Yala i farikolo farikolo yɔrɔw bɛ dɔn kɛ wa? Yala u bɛ lamaga i n’a fɔ jikuruw wa? An ka kalan kɛ Rippling Muscle Disease kan!

Yala i delila k’a ye walima k’a ye ko i farikolo yɔrɔw bɛ barika wuli, i n’a fɔ u bɛ taa jikuruw la, walima u bɛɛ lajɛlen bɛ ɲɔgɔn na wa? Tuma dɔw la, i bɛ se k’a miiri ko a ye fɛn wɛrɛ dɔrɔn de ye, nka tiɲɛ na, a bɛ se ka kɛ bana dɔ taamasyɛn ye min man teli ka sɔrɔ, nka a nafa ka bon, i ka kan ka min dɔn. An bɛna kuma o de kan bi, Rippling Muscle Disease . Aw kana aw hakili ɲagami, an k’a kɛ cogo nɔgɔman na.

Rippling Muscle Disease ye mun ye?

N’an y’a fɔ cogo nɔgɔman na, Rippling Muscle Disease ye bana ye min man teli ka sɔrɔ, min bɛ nɔ bila an farikolo ni an farikolo yɔrɔw la. Dɔgɔtɔrɔw fana b' a wele ko farikolojidɛsɛ . O la, an farikolo bɛ to ka sɔgɔsɔgɔ ani u tɛ se ka u yɛrɛ minɛ, o bɛ kɛ sababu ye u ka gɛlɛya ani tuma dɔw la u bɛ bonya kojugu, o min bɛ wele ko hypertrophie .

A ka ca a la, nin taamasiɲɛ ninnu bɛ daminɛ denmisɛnya laban na walima balikuya daminɛ na, nka a nafa ka bon ka aw hakili to a la ko tuma dɔw la, bana in bɛ se ka ye san suguya bɛɛ la.

Nin bana in taamasiɲɛw ye jumɛnw ye? Mun de bɛ ka kɛ tigitigi?

I n’a fɔ a tɔgɔ b’a jira cogo min na, Rippling Muscle Disease bɛ an farikolo degun kosɛbɛ, kɛrɛnkɛrɛnnenya la farikolo minnu ka surun an farikolo cɛmancɛ la kosɛbɛ (muscles proximités) . Kɛrɛnkɛrɛnnenya la, an cɛmancɛ fasaw (quadriceps) de bɛ tɔɔrɔ kosɛbɛ.

Ni (RMD) bɛ aw la, o kɔrɔ ye ko aw farikolo bɛ "nisɔndiya" walima "u bɛ wuli joona." O kɔrɔ ye ko i farikolo bɛ kɛ cogo la min tɛ bɛn hali ni i magara walima ni i ye i degun dɔɔnin.

Nin bana in taamasiɲɛ minnu ka teli ka ye olu ye :

• Muscle mounding (Muscle mounding): Ni an b'a fɛ ka tigitigi, i farikolo-yɔrɔ bɛ ɲɔgɔn lajɛ yɔrɔ kelen na ka kɛ i n'a fɔ "mound".
• Farikoloɲɛnajɛ segin-segin: O bɛ se ka mɛn fo sekɔndi 30 ɲɔgɔn.

A ka ca a la nin taamasiɲɛ fila bɛɛ bɛ sɔrɔ ni fɛn dɔ barika banna ka i senkɔni gosi, i n’a fɔ ni i ye fɛn dɔ gosi. Misali la, ni aw ye da dɔ gosi, aw cɛmancɛ fasa bɛ se ka barika gɛlɛya ani ka gɛlɛya.

RMD bɛ mɔgɔ minnu na, olu 60% ɲɔgɔn bɛ farikolojidɛsɛ sɔrɔ. O ye farikolo yɔrɔ jiginni ye min bɛ i n’a fɔ kɔnɔw bɛ wuluwulu fari jukɔrɔ. O bɛ Kɛ fo sekɔndi 5 fo 20 ɲɔgɔn. O bɛ kɛ tuma caman na ni aw ye farikolo yɔrɔ dɔ kɔrɔta.

