentina Kuzmina, - wajibi ne a bi da abinci No. 5, da kuma iyakance amfani da jan karfe zuwa 1 g kowace rana - ware kwayoyi, busassun 'ya'yan itatuwa, cakulan, crayfish, biscuits, dukan alkama. Hakanan ana bada shawarar yin amfani da kwayoyi na rukunin bitamin B6, unithiol, trientine.
Shahararrun tambayoyi da amsoshi
Mun yi magana game da matsalolin da cutar Wilson-Konovalov, da rikitarwa da kuma yiwuwar kai jiyya tare da. Likitan neurologist Valentina Kuzmina.
● lalacewar hanta, musamman idan cirrhosis na hanta ya tasowa;
● rashin lafiyar hankali - mahimmancin rashin hankali, rashin hankali;
● cututtuka na jijiyoyi - rashin daidaituwa, wanda kuma akwai rawar jiki, rashin tafiya, ƙara yawan salivation.