Chirwere cheWilson

Chirwere cheWilson idambudziko rekugara nhaka umo mune mhangura yakawandisa mumatumbu emuviri. Iyo yakawandisa mhangura inokuvadza chiropa uye huta system.

Chirwere cheWilson chirwere chisingawanzo kuwanikwa nhaka. Kana ivo vaviri vabereki vakatakura jenenzi isina kukwana yeWilson chirwere, pane 25% mukana mune yega yega nhumbu kuti mwana achave nedambudziko.

Chirwere cheWilson chinokonzeresa kuti muviri utore uye uchengete mhangura yakawandisa. Iyo mhangura inoisa muchiropa, huropi, itsvo, uye maziso. Izvi zvinokonzeresa kukuvara kwemaviri, kufa kwemashuga, uye vanga. Nhengo dzakakanganiswa dzinomira kushanda zvakajairika.

Aya mamiriro anowanzoitika kumabvazuva kweEuropean, Sicilians, uye maodzanyemba maItaly, asi zvinogona kuitika mune chero boka. Chirwere cheWilson chinowanzoonekwa muvanhu vari pasi pemakore makumi mana ekuberekwa. Muvana, zviratidzo zvinotanga kuratidza nezera rechina.

Zviratidzo zvinogona kusanganisira:

• Kusagadzikana kwemaoko nemaoko nemakumbo
• Arthritis
• Kuvhiringidzika kana delirium
• Dementia
• Zvakaoma kufambisa maoko nemakumbo, kuomarara
• Kunetseka kufamba (ataxia)
• Emotional kana maitiro ekuchinja
• Kuwedzera kwedumbu nekuda kwekuunganidzwa kwemvura (ascites)
• Unhu hunochinja
• Phobias, kushushikana (neuroses)
• Zvishoma nezvishoma kufamba
• Inononoka kana kudzikira kufamba uye kutaura kwechiso
• Kukanganisa kutaura
• Kudedera kwemaoko kana kwemaoko
• Kufambisa kusingadzoreki
• Chisingatarisirwe uye kufamba kwejeje
• Kusvipa ropa
• Kushaya simba
• Ganda jena (jaundice) kana yero yeruvara chena yeziso (icterus)

Bhatiri-rambi ziso bvunzo rinogona kuratidza:

• Kufamba kwemaziso kushoma
• Rhete yakasviba kana yeruvara-shava yakakomberedza iris (Kayser-Fleischer mhete)

Kuongorora kwepanyama kunogona kuratidza zviratidzo zve:

• Kukuvara kwenzvimbo yepakati yetsinga, kusanganisira kurasikirwa kwekubatana, kurasikirwa kwekudzora tsandanyama, kudedera kwemhasuru, kurasikirwa kwekufunga neIQ, kurasikirwa ndangariro, uye kuvhiringidzika
• Chiropa kana chirwere cheperene (kusanganisira hepatomegaly uye splenomegaly)

Bvunzo bvunzo dzinogona kusanganisira:

• Yakazara kuverenga ropa (CBC)
• Serum ceruloplasmin
• Serum mhangura
• Serum uric acid
• Weti mhangura

Kana paine matambudziko echiropa, bvunzo bvunzo dzinogona kuwana:

• Yakakwira AST uye ALT
• Yakakwirira bilirubin
• Yakakwirira PT uye PTT
• Yakaderera albumin

Mimwe bvunzo inogona kusanganisira:

• 24-awa weti weti mhangura bvunzo
• Abdominal x-ray
• Abdominal MRI
• CT scan yemudumbu
• Musoro CT scan
• Musoro MRI
• Chiropa biopsy
• Kumusoro GI endoscopy

Iyo geni inokonzera chirwere cheWilson yawanikwa. Inodaidzwa ATP7B. Kuongororwa kweDNA kunowanikwa kune iri geni.Taura nemupi wako wezvehutano kana wezano kana iwe uchida kuti kuongororwa kwemajini kuitwe.

Chinangwa chekurapa ndechekuderedza huwandu hwendarira mumatumbu. Izvi zvinoitwa nenzira inonzi chelation. Mimwe mishonga inopihwa inosunga mhangura uye inobatsira kuibvisa kuburikidza neitsvo kana ura. Kurapa kunofanirwa kuve kwehupenyu hwese.

