Dambudziko reGenestic rinokonzera kukuvadza kuunganidza mhangura
Chirwere cheWilson, chinonziwo hepatolenticular degeneration, inonzi genetic disorder inoguma nokuwedzera kuwedzera kwemhangura mumuviri. Icho chisingawanzogadziriswa chinokanganisa mumwe muvanhu 30 000. Kuti munhu agadziriswe, iye anofanira kunge akagara nhaka yega genetic mutation kubva kune imwe chete asi vabereki vose.
Izvi zvisiri zvisiri izvo zvemhangura zvinowanzokonzera chiropa, uropi, hono, uye maziso asi zvinogona kukanganisa mwoyo uye endocrine system.
Zviratidzo zvehutachiona hweWilson zvinowanzoonekwa pachena pakutanga kwehupenyu, kazhinji pakati pezera remakore mashanu ne 35. Zvinetso zvechirwere zvinogona kusanganisira kusakundika kwechiropa, zvinetso zvepvo, uye dzimwe nguva zvirwere zveuropiyasiyidzi.
Zvinokonzera
Chirwere chaWilson chirwere chakazvarwa nhaka mumutambo wekuzvidzivirira . Izvi zvinorevei kuti vabereki vose vari vaviri vanotakura zvinhu zve genetic mutation, kunyange zvazvo zvisingazove zviratidzo kana nhoroondo yemhuri yechirwere ichi. Vanhu vanotakura zvinogona kunge vane uchapupu hwemhangura yemasabolism asingawanzo asi kazhinji hazvikwanisi kugadzirira kurapwa kwechiremba.
Chirwere cheWilson ndechimwe chezvirwere zvakasiyana-siyana zvehutano umo mhangura inowedzera zvisina kusimba muhurongwa, kazhinji muropa. Inosanganisira geni rinonzi ATP7B iyo muviri inoshandisa kuvhara mhangura muB bile . Kuchinja kwegene iri kunodzivirira izvi uye kunopindira nepamusoro pemhangura kubva mumuviri.
Sezvo zviyero zvemhangura zvinotanga kuderedza chiropa, muviri unoedza kuaputsa iwo pasi nekuvhara hydrochloric acid uye feri yesimbi kuti inoshandisa mamolekemu emhangura. Nokufamba kwenguva, izvi zvinogona kukonzera kuparara kweropa (fibrosis), hepatitis , uye cirrhosis.
Nemhaka yokuti mhangura inonyanya kuumbwa kwegogenjini uye kuwedzerwa kwesimbi, kusakanganiswa kupi kweizvi kunogona kukonzera kukuvadza pazera remakore.
Ichi ndicho chikonzero chirwere cheWilson chinogona kukonzera hepatitis mumakore matatu ekutanga ehupenyu uye cirrhosis (chirwere chinowanzobatanidzwa nevanhu vakuru) vari kuyaruka uye vechidiki.
Zviratidzo-Zvimwe Zvinokonzera
Zviratidzo zvechirwere cheWilson chinopesana nechinzvimbo chekuvhiringidza kwenyama. Sezvo mhangura inowanzowedzera muropa uye uropi kutanga, zviratidzo zvechirwere zvinowanzooneka zvakanyanya muzvigadzirwa izvi.
Zviratidzo zvekutanga zvechiropa zvinokanganisa zvinowanzofanana neaya anoonekwa nehepatitis . Kubudirira kwekuwedzera kwe fibrosis kunogona kutungamirira kumamiriro ezvinhu anozivikanwa sehutachiona hwehutachiona umo ropa reropa riri mukati mechiropa rinotanga kubuda. Sezvo kukanganisa kwechiropa kuchiwedzera, munhu angava nezvimwe zviitiko zvakakomba uye zvinogona kutyisa upenyu, kusanganisira kubuda mukati uye kushaya simba kwechiropa .
Pakati pezviratidzo zvinowanzobatwa neropa zvinowanikwa muhosha yaWilson:
- Kuneta
- Nausea
- Kudzora
- Kufirwa kwechido
- Muscle makampu
- Yellowing yeganda nemaziso ( jaundice )
- Akaunganidza fluid mumakumbo ( edema )
- Kubatana kwemvura mumimba ( ascites )
- Mushonga webhuta wakaita seganda (spider angiomas)
- Nhamo kana kuzara mumusoro wekumucheto pamimba nekuda kwepleen yakawedzerwa
- Kuputika kweropa kana zvigaro zvemafuro nekuda kwekudzivirira zvirwere
Kunyange zvazvo cirrhosis inowanzoitika kune vanhu vane chirwere chakakomba, chisina kutenderwa naWilson, hachiwanzofambira mberi kune kenza yeropa (kusiyana nekushungurudzika kunosangana nehutachiona hwehutachipatara kana doro ).
Neurological Symptoms
Kurwara kwechiropa zvakanyanya kunoratidzirwa nekugadzirwa kwemhando yeanemia inonzi hemolytic anemia umo masero matsvuku eropa achaputsika ndokufa. Sezvo masero matsvuku eropa ane katatu muwandu weammonia se plasma (iyo inokonzerwa neropa), kuparadzwa kwemasero aya kunogona kukonzera kukurumidza kuumbwa kweammonia nedzimwe tsvina muropa.
