Maple syrup urine chirwere (MSUD) chirwere chinokonzera hutano chinokonzera kukanganisa kweuropi uye kufambira mberi kwehutano hwehutano hwehutano. Chiremera chemajini chinounza MSUD chinokonzerwa nechiremera mu enzyme inonzi branched-chain alpha-keto acid dehydrogenase, (BCKD), iyo inofanirwa kuparara kweamino acids leucine, isoleucine, uye valine. Pasina enzyme yeBKKD, aya amino acid anovaka kuhutu hwemuviri.
MSUD inowana zita rayo kubva pakuziva kuti, dzimwe nguva apo ropa reamino acid riripamusoro, urini inotora kunhuwirira kwakasiyana.
Maple syrup urine chirwere chinowanikwa munenge 1 kusvika 180 000 kuberekwa patsva uye inopikisa vose varume nevakadzi. MSUD inokanganisa vanhu vemadzinza ose, asi huwandu huri nani hwehutano hunoitika munzvimbo dzinowanikwa mikana yakawanda, yakadai senharaunda yeMennonite muPennsylvania (US).
Zviratidzo
Kune mhando dzakasiyana dzemaple syrup urine chirwere. Chinhu chinowanzoonekwa (classic) chimiro chinowanzobudisa zviratidzo mumacheche achangoberekwa ane makore 4 kusvika ku7. Zviratidzo izvi zvinogona kusanganisira:
- Kudya zvakashata
- Kudzora
- Kushaya uremu hunodiwa
- Kuwedzerwa kwehutera (zvakaoma kumuka)
- Unhu hwakapisa shuga kunhuwira mumurini
- Kuchinja mumatauriri temazwi, masaisai emasisipo, uye kugumburwa
Kana isina kusiya, vana ava vachafa nemwedzi yekutanga yeupenyu.
Vanhu vane MSUD, iyo yechipiri inowanzozivikanwa yehutachiona, inobudirira nguva dzose asi kana kurwara kuchiratidza zviratidzo zveMASU chaiye.
MSUD yechipfuva inonyanya kufanana fomu. Vanhu vane rudzi urwu vane 3-30% yehutano hwakakwana hweBKKD enzyme, saka zviratidzo zvingatanga chero panguva ipi zvayo.
Muchiedza che-thiamine-chinogadzirisa MSUD, vanhu vanoratidza humwe huri nani pavanopa thiamine kuwedzera.
Chimwe chimiro chisinganzwisisiki chechirwere chacho ndechokuti E3-yakashaya MSUD, iyo vanhu vane zvimwe zvisingakwanisi zvemamiriki emuviri.
Kuziva
Kana maple syrup urine chirwere ichifungidzirwa zvichibva pane zviratidzo zvepanyama, kunyanya huno hunhu hwekunakidza, kuongorora ropa kweamino acids kunogona kuitwa. Kana alloisoleucine ichionekwa, kuongororwa kunosimbiswa. Nguva dzose kuongororwa kwevana vatsva kwe MSUD kunoitwa mune dzimwe nyika mukati meU.S.
Kurapa
Mushonga mukuru we maple syrup urine chirwere ndiko kudziviswa kwezvokudya zvemafuta matatu amino acids leucine, isoleucine, uye valine. Izvi zvinogadziriswa zvekudya zvinofanira kuva zveupenyu hwose. Iko kune maitiro akawanda emabhizimusi uye zvokudya zvevanhu vane MSUD.
Chimwe chekuda kurapwa kweMSUD ndechokuti kana munhu akaremara ari kurwara, akakuvara, kana kuti achivhiyiwa, chirwere chacho chiri kuwedzera. Vanhu vakawanda vachada kurapwa munguva yekurapa kwekurapa kuti vadzivise matambudziko makuru.
Nenzira inofadza, nekuteerera kumiganhu yezvokudya uye kugara uchiongorora zvekurapa vanhu vane maple syrup urine zvirwere zvinogona kurarama kwenguva yakareba uye ine utano hupenyu.
Sources:
Bodamer, OA (2003). Maple syrup urine chirwere. eMedicine.
Morton DH, Strauss KA, Robinson DL, uye al. Kuongororwa uye kurapwa kwemaaple syrup chirwere: chidzidzo chevarwere 36. Pediatrics 2002; 109: 999.