Inunso nthawi zina mumamva kupweteka komanso kufooka mukamachita masewera olimbitsa thupi kapena kuchita chinthu chomwe chimafuna khama pang'ono, sichoncho? Zimenezo ndi zachilendo. Koma kwa anthu ena, vutoli ndi lopitirira muyeso. Ngakhale kuchita chinthu chaching'ono kumakupangitsani kutopa msanga, ndipo minofu yanu imapweteka komanso kulimba. Lero tikambirana za vuto losowa koma lofunika kwambiri lomwe muyenera kulidziwa. Imeneyi imatchedwa matenda a McArdle, kapena `matenda osungira glycogen type 5 (GSD5)`.
Kodi Matenda a McArdle ndi chiyani?
Mwachidule, matenda a McArdle ndi matenda a majini omwe amapezeka m'mabanja mwathu . Amakhudza kwambiri minofu ya mafupa anu. Ndiko kuti, minofu yomwe timagwiritsa ntchito poyenda, kunyamula zinthu, komanso kuyenda.
Matendawa amayamba chifukwa cha kusowa kapena kusakhalapo konse kwa enzyme yapadera mu minofu yathu yotchedwa `muscle glycogen phosphorylase` kapena `myophosphorylase`. Pamene enzyme iyi ilibe konse, minofu yathu singathe kupanga mphamvu zofunika. Ichi ndichifukwa chake zizindikiro monga kupweteka kwa minofu, kuuma, ndi kutopa zimawonekera panthawi yochita masewera olimbitsa thupi kapena tikatopa.
Zizindikiro nthawi zambiri zimayamba kuonekera munthu atatha zaka 15-20, kapena m'zaka za m'ma 20 kapena 30. Komabe, nthawi zina zimatha kuonekera munthu ali ndi zaka zilizonse. Matendawa amatchedwa "McArdle" potengera dzina la Dr. Brian McArdle, yemwe adalengeza koyamba mu 1951.
Kodi matendawa ndi ofala bwanji?
Matenda a McArdle ndi matenda osowa kwambiri . Malinga ndi ofufuza, amakhudza munthu mmodzi pa anthu 50,000 ndi mmodzi pa anthu 200,000 ku United States.
Kodi zizindikiro zake ndi ziti?
Zizindikiro za matendawa zimatha kusiyana kwambiri malinga ndi munthu. Anthu ena akhoza kukhala ndi zizindikiro zochepa kwambiri, pomwe ena angakhudzidwe kwambiri.
Chizindikiro chachikulu komanso chodziwika bwino ndi kusalolera kuchita masewera olimbitsa thupi, zomwe zikutanthauza kutopa msanga mukamachita masewera olimbitsa thupi . Zizindikiro zina ndi izi:
- Kupweteka kwa minofu
- Kufooka
- Kutopa
- Kupweteka kwa minofu
- Kuuma kwa minofu
Zizindikiro zonsezi zimatha kuwoneka mukangoyamba kuchita masewera olimbitsa thupi. Koma nthawi zambiri zimachepa mukangopuma pang'ono . Chodabwitsa n'chakuti, anthu ena amapeza kuti akayamba kutopa, ngati atapuma pang'ono kenako n'kuchitanso masewera olimbitsa thupi omwewo, amatha kuchita bwino kuposa kale. Izi zimatchedwa "mphepo yachiwiri." Zili ngati kukhala ndi mphepo yachiwiri. Mukapanda kuchita masewera olimbitsa thupi, nthawi zambiri simumva zizindikiro zilizonse.
Mungathe kuchita masewera olimbitsa thupi opepuka komanso ofatsa, monga kuyenda pansi panthaka, popanda vuto lililonse. Komabe, kuchita masewera olimbitsa thupi mwamphamvu kungayambitse zizindikiro mwachangu. Makamaka, masewera olimbitsa thupi omwe amaphatikizapo kugwira minofu pamalo ake osasuntha, monga masewera olimbitsa thupi a isometric , kugwada, ndi kuyimirira ndi zala zanu, amatha kuwononga minofu. Ganizirani za kusasangalala komwe mumamva mukanyamula silinda ya gasi kapena kunyamula thumba lolemera.