Kana zvinhu izvi zvichitsamwisa uropi, munhu anogona kukura nekudzikama , nekurasikirwa kweuropi kushanda nekuda kweswere chechiropa. Zviratidzo zvinogona kusanganisira:
- Migraines
- Kunyunyuta
- Kurasika kurasika
- Slurred speech
- Kuchinja pane zvakaratidzwa
- Zvinetso zvekufamba uye kurasikirwa kweruzhinji
- Kufunganya kana kuora mwoyo
- Unhu hunochinja (kusanganisira kusagutsikana uye kutadza kutonga)
- Parkinsonism (kusagadzikana, kudengenyeka, kunonoka kufamba)
- Psychosis
Nemhaka yekuti zvikonzero zvinokonzera zviratidzo izvi zvakakura, chirwere cheWilson hachiwanzoongororwa neurouropsychiatric zvinhu chete.
Zvimwe Zviratidzo
Kuunganidza kusingawanzo kwemhangura mumuviri kunogona kukanganisa zvakananga nezvimwewo dzimwe nhengo dzesangano, zvakare.
- Kana zvichiitika mukati meziso, chirwere cheWilson chinogona kukonzera chiratidzo chechimiro chinonzi Kayser-Fleisher zvindori. Izvo zvipfeko zvekutsvaira kwegoridhe zvakasvibiswa zvakapoteredza kumira kwe-iris inokonzerwa nehomwe yemhangura yakawanda. Izvo zvinowanikwa munzvimbo dzinenge 65 kubva muzana yevanhu vane chirwere cheWilson.
- Kana zvikaitika muitsvo, chirwere cheWilson chinogona kukonzera kuneta, kushaya simba kwepfungwa, kushaya mhirizhonga, mafungiro eminzwa, neropa mumurini nekuda kwekuwedzera kwechiropa muropa. Iyo mamiriro ezvinhu anogonawo kukonzera kuwedzerwa kudheta kwe calcium muitsvo uye, zvinonzwisisika, kushaya simba kwemapfupa nekuda kwekuregererwa nekurasikirwa kwe calcium.
Kunyange zvazvo zvisingawanzoitiki, chirwere cheWilson chinogona kukonzera kushaya mwoyo (kushaya simba kwemoyo) pamwe nekushaya simba uye kudzokorora kuberekwa semusana wehutachiona hwehutachiona.
Kuziva
Nemhaka yehutano hwakasiyana hwezvingave zviratidzo, chirwere cheWilson chinowanzova chakaoma kuongorora. Kunyanya kana zviratidzo zvisinganzwisisiki, chirwere chacho chinogona nyore kukanganisa zvinhu zvose zvichibva kune zvakanyanya zvakasvibirira chesimbi uye hepatitis C kumishonga-inokonzerwa lupus uye cerebral palsy.
Kana chirwere chaWilson chichifungidzirwa, kupfupisa kuchabatanidza kuongorora kwezviratidzo zvepanyama pamwe nekuwanda kweongororo yekuongorora, kusanganisira:
- Liver enzyme test
- Ropa kuongororwa kuti riongorore hurefu hwakawanda hwemhangura uye pasi mazinga e ceruloplasmin (protini inotakura mhangura kuburikidza neropa)
- Ropa glucose inoedzwa kuti iongorore huwandu hwehuga hwepasi
- Maminetsi makumi maviri nemasere ekuunganidza kukosha kwe acidity uye calcium levels
- Chiropa chinotsvaga kuti chione kukosha kwemhangura kuunganidza
- Genetic tests kuti kusimbise kuvapo kwekuchinja kwe ATB7B
Kurapa
Kutanga kufungidzirwa kwechirwere cheWilson kazhinji kunopa zvibereko zviri nani. Vanhu vanowanikwa vane chirwere zvinowanzobatwa mumatanho matatu:
- Mishonga inowanzotanga nekushandiswa kwemhangura- chelating madhiragi kubvisa mhangura yakawandisa kubva mumutambo. Penicillamine inowanzotanga kushandisa mishonga yekutanga. Inoshanda nekusunga nemhangura, zvichibvumira kuti simbi iwedzere kusunungurwa mumuguta. Zvimwe zvikanganiso dzimwe nguva zvinokosha uye zvingasanganisira musimba utera, kupera, uye marwadzo akabatana. Pakati peavo vane zviratidzo, 50 muzana vachaona kuwedzera kwezviratidzo. Mukudaro, mishonga yechipiri inogona kunyorwa.
- Kana imwe yenguva yemhangura yakasikwa, zinc inogona kurongerwa sechimiro chekuchengetedza chirwere. Zinc dzinotorwa nemuromo dzinodzivirira muviri kuti urege kutora mhangura. Dumbu recheche ndiyo yakawanda yakashata.
- Zvokudya zvinogadzirisa kuti iwe usadzima kudya zvisina kuvezwa mhangura. Izvi zvinosanganisira izvo zvakagadzirwa nemhangura-pfuma se shellfish, chiropa, marimu, mushwa, michero yakaoma, peanut butter, uye chocolate tsvuku. Mhangura-ine zvinowedzera, zvakadai seyentivitamines uye izvo zvinoshandiswa pakurapa osteoporosis, zvinogonawo kushandiswa.
Vanhu vane chirwere chakakomba chechiropa vanokundikana kupindura kurapwa kunoda chiropa chokupemha .
> Sources:
> Borges Pinto, R .; Reis Schneider, A .; uye da Silveira, T. "Cirrhosis muvana nevechiri kuyaruka: Mhedziso yepamusoro." World Journal of Hepatology. 2015; 7 (): 392-405.
> Patil, M .; Sheth, K .; Krishnamurthy, A. et al. "Ongororo uye Parizvino Pfungwa paWilson Disease." Zvinyorwa zveKliniki uye Ongororo Hepatology. 2013; 3 (4): 321-336.