Matenda a Wilson

Matenda a Wilson ndi matenda obadwa nawo momwe mumakhala mkuwa wochuluka kwambiri m'matumba amthupi. Mkuwa wochulukirapo umawononga chiwindi ndi dongosolo lamanjenje.

Matenda a Wilson ndi matenda obadwa nawo. Ngati makolo onse ali ndi jini losalongosoka la matenda a Wilson, pali mwayi 25% pamimba iliyonse kuti mwanayo akhale ndi vutoli.

Matenda a Wilson amachititsa kuti thupi lilowemo ndikusunga mkuwa wambiri. Mkuwa umayika m'chiwindi, ubongo, impso, ndi maso. Izi zimayambitsa kuwonongeka kwa minofu, kufa kwa minyewa, ndi zipsera. Ziwalo zomwe zakhudzidwa zimasiya kugwira ntchito mwachizolowezi.

Vutoli limapezeka kwambiri kum'mawa kwa Europe, Sicilians, ndi kumwera kwa Italiya, koma limatha kuchitika pagulu lililonse. Matenda a Wilson amapezeka mwa anthu ochepera zaka 40. Kwa ana, zizindikilo zimayamba kuwonekera pofika zaka 4.

Zizindikiro zimaphatikizapo:

• Kukhazikika kwama mikono ndi miyendo
• Nyamakazi
• Kusokonezeka kapena kusokonezeka
• Kusokonezeka maganizo
• Zovuta kusuntha mikono ndi miyendo, kuuma
• Kuvuta kuyenda (ataxia)
• Kusintha kwamalingaliro kapena kakhalidwe
• Kukulitsa kwam'mimba chifukwa chakudzikundikira kwamadzimadzi (ascites)
• Umunthu umasintha
• Phobias, mavuto (neuroses)
• Kusuntha pang'ono
• Kuchedwa kapena kuchepa kwa mayendedwe ndi mawonekedwe a nkhope
• Kuwonongeka kwamalankhulidwe
• Kugwedezeka kwa manja kapena manja
• Kuyenda kosalamulirika
• Kuyenda kosayembekezereka komanso kosasunthika
• Kusanza magazi
• Kufooka
• Khungu lachikaso (jaundice) kapena mtundu wachikaso loyera la diso (icterus)

Kuyezetsa diso kwa nyali kungasonyeze:

• Kuyenda kochepa kwa diso
• Mphete yofiira kapena yofiirira kuzungulira iris (mphete za Kayser-Fleischer)

Kuyezetsa thupi kumatha kuwonetsa zizindikilo za:

• Kuwonongeka kwa mitsempha yapakatikati, kuphatikizapo kuchepa kwa mgwirizano, kutayika kwa minofu, kunjenjemera kwa minofu, kutaya malingaliro ndi IQ, kusaiwalika, ndi chisokonezo (delirium kapena dementia)
• Matenda a chiwindi kapena ndulu (kuphatikizapo hepatomegaly ndi splenomegaly)

Mayeso a labu atha kuphatikiza:

• Kuwerengera kwathunthu kwa magazi (CBC)
• Seramu ceruloplasmin
• Seramu mkuwa
• Seramu uric acid
• Mkodzo mkuwa

Ngati pali mavuto a chiwindi, mayeso a labu angapeze:

• Mkulu AST ndi ALT
• Mkulu bilirubin
• Mkulu PT ndi PTT
• Otsika albin

Mayesero ena atha kuphatikizira:

• Kuyesa mkodzo kwa maola 24
• X-ray m'mimba
• M'mimba mwa MRI
• CT scan pamimba
• Mutu wa CT
• Mutu wa MRI
• Chiwindi
• Pamapeto pake GI endoscopy

Jini lomwe limayambitsa matenda a Wilson lapezeka. Amatchedwa Zamgululi. Kuyezetsa DNA kulipo pa jeni ili.Lankhulani ndi omwe amakuthandizani azaumoyo kapena mlangizi wamtundu ngati mungafune kuyezetsa majini.

Cholinga cha chithandizo ndikuchepetsa mkuwa m'matumba. Izi zimachitika ndi njira yotchedwa chelation. Mankhwala ena amaperekedwa omwe amangirira mkuwa ndikuthandizira kuwachotsa kudzera mu impso kapena m'matumbo. Chithandizo chiyenera kukhala cha moyo wonse.

