Hoʻoiho muscular

ʻO ka mustrular dystrophy kahi hui o nā maʻi i hoʻoili ʻia e kumu ai i ka nāwaliwali o nā mākala a me ka nalo ʻana o nāʻiʻo muscle, e ʻoi aku ana ka maikaʻi ma mua o ka manawa.

ʻO nā mele mustrular, a MD paha, kahi hui o nā kūlana hoʻoilina. ʻO kēia ka mea i hoʻoili ʻia e nā ʻohana. Kū paha lākou i ka wā kamaliʻi a makua paha. Nui a hewahewa nā ʻano like ʻole o ka muscular dystrophy. Pākuʻi ʻia lākou:

• Becker muscular dystrophy
• Duchenne muscular dystrophy
• Emery-Dreifuss muscular dystrophy
• Facioscapulohumeral muscular dystrophy
• Lig-girdle muscular dystrophy
• ʻO ka dystrophy muscular ʻo Oculopharyngeal
• ʻO ka mytronic muscular dystrophy

Hiki i ka mustrular dystrophy ke hoʻopili i nā mākua, akā ʻo nā ʻano ʻoi aku ka paʻakikī i ka wā kamaliʻi.

Kūleʻa nā ʻōuli ma waena o nā ʻano ʻokoʻa o ka mustrular dystrophy. Hoʻopili ʻia nā mākala āpau. A i ʻole, e hoʻopili wale ʻia nā pūʻulu kikoʻī o nā mākala, e like me nā mea e puni ana i ke pelvis, poʻohiwi, a i ʻole ke alo. Hele a mānoanoa ka nāwaliwali o ka mākala a hiki i nā ʻōuli ke komo pū:

• Hoʻolālā ʻia ka hoʻomohala ʻana i nā mākau kaʻa mākala
• Ka paʻakikī e hoʻohana nei i hoʻokahi a ʻoi aku nā pūʻulu ʻili
• Drooling
• E lele i lalo ka eyelid (ptosis)
• Hāʻule pinepine
• Ka nalowale o ka ikaika i kahi mākala a i ʻole hui o nā mākala ma ke ʻano he mākua
• Lilo i ka nui o nā mākala
• Hele pilikia (hele lohi)

Aia ke kīnā ʻole o ka ʻike ma kekahi ʻano o ka mustrular dystrophy.

E kōkua ana kahi hoʻokolohua kino a me kāu mōʻaukala olakino i ka mea mālama ola e hoʻoholo i ke ʻano o ka mustrular dystrophy. Hoʻopili ʻia nā hui puʻupuʻu kikoʻī e nā ʻano like ʻole o ka muscular dystrophy.

Hōʻike paha ka hōʻike:

• ʻO ke kuamoʻo ʻōiwi (scoliosis)
• Nā kuʻikahi hui (clubfoot, claw-hand, a i ʻole nā ​​mea ʻē aʻe)
• Leo haʻahaʻa puʻupuʻu (hiponia)

ʻO kekahi ʻano o ka mustrular dystrophy pili i ka puʻuwai puʻuwai, e hana ana i ka cardiomyopathy a i ʻole ka puʻuwai puʻuwai (arrhythmia).

ʻO ka pinepine, aia ka nalo o ka nui o nā mākala (ʻōpala). He paʻakikī paha kēia e ʻike ai no ka mea ʻo kekahi ʻano o ka mustrular dystrophy ka mea e hoʻokumu i ka momona a me nā mea hoʻopili e hōʻike i ka nui o ka mākala. Kapa ʻia kēia pseudohypertrophy.

Hiki ke hoʻohana ʻia kahi biopsy muso e hōʻoia i ke kuhi. I kekahi mau hihia, hiki i kahi hoʻāʻo koko DNA wale nō ka mea e pono ai.

Hiki i nā hoʻokolohua ʻē aʻe ke:

• Hōʻike puʻuwai - electrocardiography (ECG)
• Ke hoʻāʻo ʻana o ke nerve - conduction nerve and electromyography (EMG)
• ʻImi a me nā hoʻokolohua koko, me ka pae CPK
• ʻO ka hoʻāʻo genetic no kekahi ʻano o ka mustrular dystrophy

ʻAʻohe lāʻau lapaʻau i ʻike ʻia no nā maʻi like ʻole muscular. ʻO ka pahuhopu o ka mālama ʻana e kaohi i nā ʻōuli.

Hiki i ke olakino hoʻoikaika kino ke mālama i ka ikaika a me ka hana o ka mākala. Hiki i nā pale wāwae wāwae a me kahi noho huila ke hoʻomaikaʻi i ka neʻe a me ka mālama pono ʻana iā ia iho. I kekahi mau hihia, kōkua ke kahakaha ma ka iwi kuamoʻo a i ʻole nā ​​wāwae i ka hoʻomaikaʻi ʻana i ka hana.

Lawe ʻia nā Corticosteroids e ka waha i kekahi manawa i nā keiki me kekahi mau puʻupuʻu muscular e hoʻomau iā lākou e hele wāwae no ka hiki.

Pono ke kanaka e hana e like me ka hiki. ʻAʻohe hana iki (e like me ka bedrest) hiki ke hoʻonui i ka maʻi.

Hiki i kekahi poʻe me ka nāwaliwali hanu ke kōkua mai i nā hāmeʻa e kōkua i ka hanu ʻana.

Hiki iā ʻoe ke hoʻomāmā i ke koʻikoʻi o ka maʻi ma ke komo ʻana i kahi hui kākoʻo kahi e puʻunaue ai nā lālā i nā ʻike a me nā pilikia maʻamau.

Aia ka paʻakikī o ke kīnā i ke ʻano o ka mustrular dystrophy. ʻO nā ʻano ʻino āpau o ka mustrular dystrophy e hele a mānoanoa, akā pehea ka wikiwiki o kēia e loli ākea.

ʻO kekahi ʻano o ka muscular dystrophy, e like me Duchenne muscular dystrophy i nā keikikāne, he mea make. Hoʻokumu nā ʻano ʻē aʻe i kahi kīnā liʻiliʻi a he ola maʻamau nā kānaka.

Kāhea i kāu mea hāʻawi inā:

• Loaʻa iā ʻoe nā ʻōuli o ka mustrular dystrophy.
• Loaʻa iā ʻoe kahi moʻolelo pilikino a ʻohana paha o ka mustrular dystrophy a ke hoʻolālā nei ʻoe i nā keiki.

Kūkā ʻia ka ʻōlelo aʻoaʻo genetic ke loaʻa ka moʻolelo ʻohana o ka mustrular dystrophy. ʻAʻohe o nā hōʻailona hōʻailona i nā wahine, akā halihali mau i ka ʻāpana no ka maʻi. Hiki ke ʻike ʻia ka Duchenne mustrular dystrophy me kahi 95% o ka pololei ma o nā loiloi i hana ʻia i ka wā hāpai.

Myopathy hoʻoilina; MD

• Nā mākala mua ma mua
• Nā mākala mua hohonu
• Tendons a me nā mākala
• Nā mākala wāwae lalo

ʻO Bharucha-Goebel DX. Nā maʻi maʻi muscular. I: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. ʻO Puka Textbook of Pediatrics ʻo Nelson. 21st ed. Piladelapia, PA: Elsevier; 2020: chap 627.

Selcen D. Nā maʻi maʻi. I: Goldman L, Schafer AI, eds. ʻO ka lāʻau lapaʻau Goldman-Cecil. 26th ed. Piladelapia, PA: Elsevier; 2020: chap 393.