Cov leeg nqaij hauv koj lub cev puas seev cev? Lawv puas txav zoo li nthwv dej? Cia peb kawm txog Kab Mob Nqaij Ntsws!

Koj puas tau pom lossis xav tias koj cov leeg nqaij co tam sim ntawd, zoo li lawv mus ua nthwv dej, lossis lawv txhua tus sib sau ua ke? Qee zaum koj yuav xav tias nws tsuas yog lwm yam xwb, tab sis nws yeej yog ib qho cim qhia txog ib qho mob tsis tshua muaj tab sis tseem ceeb uas yuav tsum paub txog. Qhov ntawd yog qhov peb yuav tham txog hnub no, Kab Mob Nqaij Ntsws . Tsis txhob txhawj, cia peb ua kom yooj yim.

Kab Mob Rippling Muscle yog dab tsi?

Hais yooj yim xwb, Kab Mob Rippling Muscle yog ib yam mob tsis tshua muaj uas cuam tshuam rau peb lub paj hlwb thiab cov leeg. Cov kws kho mob kuj hu nws ua kab mob neuromuscular . Hauv qhov no, peb cov leeg nqaij yuav qaug zog ntau zaus thiab tsis tuaj yeem tswj tau, ua rau lawv tawv thiab qee zaum loj hlob ntau dhau, uas hu ua hypertrophy .

Cov tsos mob no feem ntau pib thaum yau lig lossis thaum hluas, tab sis nws yog ib qho tseem ceeb uas yuav tsum nco ntsoov tias qee zaum tus mob no tuaj yeem tshwm sim thaum twg los tau.

Cov tsos mob ntawm tus kab mob no yog dab tsi? Muaj dab tsi tshwm sim tiag?

Raws li lub npe qhia, Kab Mob Rippling Muscle Disease feem ntau cuam tshuam rau peb cov leeg nqaij, tshwj xeeb tshaj yog cov leeg nqaij ze tshaj plaws rau nruab nrab ntawm peb lub cev (proximal muscles) . Qhia meej dua, cov leeg nqaij hauv peb lub duav (quadriceps) raug cuam tshuam ntau tshaj plaws.

Yog tias koj muaj (RMD), nws txhais tau tias koj cov leeg nqaij "excitable" lossis "sai excitable." Qhov no txhais tau tias koj cov leeg nqaij teb tsis zoo rau txawm tias qhov kov me me lossis siab.

Cov tsos mob feem ntau ntawm tus kab mob no yog:

• Cov leeg nqaij sib sau ua ke: Yuav kom meej meej, koj cov leeg nqaij sib sau ua ke hauv ib qho chaw thiab ua zoo li "lub pob zeb".
• Kev ua kom cov leeg nqaij nruj dua: Qhov no tuaj yeem kav ntev li 30 vib nas this.

Ob qho tsos mob no feem ntau tshwm sim thaum muaj ib yam dab tsi ntaus koj lub duav tam sim ntawd, xws li thaum koj tsoo ib yam dab tsi. Piv txwv li, yog tias koj tsoo rau hauv lub qhov rooj, koj cov leeg nqaij ntawm koj lub duav yuav maj mam nruj thiab tawv.

Kwv yees li ntawm 60% ntawm cov neeg uas muaj RMD muaj cov leeg nqaij ntswj. Qhov no yog qhov kev ntswj ntawm cov leeg nqaij uas zoo li cov kab nkag mus rau hauv qab daim tawv nqaij. Qhov no kav ntev li ntawm 5 txog 20 vib nas this. Qhov no feem ntau tshwm sim thaum koj ncab cov leeg.

Lwm cov tsos mob uas yuav pom muaj xws li:

• Qaug zog: Tsis yog tsuas yog qaug zog xwb, tab sis kuj yog qaug zog ntau dhau uas los yam tsis muaj laj thawj.
• Mob leeg: Mob leeg sai sai, mob hnyav uas ua rau mob hnyav.
• Cov leeg nqaij tawv:Nws zoo li nyuaj rau khoov lossis tshem cov nqaij.

Cov tsos mob no yuav hnyav zuj zus tom qab ua si nawv hnyav lossis raug cua txias. Hauv qee tus neeg, qee cov leeg nqaij yuav loj dhau (hypertrophy) thiab tus cwj pwm taug kev yuav hloov me ntsis (kev taug kev tsis zoo li qub) .

Tus kab mob no puas mob?