Kodi izi zingayambitse mavuto aakulu? (Zovuta)
Inde, nthawi zina mavuto aakulu amatha kuchitika. Pafupifupi theka la anthu omwe ali ndi matenda a McArdle amakhala ndi vuto lotchedwa `rhabdomyolysis` atatha kuchita masewera olimbitsa thupi kwambiri . Apa ndi pamene minofu imasweka.
Rhabdomyolysis ndi vuto lalikulu, lomwe lingayambitse kulephera kwa impso mwadzidzidzi (kulephera kwa impso mwachangu) komanso kuchuluka kwa potaziyamu m'magazi (hyperkalemia).
Choncho, muyenera kusamala kwambiri za kuchuluka kwa masewera olimbitsa thupi komanso momwe mumachitira masewera olimbitsa thupi. Izi zithandiza kupewa matenda a `rhabdomyolysis`. Ngati mukukumana ndi zizindikiro monga kutupa kwa minofu, mkodzo wakuda (bulauni, wofiira, wamtundu wa tiyi) , muyenera kufunafuna upangiri wa dokotala nthawi yomweyo .
N’chifukwa chiyani izi zikuchitika? Kodi zifukwa zake ndi ziti?
Matenda a McArdle ndi matenda a majini . Makamaka, amayamba chifukwa cha kusintha kwa majini a `PYGM`. Kawirikawiri, majini a `PYGM` awa amalangiza thupi lathu kupanga enzyme yotchedwa `myophosphorylase`.
Enzyme iyi imapezeka m'maselo a minofu yathu ya mafupa okha. Imaphwanya glycogen (shuga wosungidwa) m'minofu kukhala glucose-1-phosphate. Kenako glucose-1-phosphate iyi imasinthidwa kukhala shuga. Shuga ndiye gwero lalikulu la mphamvu m'thupi lathu . Tikamachita masewera olimbitsa thupi, minofu yathu imagwiritsa ntchito shuga wambiri.
Tsopano, pamene enzyme ya `myophosphorylase` yapangidwa pang'ono kapena ayi konse chifukwa cha kusintha kwa majini a `PYGM`, maselo athu a minofu sangathe kuswa bwino `glycogen` ndikupanga mphamvu (`glucose`). Ndicho chifukwa chake minofu imatopa msanga.
Ofufuza mpaka pano apeza mitundu yosiyanasiyana 179 (zosiyanasiyana) zomwe zimakhudza jini ya `PYGM`.
Momwe matenda a McArdle amatengera
Mumalandira matendawa kuchokera kwa makolo anu enieni, mu njira ya "autosomal recessive" . Mwachidule, makolo onse awiri ayenera kukhala "onyamula" jini yosinthidwayo, zomwe zikutanthauza kuti onse ali ndi jini pafupi.Anthu omwe ali ndi kachilomboka nthawi zambiri sasonyeza zizindikiro. Komabe, ngati makolo onse awiri ali ndi kachilomboka ndipo onse awiri amapatsira ana awo jini yosinthidwayo, matendawa amayamba.
Kodi madokotala amazindikira bwanji izi? (Kuzindikira)
Madokotala amagwiritsa ntchito kwambiri mayeso a mkono kuti adziwe matendawa. Izi zimaphatikizapo kutenga magazi musanayambe komanso mukamaliza kuchita masewera olimbitsa thupi pang'ono ndi mkono wanu. Makamaka, amaona kuchuluka kwa `lactic acid` ndi `ammonia` .
Kawirikawiri, kuchuluka kwa lactic acid m'thupi kuyenera kukwera katatu mutatha kuchita masewera olimbitsa thupi. Komabe, ngati kuchuluka kwa lactic acid m'thupi lanu sikukwera mu mayeso awa, ndi chizindikiro chakuti mwina muli ndi matenda a McArdle.
