Cutar Wilson

Cutar Wilson cuta ce ta gado wacce akwai tagulla a jikin kyallen takarda. Yawan jan ƙarfe yana lalata hanta da tsarin juyayi.

Cutar Wilson cuta ce da ba'a gaji irin ta ba. Idan iyaye biyu suna dauke da kwayar cutar ta cuta ta Wilson, akwai damar 25% a cikin kowane ciki cewa yaro zai sami cuta.

Cutar Wilson na sa jiki ya ɗauki kuma ya riƙe jan ƙarfe da yawa. Abun tagulla a cikin hanta, kwakwalwa, kodoji, da idanu. Wannan yana haifar da lalacewar nama, mutuwar nama, da tabo. Gabobin da abin ya shafa sun daina aiki kullum.

Wannan yanayin ya fi zama ruwan dare a gabashin Turai, Sicican, da kudancin Italiya, amma yana iya faruwa a kowane rukuni. Cutar Wilson yawanci tana bayyana ne a cikin mutane ƙasa da shekara 40. A cikin yara, alamun suna fara nunawa tun suna shekaru 4.

Kwayar cutar na iya haɗawa da:

• Matsayi mara kyau na makamai da ƙafa
• Amosanin gabbai
• Rikicewa ko hayyaci
• Rashin hankali
• Matsalar motsi hannu da kafafu, taurin kai
• Wahala tafiya (ataxia)
• Canjin motsin rai ko halayya
• Abdomenara girman ciki saboda tarin ruwa (ascites)
• Yanayin mutum yana canzawa
• Phobias, damuwa (neuroses)
• Sannu a hankali
• Sannu a hankali ko rage motsi da bayyana fuska
• Lalacewar magana
• Girgizar makamai ko hannaye
• Motsi mara izini
• Mara hangen nesa kuma mai ban tsoro motsi
• Jinin amai
• Rashin ƙarfi
• Fatar rawaya (jaundice) ko launin rawaya na fararen ido (icterus)

Gwajin fitilar ido mai haske zai iya nuna:

• Movementuntataccen motsi ido
• Ustasa mai laushi ko launin ruwan kasa mai launin ruwan kasa kewaye da ƙira (Kayser-Fleischer ring)

Gwajin jiki na iya nuna alamun:

• Lalacewa ga tsarin juyayi na tsakiya, gami da rashin daidaituwa, asarar sarrafa tsoka, rawar jiki, raunin tunani da IQ, ƙarancin ƙwaƙwalwar ajiya, da rikicewa (delirium or dementia)
• Hanta ko cututtukan hanta (gami da ciwon ciki da na ciki)

Gwajin gwaje-gwaje na iya haɗawa da:

• Kammala ƙididdigar jini (CBC)
• Magani ceruloplasmin
• Sinadarin jan ƙarfe
• Sinadarin uric acid
• Fitsarin jan ƙarfe

Idan akwai matsalolin hanta, gwajin gwaje-gwaje na iya samo:

• Babban AST da ALT
• Babban bilirubin
• Babban PT da PTT
• Albananan albumin

Sauran gwaje-gwaje na iya haɗawa da:

• 24-gwajin gwajin jan ƙarfe
• X-ray na ciki
• MRI na ciki
• CT scan na ciki
• Shugaban CT scan
• Shugaban MRI
• Gwajin hanta
• Babban GI endoscopy

An samo asalin halittar da ke haifar da cutar Wilson. An kira shi ATP7B. Akwai gwajin DNA don wannan kwayar halittar. Yi magana da mai ba da sabis na kiwon lafiya ko mai ba da shawara kan ƙwayoyin cuta idan kuna son yin gwajin jini.

Makasudin magani shine rage adadin tagulla a cikin kayan kyallen takarda. Ana yin wannan ta hanyar hanyar da ake kira chelation. Ana ba da wasu magunguna waɗanda ke ɗaure da jan ƙarfe kuma suna taimakawa cire shi ta ƙoda ko hanji. Jiyya dole ne ya kasance na rayuwa.

