Fungidzira kuti paunochinja manapukeni emwana wako mudiki, kana kuti paunobata mwana wako, unoona kunhuhwirira kusinganzwisisike, kunotapira. Semapuropa emumaple , kana kunhuhwirira kweshuga . Kunyangwe ukasateerera pakutanga, kana kunhuhwirira uku kukaramba kuchiuya, zvakajairika kuti chero amai kana baba vanzwe kutya zvishoma. Kutaura zvazviri, ichi ndicho chiratidzo chikuru uye chakajeka chechirwere chisingawanzoitiki asi chakakosha kuziva chirwere chekutanga chinonzi 'Maple Syrup Urine Disease' (MSUD).
Zvichitaurwa zviri nyore, ichi chirwere chemetabolism. Kureva kuti, pane chikanganiso mukuita kwakaoma kunoshandura chikafu chatinodya kuita simba. Tose tinoziva kukosha kweprotein kumiviri yedu. Mapuroteni aya anoumbwa nezvidimbu zvidiki zvinonzi amino acids. Mumunhu ane MSUD, muviri haugone kuputsa zvakanaka marudzi matatu eamino acids, anoti leucine, isoleucine, uye valine , woashandura kuita simba. Izvi zvinodaro nekuti maenzymes anodiwa pakuita izvi haagadzirwe mumiviri yavo. Saka chii chinoitika ipapo? Aya maamino acids asingaputsike uye zvigadzirwa zvawo zvinotanga kuungana mumuviri nemuropa sechepfu . Chepfu idzi dzinogona kukuvadza uropi nedzimwe nhengo dzemuviri. Kana dzikasarapwa, chirwere ichi chinogona kutokonzera rufu. Saka, zvakakosha kuziva izvi.
Sei chirwere chisingawanzoitiki chichiitika?
Chirwere chemushonga wemushonga weMaple syrup chirwere chisingawanzoitiki chemajini. Pasi rose, mwana mumwe chete pavana 185,000 anoberekwa aine chirwere ichi.
Izvi zvinokonzerwa nekuchinja kwemajini edu. Kuchinja kwemajini uku kunodzivirira muviri kugadzira ma enzyme anodiwa kuti uparadze ma amino acid matatu atakataura nezvawo kare. Ichi chirwere chemajini chinokonzeresa kuberekwa . Izvi zvinoreva kuti kuti mwana awane chirwere ichi, amai nababa vanofanira kugara nhaka jini rakakanganisika.
Fungidzira kuti iwe nemumwe wako mese muri 'vatakuri' vejini iri risina kunaka. Izvi zvinoreva kuti hamuna zviratidzo, asi mune jini risina kunaka mumuviri wenyu. Kana zvakadaro:
- Pane mukana we25% wekuti mwana wako achazvarwa aine MSUD.
- Pane mukana we50% wekuti mwana wako achava 'mutakuri', sezvauri iwe, uyo achange asina zviratidzo asi aine jini racho.
- Pane mukana we25% wekuti mwana haazogari nhaka yejini iri risina kunaka zvachose uye achave neutano hwakanaka.
Kune mhando dzakasiyana dzechirwere ichi here?
Ehe, kune mhando huru ina dzeMSUD, dzinosiyana pakuoma kwadzo uye nguva inotorwa kuti zviratidzo zvionekwe.
| Rudzi rweMSUD | Zviratidzo uye hunhu hwayo |
|---|---|
| MSUD yekare | Urwu ndirwo rudzi rwakajairika uye rwakanyanya kuipa . Vana vane chirwere ichi havagadziri ma enzyme anodiwa zvachose, kana kuti havabudisi ma enzymes akawanda. Zviratidzo zvinowanzoonekwa mukati memazuva matatu ekutanga ekuzvarwa. |
| MSUD yepakati | Vanhu ava vanogadzira ma enzyme akawanda kupfuura emhando yekare. Nokudaro, zviratidzo hazvina kunyanya kuipa. Zviratidzo zvinowanzoonekwa pakati pemwedzi mishanu nemakore manomwe ekuberekwa. |
| MSUD inongogara iripo | Vana ava vanokura zvakanaka. Zviratidzo zvinongoonekwa chete kana muviri uri pasi pekushushikana, senge fivha kana kushushikana . Dzimwe nguva, vanoita sevane hutano hwakanaka. |
| MSUD inopindura thiamine | Thiamine ivhitamini B1. Vhitamini iyi inowedzera mashandiro ema enzymes. Zviratidzo zvinogona kudzorwa kana varwere vane rudzi urwu rwechirwere vakapihwa thiamine yakawanda uye kudya kwakakosha. |
Ndezvipi zviratidzo kunze kwekunhuhwirira kwemushonga wemaple?
Sezvatakambotaura, chiratidzo chinonyanya kuoneka kunhuhwirira kunoita semapuroteni emumaple kana shuga yebrown. Kunhuhwirira uku kunogona kubva muweti , ziya , uye kunyange wakisi yenzeve .
Asi kune zvimwe zviratidzo zvakawanda kunze kwekunhuhwirira uku. Izvi zvinosiyana zvichienderana nerudzi rwechirwere uye munhu wacho.