Iyi inotevera mishonga inogona kushandiswa:

• Penicillamine (senge Cuprimine, Depen) inosunga kune mhangura uye inotungamira mukuwedzera kuburitswa kwemhangura mune weti.
• Trientine (senge Syprine) inosunga (chelates) iyo mhangura uye inowedzera kuburitswa kwayo kuburikidza neweti.
• Zinc acetate (yakadai seGalzin) inovhara mhangura kuti isanyurwe mumatumbu.

Vitamin E zvinowedzera zvinogona zvakare kushandiswa.

Dzimwe nguva, mishonga inobaya mhangura (senge penicillamine) inogona kukanganisa mashandiro ehuropi uye nehurongwa hwetsinga (neurological function). Mimwe mishonga iri kuongororwa inogona kusunga mhangura isingakanganise basa retsinga.

Kudya kwakadzika-kwemhangura kunogona zvakare kukurudzirwa. Zvikafu zvekudzivisa zvinosanganisira:

• Chokoreti
• Muchero wakaomeswa
• Chiropa
• Howa
• Nuts
• Hove

Unogona kuda kunwa mvura yakadonhedzwa nekuti imwe mvura yemupombi inoyerera nepombi dzemhangura. Dzivisa kushandisa midziyo yekubikisa yemhangura.

Zviratidzo zvinogona kutarisirwa nekurovedza muviri kana kurapwa kwemuviri. Vanhu vakavhiringidzika kana vasingakwanise kuzvitarisira vanogona kutora matanho ekudzivirira.

Kuiswa kwechiropa kunogona kutariswa muzviitiko apo chiropa chakakuvara zvakanyanya nechirwere.

Mapoka eWilson ekutsigira chirwere anogona kuwanikwa pawww.wilsonsdisease.org uye www.geneticalliance.org.

Hupenyu-hwakareba kurapwa kunodiwa kudzora chirwere cheWilson. Iyo kusagadzikana kunogona kukonzera kuuraya mhedzisiro, senge kurasikirwa kwechiropa basa. Mhangura inogona kuve nehuturu pamhedzisiro. Mumamiriro ezvinhu apo kusagadzikana kusingauraye, zviratidzo zvinogona kunge zvichiremadza.

Zvinetso zvinogona kusanganisira:

• Anemia (hemolytic anemia isingawanzo)
• Central nervous system zvinetso
• Cirrhosis
• Kufa kwezvironda zvechiropa
• Chiropa chakakora
• Hepatitis
• Kuwedzera mikana yekutyoka kwemapfupa
• Kuwedzera kwehutachiona
• Kukuvara kunokonzerwa nekudonha
• Jaundice
• Joint contractures kana kumwe kuora
• Kurasikirwa kwekugona kuzviriritira
• Kurasikirwa kwekugona kushanda kubasa uye kumba
• Kurasikirwa kwekugona kudyidzana nevamwe vanhu
• Kurasikirwa kwemhasuru (mhasuru atrophy)
• Zvinetso zvepfungwa
• Zvimwe zvinokanganisa penicillamine uye mimwe mishonga inoshandiswa kurapa kusagadzikana
• Matambudziko epa spleen

Kukundikana kwechiropa uye kukuvadzwa kwepakati sisitimu (huropi, musana) ndizvo zvinowanzoitika uye zvine njodzi mhedzisiro Kana chirwere chisina kubatwa nekurapwa nekukasira, chinogona kuuraya.

Fonera mupiki wako kana iwe uine zviratidzo zvechirwere cheWilson. Shevedza genura chipangamazano kana iwe uine nhoroondo yechirwere cheWilson mumhuri yako uye urikuronga kuita vana.

Genetic counselling inokurudzirwa kune vanhu vane nhoroondo yemhuri yechirwere cheWilson.

Chirwere chaWilson; Hepatolenticular kudzikira

• Central nervous system uye yekumusoro tsinga system
• Mhangura weti bvunzo
• Chiropa anatomy

National Institute of Chirwere cheshuga uye Digestive uye Kidney Zvirwere webhusaiti. Chirwere cheWilson. www.niddk.nih.gov/health-information/liver-disease/wilson-disease. Yakagadziridzwa Mbudzi 2018. Yakasvika munaNovember 3, 2020.

Roberts EA. Chirwere cheWilson. Muna: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger uye Fordtran's Gastrointestinal uye Chirwere Chiropa. 11th ed. Philadelphia, PA: Elsevier; 2021: chits. 76.

Schilsky ML. Chirwere cheWilson. Mune: Goldman L, Schafer AI, eds. Goldman-Cecil Mushonga. 26th ed. Philadelphia, PA: Elsevier; 2020: chitsama 200.