Yog, (Kab Mob Nqaij Ntsws) tuaj yeem ua rau mob nqaij. Tshwj xeeb tshaj yog thaum cov leeg nqaij pheej nruj, qhov mob yuav hnyav dua yog tias nws kav ntev li ib pliag. Tsis tas li ntawd, thaum cov leeg nqaij dov (mob plab) qhov mob tshwm sim. Xav txog seb nws yuav nyuaj npaum li cas yog tias cov leeg nqaij hauv koj ceg nruj tas li.

Dab tsi ua rau muaj kab mob Rippling Muscle Disease?

Kab mob nqaij ntshiv uas ua rau cov leeg nqaij ntswj feem ntau yog los ntawm kev hloov pauv hauv cov noob caj noob ces hu ua CAV3, uas peb tau txais los ntawm peb niam peb txiv. Cov noob caj noob ces CAV3 qhia peb lub cev kom tsim cov protein hu ua caveolin-3 . Cov protein no pom muaj nyob rau hauv daim nyias nyias nyob ib puag ncig cov hlwb nqaij ntshiv. Cov kws tshawb fawb kuj ntseeg tias cov protein no pab tswj cov calcium , uas pab cov leeg nqaij ntswj thiab ncab.

Rau cov neeg uas muaj mob RMD, kev hloov pauv hauv cov noob caj noob ces CAV3 ua rau txo qis kev tsim cov protein caveolin-3. Cov kws tshawb fawb ntseeg tias qhov tsis muaj protein no ua rau cov calcium hauv cov leeg nqaij tsis zoo. Yog li ntawd, cov leeg nqaij yuav nruj tsis zoo thaum muaj kev thawb lossis rub me ntsis.

Muaj ntau hom kev hloov pauv hauv cov noob caj noob ces (CAV3) no. Cov kab mob ntsig txog cov qog nqaij uas tshwm sim los ntawm cov kev hloov pauv ntawm cov noob caj noob ces no hu ua (caveolinopathies) . Ntxiv rau (RMD), ntau lwm yam kab mob uas koom nrog pawg no yog:

• (Autosomal dominant limb-girdle muscular dystrophy) (yav tas los hu ua LGMD1C)
• (Hypertrophic cardiomyopathy) (Qhov no yog kab mob plawv)
• (Kab mob ntshav siab uas raug cais tawm) (Ib qho mob uas cov enzyme creatine kinase hauv cov ntshav siab dua)
• (CAV3-related distal myopathy) (ib yam kab mob uas cuam tshuam rau cov leeg nqaij hauv cov ceg tawv)

Hauv cov mob no, qee zaum cov tsos mob xws li cov leeg nqaij ntswj, xws li cov pob khaus, tuaj yeem pom hauv RMD.

Tsis tas li ntawd xwb, muaj ib hom kab mob Rippling Muscle Disease uas ua rau lub cev tsis ua haujlwm zoo , uas tshwm sim nrog tus kab mob Myasthenia gravis . Cov neeg ntawd tsis muaj qhov kev hloov pauv ntawm CAV3 gene. Qhov ntawd txhais tau tias lawv lub cev tiv thaiv kab mob tawm tsam cov leeg nqaij.

Peb yuav tau txais tus kab mob no li cas (Rippling Muscle Disease)?

Feem ntau, Kab Mob Rippling Muscle Disease yog tau txais los ntawm tus qauv autosomal dominant . Hais yooj yim xwb, qhov no txhais tau tias koj tuaj yeem mob tau yog tias koj tau txais ib daim qauv ntawm cov noob caj noob ces CAV3 uas tau hloov pauv los ntawm koj niam lossis txiv. Nws tuaj yeem tshwm sim txawm tias koj tsuas muaj ib lub noob caj noob ces xwb los xij.

Muaj tej yam tsawg tsawg uas RMD yuav tau txais los ntawm noob caj ces hauv tus qauv autosomal recessive . Qhov no txhais tau tias koj yuav tsum tau txais ob daim qauv ntawm cov noob caj ces CAV3 uas tau hloov pauv lawm, los ntawm koj niam thiab koj txiv.

Cov neeg uas muaj tus kab mob Rippling Muscle Disease hauv daim ntawv autosomal recessive yuav muaj cov tsos mob hnyav dua me ntsis dua li cov neeg uas muaj nws hauv daim ntawv autosomal dominant.

Tsis tshua muaj, RMD kuj tuaj yeem tshwm sim vim muaj kev hloov pauv tshiab, yam tsis muaj keeb kwm tsev neeg. Qhov no txhais tau tias qhov teeb meem caj ces no tuaj yeem tshwm sim hauv lub cev ntawm tus neeg thawj zaug, yam tsis tau txais los ntawm lawv niam lawv txiv.

