Mathata a Metabolism a Nahaneng
Leigh's lefu ke lefu le bakoang ke ts'ebeliso ea methapo e senyang tsamaiso ea methapo (boko, mokokotlo le methapo ea optic). Leigh's lefu le bakoa ke mathata a mitochondria, litsi tsa matla meleng ea 'mele.
Bothata ba lefutso bo bakang lefu la Leigh le ka rua ka litsela tse tharo tse fapaneng. E ka ba lefa la chromosome ea X (e tšehali) e le ho haella ha liphatsa tsa lefutso tsa enzyme e bitsoang pyruvate dehydrogenase complex (PDH-Elx).
E ka 'na ea futsatsoa e le boemo bo feteletseng ba boits'oaro bo amang' mele oa enzyme e bitsoang cytochrome-c-oxidase (COX). E ka 'na ea boela ea futsetsoa e le phetoho ea DNA ka cell cell mitochondria.
Matšoao
Matšoao a lefu la Leigh a atisa ho qala pakeng tsa likhoeli tse tharo le lilemo tse peli. Kaha lefu lena le ama tsamaiso ea methapo ea matšoao, matšoao a ka 'na a kenyelletsa:
- ho fokola habonolo
- ho thata ho tšoara hlooho
- ho lahleheloa ke lisebelisoa tsa motlakase lesea le ne le e-na le ho tšoara le ho le sisinya
- ho lahleheloa ke takatso ea lijo
- ho hlatsa
- ho halefa
- ho lla ho tsoelang pele
- ho tsietsing
Ha lefu la Leigh le ntse le mpefala ka mor'a nako, matšoao a ka 'na a kenyelletsa:
- bofokoli bo akaretsang
- ho hloka molumo oa mesifa (hypotonia)
- lihlopha tsa lactic acidosis (ho bokella ha lactic acid 'meleng le boko) tse ka senyang mosebetsi oa ho hema le oa liphio
- mathata a pelo
Tsejoa
Ho lemoha lefu la Leigh ho itšetlehile ka matšoao ao lesea kapa ngoana a nang le 'ona.
Liteko li ka 'na tsa bontša ho haella ha pyruvate dehydrogenase kapa boteng ba lactic acidosis. Batho ba nang le lefu lena la Leigh ba ka 'na ba e-ba le litlhaloso tse sa tšoaneng tsa tšenyo boko bo ka fumanoang ke ho hlahloba boko. Ho batho ba bang, ho hlahlojoa ha liphatsa tsa lefutso ho ka khoneha ho khetholla boteng ba phetoho ea lefutso.
Phekolo
Kalafo ea lefu la Leigh e atisa ho akarelletsa mavithamine a kang thiamine (vithamine B1). Mefuta e meng ea phekolo e ka 'na ea tsepamisa mohopolo matšoao a teng, a kang lithethefatsi tse khahlanong le ts'oaetso kapa meriana ea pelo kapa ea liphio. Ho phekola, ho sebetsa, le ho bua ho ka thusa ngoana hore a fihlele bokhoni ba hae ba ntshetsopele.
Lisebelisoa:
> "NINDS Leigh's Disease Information Information." Mathata. 13 Feb 2007. Mokhatlo oa Naha oa Mathata a Neurological and Stroke.
> "Lefu la Leigh." Index ea mafu a mangata. 17 Dec 2007. Mokhatlo oa Sechaba oa Mathata a Tloaelehileng.
> Monyenyane, Trisha. "Lefu la Leigh." BBC Health. July 2006.