Pali mayeso ena omwe angathandize kutsimikizira matendawa:
- Kuyezetsa magazi kwa Creatine kinase (CK): Minofu ikawonongeka, enzyme yotchedwa creatine kinase (CK) imasonkhana m'magazi. Anthu omwe ali ndi matenda a McArdle akhoza kukhala ndi CK yambiri nthawi zonse.
- Mayeso oyeserera olimbitsa thupi: Izi zitha kuyesa vuto lotchedwa "mphepo yachiwiri." Dokotala adzakupangitsani kuchita masewera olimbitsa thupi pang'ono, kupumula pakati, ndikuwona ngati mungathe kuchita masewera olimbitsa thupi bwino mukatha zina zonse.
- Kufufuza minofu: Mu mayeso awa, dokotala amatenga chitsanzo chaching'ono cha minofu yanu ya mafupa ndikutumiza ku labu kuti akayang'aniridwe pogwiritsa ntchito maikulosikopu. Katswiri wa matenda a minofu amayang'ana minofuyo kuti awone ngati pali zizindikiro zilizonse za matenda a McArdle.
- Kuyesa majini: Izi zitha kuzindikira kusintha kwa majini komwe kumayambitsa matenda a McArdle.
Kodi tingachite chiyani pa izi? (Chithandizo)
Mwatsoka, palibe mankhwala a matenda a McArdle. Chithandizo chachikulu ndikupewa zinthu zomwe zimawonjezera zizindikiro zanu. Komabe, kusachita masewera olimbitsa thupi konse sikwabwino pa thanzi lanu.
Kafukufuku wasonyeza kuti kuchita masewera olimbitsa thupi olimbitsa thupi pang'ono komanso mopanda malire kungakhale kothandiza kwa anthu omwe ali ndi matenda a McArdle. Anthu omwe amachita masewera olimbitsa thupi amtunduwu amachepetsa kwambiri kusagwirizana ndi masewera olimbitsa thupi, ndipo amathanso kumva "kupuma kwachiwiri" mwachangu panthawi yochita masewera olimbitsa thupi. Mutha kugwira ntchito ndi dokotala wanu komanso katswiri wa masewera olimbitsa thupi kuti mupeze njira yabwino kwambiri yochitira masewera olimbitsa thupi.
Komanso, chakudya chokhala ndi chakudya chambiriKafukufuku akusonyeza kuti kumwa mankhwalawa kungachepetsenso kuopsa kwa zizindikiro zomwe zimachitika panthawi yochita masewera olimbitsa thupi. Izi zimathandiza minofu yanu kugwiritsa ntchito shuga m'magazi anu m'malo mwa glycogen kuti ikhale ndi mphamvu. Dokotala wanu kapena katswiri wazakudya wovomerezeka angakulangizeni dongosolo la chakudya monga ili:
- 65% ya zopatsa mphamvu zanu za tsiku ndi tsiku ziyenera kuchokera ku chakudya chopatsa mphamvu (kuchokera ku zinthu monga ndiwo zamasamba, zipatso, buledi, pasitala, ndi mpunga).
- 20% ya ma calories ochokera ku mafuta .
- 15% ya ma calories ochokera ku mapuloteni .
Zingakhalenso zothandiza kumwa shuga wamba, monga chakumwa chamasewera chotsekemera, nthawi yochepa musanayambe kuchita masewera olimbitsa thupi.
Kodi kukhala ndi moyo ndi izi kuli bwanji?
Anthu ambiri omwe ali ndi matenda a McArdle amakhala ndi moyo wabwinobwino . Anthu ena amatha kukhala ndi kufooka kosalekeza komanso kufooka kwa minofu ('atrophy') akamakula, nthawi zambiri ali ndi zaka za m'ma 60 kapena 70.
Chofunika kwambiri ndikudziwa zizindikiro za vuto la `rhabdomyolysis` lomwe latchulidwa pamwambapa ndikuletsa kuti lisachitike momwe zingathere, chifukwa ndilo vuto lalikulu la matendawa.