Cov kws kho mob kuaj mob Rippling Muscle Disease li cas thiaj paub tseeb?

Cov kws kho mob siv cov kev kuaj mob thiab cov txheej txheem no los kuaj mob Rippling Muscle Disease, tab sis tsis yog txhua tus neeg yuav tsum ua tag nrho cov no.

• Keeb kwm kho mob: Tus kws kho mob yuav nug txog koj cov tsos mob thiab seb puas muaj leej twg hauv koj tsev neeg tau muaj RMD lossis lwm yam caveolinopathies.
• Kev kuaj lub cev thiab lub paj hlwb: Tus kws kho mob yuav kuaj xyuas koj cov leeg thiab cov hlab ntsha ua haujlwm li cas.
• Kev kuaj ntshav Creatine kinase: Thaum cov leeg nqaij raug puas tsuaj, cov enzyme no (creatine kinase) nce ntxiv hauv cov ntshav.
• Electromyography (EMG): Qhov no kuaj xyuas kev ua haujlwm hluav taws xob ntawm cov leeg nqaij.
• Kev kuaj cov leeg nqaij: Ib daim me me ntawm cov leeg nqaij raug coj mus kuaj hauv qab lub tshuab tsom iav.
• Kev kuaj mob Antibody: Cov kev kuaj no yog ua los saib seb (RMD) puas yog autoimmune.
• Kev kuaj noob caj noob ces: Qhov no yog txoj hauv kev zoo tshaj plaws los nrhiav seb puas muaj kev hloov pauv hauv (CAV3) noob caj noob ces.

Cov kev kuaj no pab tshem tawm lwm yam kab mob uas muaj cov tsos mob zoo sib xws thiab paub meej tias yog Rippling Muscle Disease.

Thaum lub sijhawm kuaj mob, koj tus kws kho mob yuav xa koj mus rau ib tus kws tshwj xeeb, xws li kws kho mob hlwb lossis kws kho caj ces .

Cov kev kho mob rau qhov no yog dab tsi?

Kev kho mob rau Rippling Muscle Disease nyob ntawm seb nws yog genetic lossis autoimmune.

Kev kho mob caj ces:

Qhov no feem ntau yog kev tswj cov tsos mob thiab xa mus rau kev sab laj txog kev hloov noob caj noob ces . Yog tias qhov kev dov lossis kev ntswj ntawm cov noob qes hnyav heev, qee cov tshuaj yuav pab tau. Cov tshuaj no suav nrog:

• (Dantrolene): Qhov no yog ib yam tshuaj ua kom cov leeg nqaij so.
• (Cov tshuaj calcium uas thaiv cov channel):Cov no yog cov tshuaj uas thaiv cov calcium channel.
• Benzodiazepines: Cov no ua rau cov leeg nqaij so thiab txo kev ntxhov siab.

Kev kho mob tiv thaiv kab mob:

Qhov no tuaj yeem kho tau los ntawm kev siv tshuaj kho mob tiv thaiv kab mob , lossis yog tias koj muaj thymoma (qog qog hauv lub qog thymus), phais kom tshem tawm lub qog thymus (thymectomy) yuav ua tau.

Peb puas tuaj yeem tiv thaiv qhov no (Rippling Muscle Disease) kom tsis txhob tshwm sim?

Vim tias Kab Mob Rippling Muscle feem ntau yog los ntawm noob caj ces, peb yeej tsis muaj dab tsi los tiv thaiv nws. Nws tsis yog ib yam dab tsi uas peb tswj tau.

Txawm li cas los xij, yog tias koj txhawj xeeb txog qhov kev pheej hmoo ntawm kev kis tus kab mob RMD lossis lwm yam kab mob caj ces rau koj tus menyuam ua ntej koj muaj menyuam, nws yog ib qho tseem ceeb heev uas yuav tsum tham nrog koj tus kws kho mob txog kev sab laj txog caj ces kom koj thiaj li nkag siab meej.

Kuv yuav tsum mus ntsib kws kho mob thaum twg?

• Yog tias koj cov tsos mob RMD zuj zus tuaj , lossis yog tias lawv tshwm sim ntau zaus , nco ntsoov nrog koj tus kws kho mob tham.
• Yog tias ib tug neeg hauv koj tsev neeg nyuam qhuav kuaj pom tias muaj tus kab mob Rippling Muscle Disease, nug koj tus kws kho mob seb koj lossis lwm tus neeg hauv tsev neeg puas muaj feem yuav muaj tus kab mob no.

