Anyone has any more details about this? I remember some reserach hinted that deflazacort+arginine would provide more benefit than deflaz alone. I do not feel that great about it after reading this:
http://www.plosone.org/article/info%3Adoi%2F10.1371%2Fjournal.pone....
Background
Duchenne muscular dystrophy (DMD) is the most common, lethal disease of childhood. One of 3500 new-born males suffers from this universally-lethal disease. Other than the use of corticosteroids, little is available to affect the relentless progress of the disease, leading many families to use dietary supplements in hopes of reducing the progression or severity of muscle wasting. Arginine is commonly used as a dietary supplement and its use has been reported to have beneficial effects following short-term administration to mdx mice, a genetic model of DMD. However, the long-term effects of arginine supplementation are unknown. This lack of knowledge about the long-term effects of increased arginine metabolism is important because elevated arginine metabolism can increase tissue fibrosis, and increased fibrosis of skeletal muscles and the heart is an important and potentially life-threatening feature of DMD.
Methodology
We use both genetic and nutritional manipulations to test whether changes in arginase metabolism promote fibrosis and increase pathology in mdx mice. Our findings show that fibrotic lesions in mdx muscle are enriched with arginase-2-expressing macrophages and that muscle macrophages stimulated with cytokines that activate the M2 phenotype show elevated arginase activity and expression. We generated a line of arginase-2-null mutant mdx mice and found that the mutation reduced fibrosis in muscles of 18-month-old mdx mice, and reduced kyphosis that is attributable to muscle fibrosis. We also observed that dietary supplementation with arginine for 17-months increased mdx muscle fibrosis. In contrast, arginine-2 mutation did not reduce cardiac fibrosis or affect cardiac function assessed by echocardiography, although 17-months of dietary supplementation with arginine increased cardiac fibrosis. Long-term arginine treatments did not decrease matrix metalloproteinase-2 or -9 or increase the expression of utrophin, which have been reported as beneficial effects of short-term treatments.
Conclusions/Significance
Our findings demonstrate that arginine metabolism by arginase promotes fibrosis of muscle in muscular dystrophy and contributes to kyphosis. Our findings also show that long-term, dietary supplementation with arginine exacerbates fibrosis of dystrophic heart and muscles. Thus, commonly-practiced dietary supplementation with arginine by DMD patients has potential risk for increasing pathology when performed for long periods, despite reports of benefits acquired with short-term supplementation.
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I will continue giving 25 grams of l-arginine per day. You will never have proof of long term benefit of anything until long after our sons are gone. Further, in Duchenne, there has been very little correlation between what works in a mouse and what works in a human. And there are constantly studies that contradict each other all over the place. Remember, it took a very long time until scientists and doctors even admitted the benefits of corticosteroids. I pay very little attention to mouse studies. Most of the scientists you meet would tell you that, if their son had Duchenne, they would be giving them these supplements. These science projects tell you very little. And no one is doing serious arginine studies in humans. Long term studies will never happen, especially in supplements. You gotta go with what you feel comfortable with. Don't look to the doctors. If I did, my son would have stopped walking years ago. Instead, he is walking at 14 and a half.
WOW - 14 and 1/2 years and still walking. What do you attribute it to - supplements or perhaps light on the spectrum for DMD? If you don't mind, would you share what mutation your son has and what supplements you give him. Thanks Char Burke
Jeff Sobel said:I will continue giving 25 grams of l-arginine per day. You will never have proof of long term benefit of anything until long after our sons are gone. Further, in Duchenne, there has been very little correlation between what works in a mouse and what works in a human. And there are constantly studies that contradict each other all over the place. Remember, it took a very long time until scientists and doctors even admitted the benefits of corticosteroids. I pay very little attention to mouse studies. Most of the scientists you meet would tell you that, if their son had Duchenne, they would be giving them these supplements. These science projects tell you very little. And no one is doing serious arginine studies in humans. Long term studies will never happen, especially in supplements. You gotta go with what you feel comfortable with. Don't look to the doctors. If I did, my son would have stopped walking years ago. Instead, he is walking at 14 and a half.
Hi; just to share some data that might or might not be useful. My son Hernán has a nonsense mutation (a stop codon that would make him a candidate for Ataluren if this drug ever takes off). His biobsy showed no traces of dystrophin. He stopped walking at age 16. We chose not to put him through tendon release surgery. He is close to 20 now. He has been on deflazacort since diagnosed at age 8. For a short time he also took creatine, but that was all. Because of the corticosteroids his growth was stalled, but fortunately his weight was never a problem. I suppose there is great variation among the different cases.
Hi; just to share some data that might or might not be useful. My son Hernán has a nonsense mutation (a stop codon that would make him a candidate for Ataluren if this drug ever takes off). His biobsy showed no traces of dystrophin. He stopped walking at age 16. We chose not to put him through tendon release surgery. He is close to 20 now. He has been on deflazacort since diagnosed at age 8. For a short time he also took creatine, but that was all. Because of the corticosteroids his growth was stalled, but fortunately his weight was never a problem. I suppose there is great variation among the different cases.
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