Ana iya amfani da magunguna masu zuwa:

• Penicillamine (kamar Cuprimine, Depen) yana ɗaure da tagulla kuma yana haifar da ƙara sakin jan ƙarfe a cikin fitsari.
• Trientine (kamar su Syprine) yana ɗaure (ƙarfe) tagulla kuma yana ƙara sakinsa ta cikin fitsari.
• Zinc acetate (kamar Galzin) yana toshe jan ƙarfe daga shafan cikin hanji.

Hakanan ana iya amfani da kari na Vitamin E.

Wani lokaci, magungunan da ke toshe jan ƙarfe (kamar su penicillamine) na iya shafar aikin kwakwalwa da tsarin juyayi (aikin jijiya). Sauran magunguna da ke ƙarƙashin bincike na iya ɗaure jan ƙarfe ba tare da shafar aikin jijiyoyin jiki ba.

Hakanan za'a iya ba da shawarar ƙarancin jan ƙarfe. Abincin da za'a guji sun haɗa da:

• Cakulan
• 'Ya'yan itacen da aka bushe
• Hanta
• Namomin kaza
• Kwayoyi
• Shellfish

Kuna iya shan ruwan da aka sha domin wasu ruwan famfo yana ratsawa ta bututun jan ƙarfe. Guji amfani da kayan girkin jan ƙarfe.

Ana iya gudanar da alamun cutar tare da motsa jiki ko magani na jiki. Mutanen da suka rikice ko suka kasa kula da kansu na iya buƙatar matakan kariya na musamman.

Ana iya yin tunanin dashen hanta a cikin yanayin da cutar ta lalata hanta sosai.

Za a iya samun kungiyoyin tallafi na cutar Wilson a www.wilsonsdisease.org da www.geneticalliance.org.

Ana buƙatar tsawon rai don sarrafa cutar Wilson. Rashin lafiyar na iya haifar da mummunan sakamako, kamar asarar aikin hanta. Copper na iya samun sakamako mai guba akan tsarin juyayi. A cikin yanayin da cutar ba ta mutu ba, alamun bayyanar na iya zama masu rauni.

Matsaloli na iya haɗawa da:

• Anaemia (anemi karancin jini)
• Tsarin rikice-rikice na tsakiya
• Ciwan Cirrhosis
• Mutuwar kayan hanta
• Hanta mai ƙoshi
• Ciwon hanta
• Chancesara yawan raunin kashi
• Numberara yawan kamuwa da cuta
• Raunin da faduwa ya haifar
• Jaundice
• Yarjejeniyar hadin gwiwa ko wata nakasa
• Rashin ikon kulawa da kai
• Rashin ikon aiki a wurin aiki da gida
• Rashin ikon hulɗa tare da wasu mutane
• Rashin ƙwayar tsoka (atrophy na tsoka)
• Rikicin ilimin halin dan Adam
• Illolin penicillamine da sauran magunguna da ake amfani dasu don magance matsalar
• Saifa matsaloli

Rashin hanta da lalacewar tsarin jijiyoyi (kwakwalwa, lakar kashin baya) sune mafi haɗari da haɗarin cutar. Idan ba a kamu da cutar ba kuma ba a magance ta da wuri, zai iya yin kisa.

Kira mai ba ku sabis idan kuna da alamun cutar Wilson. Kira mai ba da shawara kan kwayoyin halitta idan kuna da tarihin cutar Wilson a cikin danginku kuma kuna shirin samun yara.

Ana ba da shawara kan kwayoyin halitta don mutanen da ke da tarihin iyali na cutar Wilson.

Cutar Wilson; Rashin lafiyar hepatolenticular

• Tsarin juyayi na tsakiya da tsarin juyayi na gefe
• Gwajin fitsari na jan karfe
• Hanta jikin mutum

Cibiyar Nazarin Ciwon Suga ta Duniya da Cutar Kula da Lafiya da Koda. Cutar Wilson. www.niddk.nih.gov/health-information/liver-disease/wilson-disease. An sabunta Nuwamba 2018. An shiga Nuwamba 3, 2020.

Roberts EA. Cutar Wilson. A cikin: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger da Cututtukan Cutar hanta da na Fordtran. 11th ed. Philadelphia, PA: Elsevier; 2021: babi na 76.

Schilsky ML. Cutar Wilson. A cikin: Goldman L, Schafer AI, eds. Magungunan Goldman-Cecil. 26th ed. Philadelphia, PA: Elsevier; 2020: chap 200.