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Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy. Related Articles. Lorenzo Odone's Life and Disease. How Is Radiation Sickness Treated? Phillips, Andrea L. Dammann, Robert T. Leshner, Sandra S. Jennings, Joel Avigan, and Virginia K. Moser, Hugo W. Muenz, Ann B.
Moser, Jiahong Xu, Richard O. Jones, et al. Miller, W. Rothman, D. This failure of dietary effect led to the understanding that there must also be a significant production of VLCFA within the body. There have been a variety of attempts to interfere with long-chain fatty acid elongation. The early focus was on the use of mono-unsaturated long-chain fatty acids. These mono-unsaturated long-chain fatty acids are slightly different than the saturated long chain fatty acids, because they have a single double bond in the fat-chain.
Some victims, like Lorenzo Odone portrayed in the film, get the childhood form which usually kills within just two years. Others get the adult form of the disease, which strikes people in their late twenties and acts more slowly. The new study shows that the boys whose parents scrupulously administered the oil — often in the face of scepticism from the medical establishment — were much less likely to go on to develop symptoms in childhood than boys who did not get the oil on a regular basis.
Boys carrying the defect have extremely high levels of very long chain saturated fatty acids in their blood. The oil brings these levels back down to normal in a way that can be tested, and this enabled the researchers to monitor which boys were getting the treatment on a regular basis.
Between and two teams, one led by Moser at the Kennedy Krieger Institute in Baltimore, Maryland, the other based in Europe, looked at the progress of young boys with the defect.
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