Taamasiɲɛ wɛrɛ minnu bɛ se ka ye, olu ye:

• Sɛgɛn: Sɛgɛn dɔrɔn tɛ, nka sɛgɛn kojugu min bɛ na ni kun tɛ.
• Farikolodimi: Farikolodimi barikama, min ka jugu, min bɛ dimi jugumanba bila mɔgɔ la.
• Farikolo gɛlɛya: .A bɛ kɛ i n’a fɔ a ka gɛlɛn ka sogo kuru walima k’a kuru.

Nin taamasiɲɛ ninnu bɛ se ka juguya farikoloɲɛnajɛ gɛlɛn kɔfɛ walima farigan kɛlen kɔfɛ. Mɔgɔ dɔw la, farikolo dɔw bɛ se ka bonya cogo la min tɛ deli ka kɛ (hypertrophie) ani taamacogo bɛ se ka Changé dɔɔni (taamacogo atypical) .

Yala nin bana in bɛ dimi wa?

Ɔwɔ, (Rippling Muscle Disease) bɛ se ka farikolodimi bila mɔgɔ la. Kɛrɛnkɛrɛnnenya la ni fasa bɛ to ka sɔgɔsɔgɔ, dimi bɛ juguya ni a bɛ mɛn sen na waati dɔ kɔnɔ. Ani fana, ni fasa bɛ ka girin (kɛrɛnkɛrɛnnenya la) dimi bɛ kɛ. Miiri k’a filɛ a bɛna gɛlɛya cogo min na ni i sen fasa dɔ tun bɛ ka gɛlɛya tuma bɛɛ.

Mun de bɛ Rippling Muscle Disease lase mɔgɔ ma?

Rippling Muscle Disease ka teli ka sɔrɔ fɛn caman cili fɛ jamu dɔ la min bɛ wele ko CAV3, an bɛ o ciyɛn sɔrɔ an bangebagaw fɛ. CAV3 jamu b’a fɔ an farikolo ye ko a ka farikolojɔlifɛn dɔ dilan min bɛ wele ko caveolin-3 . O farikolojɔlifɛn in bɛ sɔrɔ farikolo yɔrɔw lamini na. Ɲininikɛlaw dalen b’a la fana ko nin farikolojɔli dumuni in bɛ dɛmɛ ka kalisiyɔmu hakɛ kɔlɔsi, o min bɛ farikolo yɔrɔw dɛmɛ ka u sɔgɔsɔgɔ ani ka u senw kɔrɔta.

RMD bɛ mɔgɔ minnu na, fɛn dɔ jiginni CAV3 jamu la, o bɛ kɛ sababu ye ka dɔ bɔ farikolojɔli dumuni na min tɔgɔ ye ko caveolin-3. Ɲininikɛlaw dalen b’a la ko o farikolojɔli dumuni dɛsɛ in bɛ kɛ sababu ye ka farikolokisɛw ka kalisiyɔmu hakɛ labɛn ka ɲɛ. O de kosɔn, farikolo yɔrɔw bɛ ɲɔgɔn minɛ cogo la min tɛ cogo la, ka kɛɲɛ ni u gɛrɛgɛrɛ walima u samali dɔɔni ye.

Yɛlɛma suguya caman bɛ nin (CAV3) jamu in na. Bana minnu bɛ sɔrɔ sogo glandew la minnu bɛ sɔrɔ o jamu caman sɛgɛsɛgɛli fɛ, olu bɛ wele ko (caveolinopathies) . Ka fara (RMD) kan, bana damadɔ wɛrɛ minnu bɛ nin kulu in kɔnɔ olu ye :

• (Autosomal dominant limb-girdle muscular dystrophy) (a tun bɛ wele fɔlɔ ko LGMD1C) .
• (Hypertrophique cardiomyopathie) (Nin ye dusukunnabana ye) .
• (Isolated hyperCKemia) (Bana min na, anzimu kreyatini kinase bɛ wuli joli la) .
• (CAV3-related distal myopathy) (bana min bɛ farikolo yɔrɔw minɛ) .