Mankhwala otsatirawa atha kugwiritsidwa ntchito:

• Penicillamine (monga Cuprimine, Depen) amamangirira mkuwa ndipo amatsogolera kukutulutsani mkuwa mumkodzo.
• Trientine (monga Syprine) imamanga (chelates) mkuwa ndikuwonjezera kutulutsidwa kwake mkodzo.
• Zinc acetate (monga Galzin) imatchinga mkuwa kuti usatengeke m'matumbo.

Vitamini E amathanso kugwiritsidwa ntchito.

Nthawi zina, mankhwala omwe amatulutsa mkuwa (monga penicillamine) amatha kukhudza magwiridwe antchito a ubongo ndi zamanjenje (neurological function). Mankhwala ena omwe akufufuzidwa amatha kumanga mkuwa popanda kukhudza magwiridwe antchito.

Zakudya zamkuwa zochepa zingalimbikitsidwenso. Zakudya zomwe muyenera kupewa ndi izi:

• Chokoleti
• Zipatso zouma
• Chiwindi
• Bowa
• Mtedza
• Nkhono

Mungafune kumwa madzi osungunuka chifukwa madzi ena apampopi amayenda m'mapaipi amkuwa. Pewani kugwiritsa ntchito ziwiya zophikira zamkuwa.

Zizindikiro zimatha kuyang'aniridwa ndi masewera olimbitsa thupi kapena mankhwala. Anthu omwe asokonezeka kapena sangathe kudzisamalira angafunike njira zina zodzitetezera.

Kuika chiwindi kumatha kuganiziridwa ngati chiwindi chawonongeka kwambiri ndi matendawa.

Magulu othandizira a Wilson amapezeka ku www.wilsonsdisease.org ndi www.geneticalliance.org.

Chithandizo cha moyo wonse chikufunika kuti muchepetse matenda a Wilson. Matendawa amatha kupha, monga kuchepa kwa chiwindi. Mkuwa ukhoza kukhala ndi zotsatira zoyipa pamanjenje. Nthawi yomwe matendawa sakupha, zizindikilo zimatha kulepheretsa.

Zovuta zingaphatikizepo:

• Kuchepa kwa magazi (hemolytic anemia ndikosowa)
• Zovuta zamkati zamanjenje
• Matenda a chiwindi
• Imfa ya minyewa ya chiwindi
• Chiwindi chamafuta
• Chiwindi
• Zowonjezera mwayi wamafupa
• Kuchuluka kwa matenda
• Kuvulala komwe kumachitika chifukwa cha kugwa
• Jaundice
• Ma contract olumikizana kapena zolakwika zina
• Kutaya luso lodzisamalira
• Kutaya ntchito kuntchito ndi kunyumba
• Kutaya kuyanjana ndi anthu ena
• Kutayika kwa minofu (atrophy ya minofu)
• Zovuta zamaganizidwe
• Zotsatira zoyipa za penicillamine ndi mankhwala ena omwe amagwiritsidwa ntchito kuthana ndi vutoli
• Mavuto amphaka

Kulephera kwa chiwindi ndi kuwonongeka kwa mitsempha yapakati (ubongo, msana) ndizofala kwambiri komanso zowopsa pamatenda. Ngati matendawa sanagwidwe ndikuchiritsidwa msanga, amatha kupha.

Itanani omwe akukuthandizani ngati muli ndi zizindikiro za matenda a Wilson. Itanani aphungu amtundu wa chibadwa ngati muli ndi mbiri ya matenda a Wilson m'banja mwanu ndipo mukukonzekera kukhala ndi ana.

Upangiri wa chibadwa umalimbikitsidwa kwa anthu omwe ali ndi mbiri yakubadwa ya matenda a Wilson.

Matenda a Wilson; Kuchepa kwa hepatolenticular

• Central dongosolo lamanjenje ndi zotumphukira zamanjenje
• Kuyesa mkodzo wamkuwa
• Matenda a chiwindi

National Institute of Diabetes ndi tsamba la Digestive and Impso Diseases. Matenda a Wilson. www.niddk.nih.gov/health-information/liver-disease/wilson-disease. Idasinthidwa Novembala 2018. Idapezeka Novembala 3, 2020.

Roberts EA. Matenda a Wilson. Mu: Feldman M, Friedman LS, Brandt LJ, olemba. Sleisenger ndi Matenda a Mimba ndi a Fordtran Amatenda a Chiwindi. 11th ed. Philadelphia, PA: Elsevier; 2021: mutu 76.

Pezani nkhaniyi pa intaneti Schilsky ML. Matenda a Wilson. Mu: Goldman L, Schafer AI, olemba. Mankhwala a Goldman-Cecil. 26 wa. Philadelphia, PA: Elsevier; 2020: chaputala 200.