Une kurwadziwa kwetsandanyama here kunyangwe mushure mekurovedza muviri zvishoma? Ichi chingave chirwere cheMcArdle!

Iwewo dzimwe nguva unonzwa kurwadziwa uye kushaya simba kana uchiita maekisesaizi kana kuita chimwe chinhu chinoda kushanda nesimba, handiti? Izvozvo zvakajairika. Asi kune vamwe vanhu, chirwere ichi chakanyanya. Kunyangwe kuita chimwe chinhu chidiki kunoita kuti unzwe kuneta nekukurumidza, uye tsandanyama dzako dzinorwadza uye dzakasimba. Nhasi tichataura nezvechirwere chisingawanzoitiki asi chakakosha kwazvo chekuziva. Ichi chinonzi chirwere cheMcArdle, kana kuti `glycogen storage disease type 5 (GSD5)`.

Chii chinonzi McArdle Disease?

Zvichitaurwa zviri nyore, chirwere chaMcArdle chirwere chemajini chinowanikwa mumhuri dzedu . Chinonyanya kukanganisa tsandanyama dzemapfupa edu. Kureva, tsandanyama dzatinoshandisa kufamba-famba, kusimudza zvinhu, uye kufamba.

Chirwere ichi chinokonzerwa nekushaikwa kana kusavapo zvachose kwe enzyme yakakosha mutsandanyama dzedu inonzi `muscle glycogen phosphorylase` kana `myophosphorylase`. Kana enzyme iyi isipo zvachose, tsandanyama dzedu hadzigone kugadzira simba rinodiwa. Ndosaka zviratidzo zvakaita sekurwadziwa kwetsandanyama, kuomarara, uye kuneta zvichionekwa panguva yekurovedza muviri kana patinenge taneta.

Zviratidzo zvinowanzotanga kuoneka mushure memakore 15-20, kana mumakore ekuma20 kana 30. Zvisinei, dzimwe nguva zvinogona kuonekwa pazera ripi zvaro. Chirwere ichi chinonzi "McArdle" zvichibva pana Dr. Brian McArdle, uyo akatanga kuchizivisa muna 1951.

Chirwere ichi chakajairika zvakadii?

Chirwere cheMcArdle chirwere chisingawanzoitiki . Sekureva kwevaongorori, chinobata munhu mumwe chete pavanhu 50,000 uye mumwe chete pavanhu 200,000 muUnited States.

Zviratidzo zvacho ndezvipi?

Zviratidzo zvechirwere ichi zvinogona kusiyana zvikuru kubva pamunhu kusvika pamunhu. Vamwe vanhu vanogona kuona zviratidzo zvisina kunyanya kuoma, nepo vamwe vanogona kubatwa zvakanyanya.

Chiratidzo chikuru uye chinowanzoonekwa ndechekusashivirira maekisesaizi, zvinoreva kunzwa kuneta nekukurumidza paunenge uchiita maekisesaizi . Zvimwe zviratidzo ndeizvi:

• Kurwadziwa kwetsandanyama
• Kushaya simba
• Kuneta
• Kurwadziwa kwetsandanyama
• Kuomarara kwetsandanyama

Zviratidzo izvi zvese zvinogona kuoneka paunotanga kurovedza muviri. Asi zvinowanzo pera mushure mekuzorora kwenguva pfupi . Zvinoshamisa kuti vamwe vanhu vanoona kuti mushure mekunzwa kuneta pakutanga, kana vakazorora kwenguva pfupi vozobva vaita kurovedza muviri zvakare, vanogona kuzviita zviri nani kupfuura kare. Izvi zvinonzi "mhepo yechipiri." Zvakafanana nekuva nemhepo yechipiri. Kana usingaite kurovedza muviri, kazhinji haunzwi zviratidzo.