Nin bana ninnu na, tuma dɔw la, taamasiɲɛw i n’a fɔ farikolojidɛsɛ, i n’a fɔ kurukuru, olu bɛ se ka ye (RMD) la.

Ani fana, Rippling Muscle Disease suguya dɔ min bɛ sɔrɔ farikolo tangacogo fɛ , o min bɛ sɔrɔ ni farikolo tangacogo bana ye min tɔgɔ ye ko Myasthenia gravis, o fɔra. O mɔgɔw tɛ ni CAV3 jamu jiginni ye. O kɔrɔ ye k’u yɛrɛ ka farikolo tangalanw bɛ bin farikolo yɔrɔw kan.

An bɛ nin (Rippling Muscle Disease) in ciyɛn ta cogo di?

A ka ca a la, Rippling Muscle Disease bɛ sɔrɔ ciyɛn na ni autosomal dominant pattern ye . N’an y’a fɔ cogo nɔgɔman na, o kɔrɔ ye ko bana in bɛ se ka sɔrɔ i la ni i ye CAV3 jamu caman cili ɲɔgɔn sɔrɔ i ba walima i fa fɛ. A bɛ se ka kɛ hali ni jeninida kelen dɔrɔn de bɛ aw bolo.

A man teli ka kɛ, RMD bɛ se ka ciyɛn sɔrɔ otosomali kɔsegincogo la . O kɔrɔ ye ko aw ka kan ka CAV3 jamu caman ciyɛntalen fila ciyɛn ta, ka bɔ aw ba ni aw fa fila bɛɛ la.

Mɔgɔ minnu bɛ ni Rippling Muscle Disease ye otosomal recessive cogo la, olu taamasiɲɛw bɛ se ka juguya dɔɔni ka tɛmɛ a bɛ mɔgɔ minnu kan otosomal dominant cogo la.

A man teli kosɛbɛ, RMD fana bɛ se ka sɔrɔ fɛn falen kuraw fɛ, ni denbaya ka tariku si tɛ a la. O kɔrɔ ye ko nin jamu dɛsɛ in bɛ se ka wuli mɔgɔ fari la a siɲɛ fɔlɔ la, k’a sɔrɔ a ma ciyɛn sɔrɔ a bangebagaw fɛ.

Dɔgɔtɔrɔw bɛ Rippling Muscle Disease sɛgɛsɛgɛ cogo tigitigi la?

Dɔgɔtɔrɔw bɛ nin sɛgɛsɛgɛliw ni fɛɛrɛ ninnu kɛ walasa ka Rippling Muscle Disease sɛgɛsɛgɛ, nka bɛɛ man kan ka ninnu bɛɛ kɛ.

• Furakɛcogo : Dɔgɔtɔrɔ bɛna aw ɲininka aw ka bana taamasiɲɛw la ani ni aw ka denbaya kɔnɔ mɔgɔ dɔ ye RMD walima caveolinopathie wɛrɛw sɔrɔ.
• Farikolo ni hakiliɲagami sɛgɛsɛgɛliw: Dɔgɔtɔrɔ bɛna aw farikolo ni aw farikolo yɔrɔw cogoya lajɛ.
• Kɛrɛyatini kinase joli sɛgɛsɛgɛli : Ni farikolo yɔrɔw tiɲɛna, o anzimu (kɛrɛyatini kinase) bɛ caya joli la.
• Electromyography (EMG): O bɛ farikolo yɔrɔw kuran baara sɛgɛsɛgɛ.
• Muscle biopsy : Farikolo yɔrɔ fitinin dɔ bɛ ta ka a lajɛ ni mikroskɔpu ye.
• Banakisɛfagalanw sɛgɛsɛgɛli : Nin sɛgɛsɛgɛli ninnu bɛ kɛ walasa ka a dɔn ni (RMD) ye farikolo tangalan ye.
• Jɛnkulu sɛgɛsɛgɛli : Nin ye fɛɛrɛ ɲuman ye walasa ka a dɔn ni fɛn dɔw b’a la (CAV3) jamu la.

