The muscular dystrophies zimagwirizana ndi banja la matenda a minyewa omwe amadziwika ndi kufooka komanso kuchepa kwamphamvu kwa minofu: ulusi wa minofu ya thupi umachepa. Minofu pang'onopang'ono atrophy, ndiko kuti, iwo amataya voliyumu ndipo motero mphamvu zawo.

Izo ziri matenda choyambirira majini zomwe zingawonekere pa msinkhu uliwonse: kuyambira kubadwa, ubwana kapena ukalamba. Pali mitundu yoposa 30 yomwe imasiyana ndi zaka zoyambira zizindikiro, chikhalidwe cha minofu yomwe imakhudzidwa ndi kuuma kwake. Ambiri a muscular dystrophies amakula pang'onopang'ono. Panopa palibe mankhwala. Chodziwika bwino komanso chodziwika bwino cha muscular dystrophies ndi Duchenne muscular dystrophy, amatchedwanso "Duchenne muscular dystrophy".

Mu muscular dystrophy, minofu yomwe imakhudzidwa ndi muscular dystrophy imakhala makamaka yomwe imalola mayendedwe odzifunira, makamaka minofu, ntchafu, miyendo, mikono ndi manja. M'ma dystrophies ena, minofu yopuma ndi mtima zimatha kukhudzidwa. Anthu omwe ali ndi vuto la muscular dystrophy amatha kusiya kuyenda pang'onopang'ono poyenda. Zizindikiro zina zimatha kugwirizana ndi kufooka kwa minofu, makamaka mtima, m'mimba, mavuto a maso, ndi zina zotero.

Kukula

The muscular dystrophies ndi ena mwa matenda osowa ndi amasiye. Ndizovuta kudziwa pafupipafupi, chifukwa zimabweretsa matenda osiyanasiyana. Kafukufuku wina amayerekezera kuti munthu mmodzi mwa anthu atatu aliwonse ali nacho.

Pakuti exemple:

• La myopathy duchenne¹ duchenne¹zimakhudza pafupifupi mmodzi mwa anyamata 3500.
• Becker muscular dystrophy imakhudza mnyamata mmodzi mwa 1²,
• Facioscapulohumeral dystrophy imakhudza pafupifupi munthu mmodzi mwa anthu 1.
• La maladie d'Emery-Dreifuss, zimakhudza 1 mwa anthu 300 ndipo zimayambitsa kubweza kwa tendon ndi kuwonongeka kwa minofu ya mtima

Matenda ena a muscular dystrophies amapezeka kwambiri m'madera ena a dziko lapansi. Potero: 

• Zomwe zimatchedwa Fukuyama congenital myopathy makamaka zimakhudza Japan.
• Ku Quebec, kumbali ina, kuli oculopharyngeal muscular dystrophy zomwe zimalamulira (mlandu umodzi pa anthu 1), pomwe ndizosowa kwambiri padziko lonse lapansi (zochitika 1 pa munthu mmodzi pa avareji¹) Monga momwe dzinalo likusonyezera, matendawa amakhudza kwambiri minofu ya zikope ndi mmero.
• Kumbali yake, Matenda a Steinert kapena “Steinert's myotonia”, ndiyofala kwambiri m’chigawo cha Saguenay-Lac St-Jean, kumene imakhudza munthu mmodzi mwa anthu 1 alionse.
• The sarcoglycanopathies ndizofala kwambiri kumpoto kwa Africa ndipo zimakhudza munthu mmodzi mwa anthu 200 kumpoto chakum'mawa kwa Italy.
• Calpainopathy zinafotokozedwa koyamba ku Reunion Island. Munthu mmodzi pa anthu 200 aliwonse amakhudzidwa.

Zimayambitsa

The muscular dystrophies ndi matenda obadwa nawo, ndiko kunena kuti chifukwa cha kusokonezeka (kapena kusintha) kwa jini yofunikira kuti minofu igwire bwino ntchito kapena chitukuko chawo. Jinili likasinthidwa, minofu simathanso kugwirizanitsa bwino, imataya mphamvu ndi atrophy.

Ma jini angapo osiyanasiyana amakhudzidwa ndi ma muscular dystrophies. Nthawi zambiri, awa ndi majini omwe "amapanga" mapuloteni omwe amakhala mu nembanemba ya maselo a minofu.³.

Pakuti exemple:

• Duchenne muscular dystrophy myopathy imalumikizidwa ndi kuchepa kwa thupi dystrophine, puloteni yomwe ili pansi pa nembanemba ya maselo a minofu ndipo imagwira ntchito yochepetsera minofu.
• Pafupifupi theka la congenital muscular dystrophies (omwe amawonekera pakubadwa), ndikusowa kwa merosine, gawo la nembanemba ya maselo a minofu, yomwe imakhudzidwa.