Unogona kukwanisa kuita maekisesaizi akareruka, akapfava, sekufamba panzvimbo yakati sandara, pasina matambudziko. Zvisinei, kurovedza muviri kwakaoma kunogona kukonzera zviratidzo nekukurumidza. Kunyanya, maekisesaizi anosanganisira kubata tsandanyama panzvimbo pasina kufamba, akadai sekurovedza muviri kwe isometric , kukotama, uye kumira nezvigunwe zvako, zvinogona kukuvadza tsandanyama. Funga nezvekusagadzikana kwaunonzwa paunenge uchisimudza silinda yegesi kana kutakura bhegi rinorema.

Izvi zvinogona kukonzera matambudziko akakomba here? (Matambudziko)

Ehe, dzimwe nguva matambudziko akakomba anogona kuitika. Vanhu vanosvika hafu yevanhu vane chirwere cheMcArdle vanowana chirwere chinonzi `rhabdomyolysis` mushure mekurovedza muviri zvakanyanya . Apa ndipo apo tsandanyama dzinoputsika.

Rhabdomyolysis chirwere chakakomba, chinoisa upenyu panjodzi chinogona kukonzera kutadza kushanda kweitsvo kamwe kamwe (kutadza kushanda kweitsvo zvakanyanya) uye huwandu hwakawanda hwepotassium muropa (hyperkalemia).

Saka, unofanira kungwarira zvikuru kuti unoita maekisesaizi akawanda sei uye kuti unoomesa sei muviri. Izvi zvichabatsira kudzivirira chirwere che `rhabdomyolysis`. Kana ukaona zviratidzo zvakaita sekuzvimba kwetsandanyama, weti yakasviba (yebrown, tsvuku, ine ruvara rwetii) , unofanira kutsvaga rubatsiro rwechiremba nekukasika .

Sei izvi zvichiitika? Ndezvipi zvikonzero zveizvi?

Chirwere cheMcArdle chirwere chemajini . Zvikuru sei, chinokonzerwa nekuchinja kwemajini e'PYGM'. Kazhinji, jini iri re'PYGM' rinorayira muviri wedu kugadzira enzyme inonzi 'myophosphorylase'.

Iyi enzyme inowanikwa chete mumasero edu emhasuru. Inoputsa glycogen (shuga yakachengetwa) mumhasuru kuita glucose-1-phosphate. Iyi glucose-1-phosphate inozoshandurwa kuita glucose. Glucose ndiyo sosi huru yesimba remuviri wedu . Patinoita maekisesaizi, tsandanyama dzedu dzinoshandisa yakawanda yeglucose iyi.

Zvino, kana enzyme ye`myophosphorylase` ikasagadzirwa zvishoma kana kusagadzirwa zvachose nekuda kwekuchinja kwejini re`PYGM`, masero edu emhasuru haakwanise kuputsa `glycogen` nemazvo uye kugadzira simba (`glucose`). Ndosaka tsandanyama dzichikurumidza kuneta.

Vaongorori kusvika pari zvino vawana mhando dzakasiyana-siyana dzinosvika zana nemakumi manomwe nepfumbamwe (variants) dzinokanganisa jini re'PYGM'.

Nzira iyo chirwere cheMcArdle chinogarwa nhaka nayo

Unogara nhaka chirwere ichi kubva kuvabereki vako chaivo, nenzira ye "autosomal recessive" . Zvichitaurwa zviri nyore, vabereki vese vanofanira kunge vari "vatakuri" vejini rakachinjwa, zvichireva kuti vese vane jini iri pedyo.Vanhu vane chirwere ichi havawanzo ratidza zviratidzo. Zvisinei, kana vabereki vese vari ivo vanochitakura uye vese vachipa majini akachinjwa kuvana vavo, chirwere ichi chinozotanga.

Vanachiremba vanoongorora sei izvi? (Kuongororwa)

Vanachiremba vanoshandisa bvunzo yekurovedza muviri yeruoko kuti vaone chirwere ichi. Izvi zvinosanganisira kutora ropa usati waita maekisesaizi mashoma neruoko rwako. Kunyanya, vanotarisa huwandu hwe `lactic acid` ne `ammonia` .