Kuv yuav tsum nug kuv tus kws kho mob li cas?

Tej zaum koj yuav pom tias nws yuav pab tau koj nug koj tus kws kho mob cov lus nug no:

• Kuv ua tau dab tsi los tswj kuv cov tsos mob?
• Txoj kev kho mob twg zoo tshaj plaws rau kuv?
• Kuv cov menyuam puas tau txais tus kab mob Rippling Muscle Disease los ntawm kuv?
• Puas yuav tsum kuaj lwm tus neeg hauv kuv tsev neeg rau tus kab mob Rippling Muscle Disease?

Thaum koj nug cov lus nug no, koj yuav nkag siab zoo dua txog qhov xwm txheej no.

Kab mob Rippling Muscle puas cuam tshuam rau lub plawv?

Kab mob Rippling Muscle Disease tsis cuam tshuam ncaj qha rau lub plawv. Txawm li cas los xij, ob qho tib si RMD thiab hypertrophic cardiomyopathy yog tshwm sim los ntawm kev hloov pauv hauv tib lub gene (CAV3), yog li muaj kev sib txuas ntawm ob hom kab mob. Txawm hais tias tsis tshua muaj heev, kev hloov pauv hauv CAV3 gene tuaj yeem cuam tshuam rau ob qho tib si cov leeg nqaij plawv thiab lwm cov leeg nqaij hauv lub cev. Tab sis nws tsis tshua muaj heev.

Kab mob Rippling Muscle Disease (Kab mob nqaij ntshiv) puas ua rau tuag taus?

Kab mob Rippling Muscle Disease nws tus kheej tsis yog qhov ua rau tuag taus. Qhov ntawd yog, nws tsis yog qhov txaus ntshai rau lub neej. Txawm li cas los xij, cov tsos mob ntawm (RMD) xws li cov leeg nqaij ntswj zoo li wrinkles kuj tuaj yeem yog ib qho tsos mob ntawm lwm yam kab mob (caveolinopathies) uas tshwm sim los ntawm cov kev hloov pauv ntawm cov noob caj noob ces (CAV3).

Ntawm cov kab mob caveolinopathies, cov mob hnyav ntawm autosomal dominant limb-girdle muscular dystrophy thiab hypertrophic cardiomyopathy qee zaum tuaj yeem ua rau tuag taus.

Nws yog ib qho ib txwm muaj uas koj yuav xav tias ntxhov siab thaum koj raug kuaj pom tias muaj tus mob caj ces, tshwj xeeb yog tus mob uas tsis tshua muaj. Tab sis nco ntsoov, koj pab neeg kho mob yuav nyob ntawd los piav qhia txog Kab Mob Rippling Muscle rau koj, qhia koj tias nws yuav cuam tshuam li cas rau koj, thiab pab koj nrhiav txoj hauv kev zoo tshaj plaws los tswj koj cov tsos mob.

Tej yam tseem ceeb tshaj plaws uas yuav tsum nco ntsoov (Cov Lus Coj Mus Tsev)

Zoo, yog li tam sim no koj nkag siab qee yam txog yam peb tau tham txog (Rippling Muscle Disease). Hauv kev xaus:

• Kab Mob Rippling Muscle Disease yog ib qho kab mob tsis tshua muaj uas ua rau cov leeg nqaij co, ntsws, tawv, thiab nkhaus.
• Feem ntau, qhov no yog tshwm sim los ntawm cov kab mob caj ces (CAV3 gene) . Qee zaum, kuj tseem muaj cov kab mob autoimmune ua rau muaj mob.
• Qhov no tsis yog ib yam kab mob uas ua rau tuag taus . Txawm li cas los xij, qee qhov lwm yam kab mob caveolinopathies uas cuam tshuam nrog nws tuaj yeem ua rau mob hnyav.
• Yog koj muaj cov tsos mob no, tsis txhob ntshai thiab nrhiav kev pab kho mob . Qhov tseem ceeb tshaj plaws yog kom tau txais kev kuaj mob kom raug thiab tau txais kev kho mob thiab kev qhia tsim nyog.
• Kev qhia txog noob caj ces tuaj yeem pab tau zoo heev rau cov xwm txheej zoo li no.

Yog koj xav paub ntxiv txog qhov no, lossis yog koj xav tias koj muaj cov tsos mob no, thov mus ntsib kws kho mob. Tsis txhob ntshai, muaj kev daws teeb meem rau txhua yam.

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