Zviratidzo zvevana vachangoberekwa (Classic MSUD)
Kana vana ava vakasarapwa, vanogona kuratidza zviratidzo zvakaita se:
- Kusagadzikana nguva dzose, kushatirwa
- Kuoma kana kuramba kunwa mukaka
- Kurara zvisina kujairika kana kunzwa kuora mwoyo nguva dzese
- Matambudziko ekufema
- Kurwadziwa kwetsandanyama kana kukotama kwemuviri
- Kushaya simba
Yambiro: Izvi zvirwere zvakakomba uye zvinokurumidzira. Kana mwana wako aine chimwe chezviratidzo izvi, usanonoka kana miniti imwe chete wobva waona chiremba nekukasika kana kumuendesa kuEmergency Department (ETU) yechipatara chiri pedyo.
Zviratidzo muvana vakuru nevakuru
Mumhando dzepakati, dzisingawanzoitiki, uye dzinopindura thiamine, zviratidzo zvinogona kuonekwa pazera ripi zvaro. Zvinogona kuonekwa kana kuipa panguva yefivha, kurwara, kana kushushikana.
- Kurwadziwa kwemudumbu nekurutsa
- Kurasikirwa nechido chekudya uye kudzikira kwehuremu
- Kushaya simba kwetsandanyama kana kurasikirwa nekuzvidzora pakufamba
- Kufamba kusingatarisirwi
- Kutaura kusina kujeka
- Kurasikirwa nepfungwa kana kuoma kuramba wakamuka
Nzira yekuziva sei chirwere ichi?
Munyika dzakawanda dzakabudirira, kuongororwa kwevana vacheche kunosanganisira kuongororwa kweMSUD. Saka, chimiro chekare chinogona kuonekwa mukati memazuva mashoma ekuzvarwa. MuSri Lanka, vacheche vanoongororwawo zvirwere zvakasiyana-siyana. Sezvo chirwere ichi chisingawanzoitiki, havasi vacheche vese vanogona kuongororwa chirwere ichi.
Zvisinei, chiremba anogona kufungidzira chirwere ichi kana akaona zviratidzo zvemwana, kunyanya kunhuhwirira kwakasiyana. Zvadaro, bvunzo dzeropa neweti dzinoitwa kuti dzisimbise chirwere chacho. Ongororo idzi dzinogona kutarisa huwandu hwakakwira hwemaamino acids ari muropa neweti. Mune zvimwe zviitiko, bvunzo dzemajini dzinoshandiswawo kusimbisa chirwere chacho.
Mishonga yacho ndeipi? Zvinokwanisika here kuirapa zvachose?
Chinangwa chikuru chekurapa chirwere ichi ndechekudzora huwandu hwemaamino acid anokuvadza anoungana mumuviri.
Kurapa kukuru kudya kwakakosha kunofanira kutevedzerwa muhupenyu hwese.Izvi zvinosanganisira kuderedza kudya kwemapuroteni (semuenzaniso, nyama, hove, mazai, zvigadzirwa zvemukaka, nezvimwe zviyo) zvine amino acids dzakawanda (leucine, isoleucine, uye valine). Panguva imwe chete, kune zvimwe zvinovaka muviri zvinodiwa pakukura, zvakaita sehupfu hwemukaka hwakagadzirwa nema supplements. Chirongwa ichi chekudya chinoshandiswa pasi pekutarisirwa kwachiremba uye nyanzvi yezvekudya .
Kana zviratidzo zvanyanya kuipa, kuchipatara kunodiwa uye mamwe marapirwo anoshandiswa.
- Ipai muviri chikafu chinodiwa nekupa chikafu kuburikidza netsinga (IVs) kana machubhu akakosha.
- Dzora huwandu hweamino acid muropa nekupa glucose kana insulin kuburikidza neIV.
- Kuti ubvise chepfu dzakaungana muropa nekukurumidza, isa ropa kuti riseferwe (nzira yakaita sedialysis).
Mushonga wekugara kwenguva refu wechirwere ichi kuisirwa chiropa. Kana chiropa chinobva kumunhu ane hutano chikaisirwa, chiropa chinogadzira ma enzyme anodiwa, zvichiita kuti murwere ararame zvakanaka, pasina zvirambidzo pakudya.
Mharidzo Yekuenda Nayo Kumba
- MSUD chirwere chisingawanzoitiki, asi chakakomba chemajini.
- Kana weti yemwana wako, ziya, kana wakisi zvichinhuwirira zvinotapira, semanyuchi emumaple, izvi zvinogona kuva chiratidzo chikuru.
- Kuongororwa nekukurumidza uye kurapwa kwakakosha kudzivirira kukuvara kusingaperi kuuropi nedzimwe nhengo dzemuviri.
- Kurapa kukuru kudya kwakakosha kunofanira kutevedzerwa muhupenyu hwese.
- Kana ukaona chimwe chezviratidzo zvataurwa munyaya ino, kunyanya kumwana achangoberekwa , ndapota shanyira chiremba wako nekukasika. Kana chirwere ichi chikaongororwa nekukurumidza, mhedzisiro yacho inova nani.
Chirwere cheMaple Syrup Urine, MSUD, Zvirwere zveMajini, Zvirwere zveVana, Zvirwere zveMetabolic, Amino Acids, Kunhuhwirira kweWeti yeVacheche
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