Kazhinji, huwandu hwe lactic acid hunofanira kuwedzera kanenge katatu mushure mekurovedza muviri. Zvisinei, kana huwandu hwe lactic acid yako husina kukwira muongororo iyi, chiratidzo chekuti ungangove uine chirwere cheMcArdle.

Kune mimwe miedzo inogona kubatsira kusimbisa chirwere ichi:

• Kuongororwa kweropa reCreatine kinase (CK): Kana tsandanyama dzakuvara, enzyme inonzi creatine kinase (CK) inoungana muropa. Vanhu vane chirwere cheMcArdle vanogona kunge vaine huwandu hwakawanda hweCK nguva dzose.
• Bvunzo yekumanikidzwa kwekurovedza muviri yakarongwa: Izvi zvinogona kuyedza chiitiko chinozivikanwa se "mhepo yechipiri." Chiremba achaita kuti uite maekisesaizi mashoma, wozorora pakati, woona kana uchikwanisa kuita maekisesaizi zvakanaka mushure memamwe ese.
• Kuongororwa kwetsandanyama: Mubvunzo uyu, chiremba anotora sampuli diki yetsandanyama yako yemapfupa oitumira kurabhoritari kuti iongororwe nemaikorosikopu. Nyanzvi yezvirwere inotarisa nyama kuti ione kana paine zviratidzo zvechirwere cheMcArdle.
• Kuongororwa kwemajini: Izvi zvinogona kukwanisa kuona shanduko chaiyo yemajini inokonzera chirwere cheMcArdle.

Chii chatingaita nezveizvi? (Kurapwa)

Zvinosuwisa kuti hapana mushonga wechirwere cheMcArdle. Mushonga mukuru ndewekudzivisa zvinhu zvinowedzera zviratidzo zvako. Zvisinei, kusaita chero chiito chemuviri zvachose hakuna kunaka pahutano hwako futi.

Tsvagiridzo yakaratidza kuti kurovedza muviri kwe aerobic kwakasimba uye kwakaringana kunogona kubatsira vanhu vane chirwere cheMcArdle. Vanhu vanoita rudzi urwu rwekurovedza muviri vakaderedza zvakanyanya kusashivirira kurovedza muviri, uye vanogonawo kuwana "mhepo yechipiri" nekukurumidza panguva yekurovedza muviri. Unogona kushanda nachiremba wako nenyanzvi yekurovedza muviri kuti uwane chirongwa chakanakisa chekurovedza muviri chakakodzera iwe.

Uyewo, kudya kwakapfuma mumakabhohaidhiretsiOngororo dzinoratidza kuti kunwa kunogonawo kuderedza kuoma kwezviratidzo zvinoitika panguva yekurovedza muviri. Izvi zvinobatsira tsandanyama dzako kushandisa glucose (shuga) muropa rako pachinzvimbo cheglycogen kuti uwane simba. Chiremba wako kana nyanzvi yezvekudya inogona kukurudzira chirongwa chekudya chakadai:

• 65% yemacalorie ako ezuva nezuva anofanira kubva kumacarbohydrates akaomarara (kubva muzvinhu zvakaita semiriwo, michero, chingwa, pasta, nemupunga).
• 20% yemacalorie anobva mumafuta .
• 15% yemacalorie anobva kumapuroteni .

Zvinogonawo kubatsira kunwa shuga yakapfava, senge chinwiwa chemitambo chine shuga, nguva pfupi usati waita maekisesaizi.

Zviri sei kurarama neizvi?

Vanhu vazhinji vane chirwere cheMcArdle vanorarama hupenyu hwakajairika . Vamwe vanhu vanogona kushaya simba nguva dzose uye kurasikirwa netsandanyama ('atrophy') gare gare muhupenyu hwavo, kazhinji pavanenge vava nemakore ekuma60 kana 70.

