அனைவருக்கும் வணக்கம், நான் மருத்துவர் வினோத்குமார், MBBS MS. Muscular Dystrophy - தசைநார் அழிவு நோய் பற்றி பலர் சந்தேகங்களை கேட்டிருந்தார்கள் , இந்த பதிவில் அதைப்பற்றி விளக்கியுள்ளேன்.
தசைநார் அழிவு நோய் ஏன் ஏற்படுகிறது ?
muscular dystrophy எத்தனை வகைப்படும் ?
muscular Dystrophy அறிகுறிகள் என்ன ?
Duchenne muscular Dystrophy என்றால் என்ன ?
Limb Girdle Muscular Dystrophy என்றால் என்ன ?
LGMD நோயின் அறிகுறிகள் என்ன ?
LGMD சிகிச்சை முறைகள் என்ன ?
நவீன மருத்துவம் தொடர்பான சந்தேகங்களுக்கு
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What Is Muscular Dystrophy?
Muscular dystrophy (MD) is a group of inherited diseases in which the muscles that control movement (called voluntary muscles) progressively weaken. In some forms of this disease, the heart and other organs are also affected.
There are nine major forms of muscular dystrophy:
Myotonic
Duchenne
Becker
Limb-girdle
Facioscapulohumeral
Congenital
Oculopharyngeal
Distal
Emery-Dreifuss
Duchenne. The most common form of muscular dystrophy in children, Duchenne muscular dystrophy typically affects only males.
It appears between the ages of 2 and 6. The muscles decrease in size and grow weaker over time yet may appear larger.
Disease progression varies, but many people with Duchenne (1 in 3,500 boys) need a wheelchair by the age of 12.
In most cases, the arms, legs, and spine become progressively deformed, and there may be some cognitive impairment.
Severe breathing and heart problems mark the later stages of the disease. Those with Duchenne MD usually die in their late teens or early 20s.
Becker. This form is similar to Duchenne muscular dystrophy, but the disease is much milder: symptoms appear later and progress more slowly.
It usually appears between the ages of 2 and 16 but can appear as late as age 25.
Like Duchenne muscular dystrophy, Becker muscular dystrophy typicallyaffects only males (1 in 30,000) and causes heart problems. Disease severity varies.
Those with Becker can usually walk into their 30s and live further into adulthood.
Limb-girdle. This appears in the teens to early adulthood and affects males and females.
In its most common form, Limb-girdle muscular dystrophy causes progressive weakness that begins in the hips and moves to the shoulders, arms, and legs.
Within 20 years, walking becomes difficult or impossible. Sufferers typically live to middle age to late adulthood
Treatment
Although there's no cure for any form of muscular dystrophy, treatment for some forms of the disease can help extend the time a person with the disease can remain mobile and help with heart and lung muscle strength. Trials of new therapies are ongoing.
Treatment options include medications, physical and occupational therapy, and surgical and other procedures. Ongoing assessments of walking, swallowing, breathing and hand function enable the treatment team to adjust treatments as the disease progresses.
Medications
Your doctor might recommend:
Corticosteroids, such as prednisone and deflazacort (Emflaza), which can help muscle strength and delay the progression of certain types of muscular dystrophy. But prolonged use of these types of drugs can cause weight gain and weakened bones, increasing fracture risk.
Newer drugs include eteplirsen (Exondys 51), the first medication to be approved by the Food and Drug Administration (FDA) specifically to treat some people with Duchenne muscular dystrophy. It was conditionally approved in 2016.
Therapy
Range-of-motion and stretching exercises. .
Exercise. Low-impact aerobic exercise, such as walking and swimming, can help maintain strength, mobility and general health. Some types of strengthening exercises also might be helpful.
Braces. Braces can help keep muscles and tendons stretched and flexible, slowing the progression of contractures. Braces can also aid mobility and function by providing support for weakened muscles.
Mobility aids. Canes, walkers and wheelchairs can help maintain mobility and independence.
Breathing assistance. As respiratory muscles weaken, a sleep apnea device might help improve oxygen delivery during the night. Some people with severe muscular dystrophy need to use a machine that forces air in and out of their lungs (ventilator).
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