Kodi munthu amene ali ndi matendawa amakhala ndi moyo wautali bwanji?
Matenda a McArdle nthawi zambiri sakhudza moyo wa munthu .
Komabe, nthawi zambiri, pamakhala vuto lotchedwa `infantile McArdle syndrome` , lomwe limawonekera nthawi yomweyo mwana akangobadwa. Izi zimapha - zizindikiro zake zimakhala zazikulu ndipo zimakula mofulumira.
Kodi matenda a McArdle angapewedwe?
Popeza ichi ndi vuto la majini, palibe chomwe tingachite kuti tipewe .
Ngati mukuda nkhawa ndi chiopsezo chanu chotengera matenda a McArdle kapena matenda ena a majini musanakhale ndi mwana, ndi bwino kulankhula ndi dokotala wanu za uphungu wa majini .
Kodi ndiyenera kupita kwa dokotala liti?
Ngati zizindikiro zanu zikuipiraipira, kapena ngati luso lanu lochita zinthu zolimbitsa thupi lasintha, onani dokotala wanu.
Mungafunikenso kuonana ndi katswiri wa zamaganizo ndi/kapena katswiri wa zakudya nthawi zonse kuti akuthandizeni pa dongosolo lanu la chithandizo.
Kodi muyenera kupita ku Chipinda Chothandizira Anthu Odwala Mwadzidzidzi (ETU) liti?
Ngati muli ndi zizindikiro za `rhabdomyolysis`, muyenera kupita ku chipinda chadzidzidzi (ETU) mwachangu momwe mungathere . Zizindikiro zake ndi izi:
- Kutupa kwa minofu
- Kufooka kwa minofu
- Kupweteka ndi kupweteka mukakhudza minofu
- Mkodzo wakuda (bulauni, wofiira, wamtundu wa tiyi)
- Kusowa madzi m'thupi
- Kuchepa kwa kukodza
- Kusanza
- Kutaya chidziwitso
Matenda a McArdle angapangitse kuti zikhale zovuta kuchita masewera olimbitsa thupi. Koma nkhani yabwino ndi yakuti,Ndi yotheka kuisamalira ndipo siikhudza kwambiri thanzi lanu lonse. Dokotala wanu adzagwira nanu ntchito kuti akupezereni njira yabwino kwambiri yothandizira.
Uthenga Wopita Nawo Kunyumba
Chabwino, tiyeni tikukumbutseni zinthu zingapo zomwe takambirana zomwe tikuganiza kuti ndizofunikira kwa inu:
Matenda a McArdle ndi matenda a majini omwe amayamba chifukwa cha kusowa kwa enzyme yomwe imathandiza minofu kupanga mphamvu.
Zizindikiro zazikulu ndi kutopa msanga, kupweteka kwa minofu, ndi kugubuduzika pamene mukuchita masewera olimbitsa thupi.
Dziwani vuto lalikulu lotchedwa `rhabdomyolysis`. Ngati mukumva zizindikiro izi, pitani kwa dokotala nthawi yomweyo.
Ngakhale kuti palibe mankhwala enieni a izi, zitha kuthandizidwa bwino ndi masewera olimbitsa thupi oyenera komanso zakudya zoyenera ndipo mutha kukhala ndi moyo wabwinobwino.
Ngati muli ndi mafunso ena okhudza izi, musazengereze kulankhula ndi dokotala wanu.
Kumbukirani, simuli nokha. Ndi chidziwitso choyenera ndi chithandizo, mutha kuthana ndi vutoli.
Matenda a McArdle , GSD5, Matenda a McArdle, Kupweteka kwa Minofu, Kuchita Masewera Olimbitsa Thupi, Matenda a Majini, Glycogen, Myophosphorylase, Rhabdomyolysis, Kusalolera Kuchita Masewera Olimbitsa Thupi











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