Chinhu chinonyanya kukosha kuziva zviratidzo zvechirwere che "rhabdomyolysis" chataurwa pamusoro apa uye kudzivirira kuti chisaitike zvakanyanya sezvinobvira, sezvo chiri icho chinokonzera chirwere ichi.

Munhu ane chirwere ichi anotarisirwa kurarama hupenyu hwakadii?

Chirwere cheMcArdle hachiwanzo kukanganisa hupenyu hwemunhu .

Zvisinei, kashoma, pane chirwere chinonzi `infantile McArdle syndrome` , chinoonekwa nguva pfupi mushure mekuzvarwa. Izvi zvinouraya - zviratidzo zvacho zvakanyanya uye zvinokurumidza kuipa.

Chirwere chaMcArdle chinogona kudzivirirwa here?

Sezvo ichi chiri chirwere chemajini, hapana chatingaita kuti chisaitike .

Kana uchinetseka nezvenjodzi yako yekuwana chirwere cheMcArdle kana zvimwe zvirwere zvemajini usati wava nemwana, zvakanaka kutaura nachiremba wako nezvemazano ezvemajini .

Ndinofanira kuona chiremba rini?

Kana zviratidzo zvako zvikawedzera, kana kuti kana kugona kwako kuita mabasa ako emuviri kuchichinja, ona chiremba wako.

Ungangodawo kuona chiremba wemuviri kana nyanzvi yezvekudya nguva nenguva kuti akupe mazano ekurapwa kwako.

Unofanira kuenda kuEmergency Treatment Unit (ETU) riini?

Kana uine zviratidzo zve `rhabdomyolysis`, unofanira kuenda ku emergency room (ETU) nekukurumidza . Zviratidzo zvacho ndeizvi:

• Kuzvimba kwetsandanyama
• Kushaya simba kwetsandanyama
• Kurwadziwa kana uchibata tsandanyama
• Weti yakasviba (yebrown, tsvuku, ine ruvara rwetii)
• Kushaya mvura mumuviri
• Kudzikira kweweti
• Kusvotwa
• Kurasikirwa nepfungwa

Chirwere chaMcArdle chinogona kuita kuti zviome kuita mamwe mabasa emuviri. Asi mashoko akanaka ndeekuti,Zvinokwanisika uye hazvina zvazvinokanganisa hutano hwako. Chiremba wako achashanda newe kuti akutsvagire nzira yakanakisisa yekurapa.

Mharidzo Yekuenda Nayo Kumba

Zvakanaka, ngatikuyeuchidzei zvinhu zvishoma zvatataura nezvazvo zvatinofunga kuti zvakakosha kwamuri:

Chirwere cheMcArdle chirwere chinokonzerwa nekushaikwa kweenzyme inobatsira tsandanyama kugadzira simba.

Zviratidzo zvikuru kuneta nekukurumidza, kurwadziwa kwemhasuru, uye kugumburwa panguva yekurovedza muviri.

Ziva dambudziko guru rinonzi `rhabdomyolysis`. Kana ukaona zviratidzo izvi, enda kuna chiremba nekukasika.

Kunyangwe pasina mushonga chaiwo weizvi, zvinogona kurapwa zvakanaka nekurovedza muviri kwakakodzera uye kudya zvakanaka uye unogona kurarama hupenyu hwakajairika.

Kana muine mimwe mibvunzo nezvenyaya iyi, musazeza kutaura nachiremba wenyu.

Yeuka kuti hausi wega. Neruzivo rwakakodzera nerutsigiro, unogona kurarama nemamiriro ezvinhu aya.

` Chirwere cheMcArdle, GSD5, Chirwere cheMcArdle, Kurwadziwa kwetsandanyama, Kurovedza muviri, Chirwere chemajini, Glycogen, Myophosphorylase, Rhabdomyolysis, Kusashivirira Kurovedza Muviri