Monga ambiri matenda obadwa nawo, matenda a muscular dystrophies kaŵirikaŵiri amapatsiridwa ndi makolo kwa ana awo. Nthawi zambiri, amathanso "kuwonekera" modzidzimutsa, pamene jini imasintha mwangozi. Pamenepa, jini la matenda silipezeka mwa makolo kapena achibale ena.

Nthawi zambiri, kupweteka kwa thupi imafalitsidwa m'njira wokhazikika. M’mawu ena, kuti matendawa asonyezedwe, makolo onse aŵiri ayenera kukhala onyamulira ndi kupatsira mwana jini yachilendo. Koma matendawa samadziwonetsera okha mwa makolo, chifukwa aliyense amanyamula jini imodzi yokha yachilendo ya makolo osati chibadwa cha makolo. Komabe, jini imodzi yachibadwa ndiyokwanira kuti minofu igwire ntchito bwino.

Kuphatikiza apo, ma dystrophies ena amangokhudza anyamata : izi ndizochitika ndi Duchenne muscular dystrophy ndi Becker's muscular dystrophy. Muzochitika zonsezi, jini yomwe imakhudzidwa ndi matenda awiriwa ili pa X chromosome yomwe imapezeka mumtundu umodzi mwa amuna.

Mabanja awiri akuluakulu

Pali mabanja awiri akuluakulu a muscular dystrophies:

- pa muscular dystrophies kunena kobadwa nako (DMC), yomwe imapezeka m'miyezi 6 yoyambirira ya moyo. Pali pafupifupi mitundu khumi ya izo, za kuopsa kosiyanasiyana, kuphatikizapo CMD yokhala ndi vuto lalikulu la merosin, Ullrich's CMD, stiff spine syndrome ndi Walker-Warburg syndrome;

- pa muscular dystrophies kuwonekera kenako ubwana kapena ukalamba, monga zitsanzo³ :

• Duchenne muscular dystrophy
• Matenda a myopathy a Becker
• Emery-Dreyfuss myopathy (pali mitundu ingapo)
• Facioscapulo-humeral myopathy, yomwe imatchedwanso Landouzy-Déjerine myopathy
• The myopathies la malamba, otchedwa, chifukwa makamaka zimakhudza minofu yomwe ili mozungulira mapewa ndi m'chiuno.
• Myotonic dystrophies (mitundu I ndi II), kuphatikizapo matenda a Steinert. Iwo amadziwika ndi a myotonie, ndiko kuti, minofu imalephera kumasuka bwino pambuyo pa kutsika.
• Oculopharyngeal myopathy

Evolution

Kusintha kwa muscular dystrophies amasinthasintha kwambiri kuchokera ku mawonekedwe kupita ku ena, komanso kuchokera kwa munthu kupita ku wina. Mitundu ina imasintha msangamsanga, zomwe zimapangitsa kuti munthu asamayende bwino komanso nthawi zina amapha mtima kapena kupuma, pomwe ena amasintha pang'onopang'ono kwa zaka zambiri. Ma congenital muscular dystrophies, mwachitsanzo, sapita patsogolo pang'ono kapena sapita konse, ngakhale kuti zizindikiro zimatha kukhala zowopsa nthawi yomweyo.³.

Mavuto

Zovuta zimasiyanasiyana malinga ndi mtundu wa muscular dystrophy. Ma dystrophies ena amatha kukhudza minofu yopuma kapena mtima, nthawi zina ndi zotsatira zoyipa kwambiri.

Motero, mavuto a mtima ndizofala kwambiri, makamaka kwa anyamata omwe ali ndi vuto la Duchenne muscular dystrophy.

Komanso, a kuchepa kwa minofu zimapangitsa kuti thupi ndi zimfundo ziwonongeke pang'onopang'ono: odwala amatha kudwala scoliosis. Kufupikitsa kwa minofu ndi tendon nthawi zambiri kumawonedwa, zomwe zimapangitsa kubweza kwa minofu (kapena tendons). Kuukira kosiyanasiyana kumeneku kumabweretsa kupunduka kolumikizana: mapazi ndi manja amatembenuzira mkati ndi pansi, mawondo kapena zigongono zimapunduka ...

Pomaliza, ndizofala kuti matendawa ayende limodzi nkhawa kapena kupsinjika maganizo zomwe ziyenera kusamalidwa.