Nin sɛgɛsɛgɛliw bɛ dɛmɛ ka bana wɛrɛw bɔ kɛnɛ kan minnu taamasiɲɛw bɛ ɲɔgɔn ta ani ka Rippling Muscle Disease (Rippling Muscle Disease) dafa.

Sɛgɛsɛgɛli kɛtuma na, dɔgɔtɔrɔ bɛ se k’i bila dɔgɔtɔrɔ kɛrɛnkɛrɛnnen dɔ fɛ, i n’a fɔ hakiliɲagami dɔgɔtɔrɔ walima jamu dɔnbaga .

O furakɛcogo jumɛnw bɛ yen?

Rippling Muscle Disease furakɛli bɛ bɔ a la ni a bɛ sɔrɔ jamu fɛ walima ni a bɛ sɔrɔ farikolo tangacogo fɛ.

Furakɛli min bɛ sɔrɔ jamu fɛ:

o bɛ tali kɛ kosɛbɛ bana taamasiɲɛw kɔlɔsili la ani ka taa ladilikanw di u ma jamu siratigɛ la . Ni cɛya jiginni walima a dɔgɔyali ka jugu kosɛbɛ, fura dɔw bɛ se ka dɛmɛ don. O fura suguw dɔw ye:

• (Dantrolene): Nin ye farikolo lafiyalan ye.
• (Kalisiyɔmu kanal antagonistes):Olu ye furaw ye minnu bɛ kalisiyɔmu siraw bali.
• Benzodiazepines : Olu bɛ farikolo lafiya ani ka dɔ bɔ jɔrɔnanko la.

Banakisɛfagalanw ka furakɛli:

O bɛ se ka furakɛ ni farikolo tangalanw ye , walima ni timoma (tumu dɔ bɛ timisɛri la), opereli bɛ se ka kɛ ka timisi gland bɔ (thymectomy) .

Yala an bɛ se ka nin (Rippling Muscle Disease) bali ka kɛ wa?

Ikomi Rippling Muscle Disease ka teli ka kɛ jamu ye, tiɲɛ na, foyi tɛ yen an bɛ se ka min kɛ walasa k’a bali. O tɛ fɛn ye an bɛ se ka min mara.

Nka, ni aw bɛ hami farati la ka RMD walima bana wɛrɛw lase aw den ma sani aw ka den sɔrɔ, a nafa ka bon kosɛbɛ aw ka kuma aw ka dɔgɔtɔrɔ fɛ jamu ladilikanw kan walasa aw ka se ka faamuyali jɛlen sɔrɔ.

Tuma jumɛn na ne ka kan ka taa dɔgɔtɔrɔso la?

• Ni aw ka RMD taamasiɲɛw bɛ juguya , walima ni u bɛ to ka caya , aw ye aw jija ka kuma aw ka dɔgɔtɔrɔ fɛ.
• Ni aw ka denbaya kɔnɔmɔgɔ dɔ sɔrɔla kɔsa in na ni Rippling Muscle Disease ye, aw bɛ dɔgɔtɔrɔ ɲininka ni aw walima denbaya kɔnɔmɔgɔ wɛrɛw bɛ se ka nin bana in sɔrɔ.

Ne ka kan ka ɲininkali jumɛnw kɛ n ka dɔgɔtɔrɔ la?

Aw bɛ se ka a ye ko a nafa ka bon ka nin ɲininkali ninnu kɛ aw ka dɔgɔtɔrɔ la:

• Ne bɛ se ka mun kɛ walasa ka ne ka bana taamasiɲɛw kunbɛn?
• Furakɛli fɛɛrɛ jumɛn ka fisa ne ma?
• Yala ne denw bɛ se ka Rippling Muscle Disease ciyɛn ta ne fɛ wa?
• Yala ne ka denbaya kɔnɔmɔgɔ tɔw ka kan ka sɛgɛsɛgɛli kɛ ni Rippling Muscle Disease tɛ wa?

Ni aw ye nin ɲininkali ninnu kɛ, aw bɛna se ka nin ko in faamuya ka ɲɛ.

