This blog is created and edited by Alisha and Nevena,
We have decided to do this blog of adrenoleukodystrophy based on the movie Lorenzo's oil. If you are interested in viewing this movie there is a link in YouTube where it can be viewed;
http://www.youtube.com/watch?v=p1aDnGlT-Sg
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Background on adrenoleukodystrophy:
The first clinical description of ADL was in 1910 by two physicians Haberfeld and Spieler. The boy presented with hyperpigmentation, impaired visual acuity, and his school performance deteriorated. He was hospitalized at the age of 7 and died 8 months later. In 1963 with comparable cases being reported there was enough evidence to assume that it was an X-linked disorder. In 1970 the name adrenoleukodystrophy was introduced based on the striking association of a leukodystrophy with adrenocortical insufficiency. In 1976 an adult form of the disease were also being documented. Today experiments are being developed to try treat and cure this progressive disease.
For more information on the history and advancements in this area please refer to; http://www.x-ald.nl/clinical-diagnosis/historical-overview/
What is Adrenoleukodystrophy?
Adrenoleukodystrophy is an inherited X-linked disorder that disrupts the break down of very-long-chain-fatty-acids. When an individual is not able to break down these VLCFAs there is a build up in the brain, gonads, and adrenal gland resulting is disruption of normal activity. The myelin sheaths of the nerves are progressively damaged because of the faulty gene. Individuals with this disease don't produce an essential protein transporter which carries the enzymes that are needed to breakdown VLCFAs.
Alisha M.