Yala Rippling Muscle Disease bɛ nɔ bila dusukun na wa?

Rippling Muscle Disease tɛ dusukun minɛ k’a ɲɛsin a ma. Nka, RMD ni dusukunnabana camanba fila bɛɛ bɛ sɔrɔ fɛn falenw fɛ jamu kelen na (CAV3), o la, jɛɲɔgɔnya bɛ bana fila ninnu cɛ. Hali ni a man teli kosɛbɛ, fɛn caman cili CAV3 jamu la, o bɛ se ka nɔ bila dusukun fasa ni farikolo yɔrɔ wɛrɛw la. Nka a man ca kosɛbɛ.

Yala (Rippling Muscle Disease) bɛ mɔgɔ faga wa?

Rippling Muscle Disease yɛrɛ tɛ mɔgɔ faga. O kɔrɔ ye ko a tɛ mɔgɔ ka ɲɛnamaya bila farati la. Nka (RMD) taamasiɲɛw i n’a fɔ farikolojidɛsɛ i n’a fɔ kurukuruw, olu fana bɛ se ka kɛ bana wɛrɛw taamasiɲɛ ye (caveolinopathies) minnu bɛ sɔrɔ jamu caman cili fɛ minnu kofɔlen don ka tɛmɛ (CAV3).

Caveolinopathies la, autosomal dominant limb-girdle muscular dystrophy ani hypertrophic cardiomyopathie jugumanw bɛ se ka kɛ mɔgɔ ka ɲɛnamaya bila farati la tuma dɔw la.

A bɛnnen don ka i yɛrɛ degun ni a sɔrɔla ko bana dɔ bɛ i la min bɛ sɔrɔ jamu fɛ, kɛrɛnkɛrɛnnenya la bana min man teli ka sɔrɔ. Nka aw hakili to a la ko aw ka dɔgɔtɔrɔso jɛkulu bɛna kɛ yen walasa ka Rippling Muscle Disease ɲɛfɔ aw ye, k’a fɔ aw ye a bɛna nɔ bila aw la cogo min na, ani ka aw dɛmɛ ka aw ka bana taamasiɲɛw kunbɛncogo ɲuman sɔrɔ.

Fɛn minnu nafa ka bon kosɛbɛ an ka kan ka to an hakili la (Take-Home Message) .

Okey, donc sisan aw ye faamuyali dɔ sɔrɔ an tun bɛ kuma min kan (Rippling Muscle Disease). Kuma surun na:

• Rippling Muscle Disease ye farikolo bana ye min man teli ka sɔrɔ, a taamasiɲɛw ye farikolojidɛsɛ, kurukuruni, gɛlɛya ani kurukuruni ye.
• A ka ca a la, o bɛ sɔrɔ jamu sababuw fɛ (CAV3 jamu) . Tuma dɔw la, farikolo tangalanw fana bɛ se ka sɔrɔ.
• nin tɛ bana ye min bɛ mɔgɔ faga . Nka, caveolinopathie wɛrɛ minnu bɛ sɔrɔ a la, olu dɔw bɛ se ka juguya.
• Ni nin taamasiɲɛ ninnu bɛ aw la, aw kana siran ani ka dɔgɔtɔrɔ ka ladilikan ɲini . Min nafa ka bon kosɛbɛ, o ye ka bana sɛgɛsɛgɛli tigitigi sɔrɔ ani ka furakɛli ni ladilikan wajibiyalenw sɔrɔ.
• Ladilikan minnu bɛ kɛ jamu kan, olu bɛ se ka nafa sɔrɔ kosɛbɛ nin ko suguw la.

Ni aw b’a fɛ ka fɛn caman dɔn nin ko in kan, walima ni aw hakili la ko nin taamasiɲɛ ninnu bɛ aw la, aw bɛ taa dɔgɔtɔrɔso la. Aw kana siran, fura bɛ fɛn bɛɛ la.

` Rippling Muscle Disease, farikolojidɛsɛ, farikolojidɛsɛ, CAV3 jamu, farikolo dimi, jamu banaw, farikolo tangalanw ka banaw