ISSN:
1432-1076
Keywords:
Dihydrolipoyl dehydrogenase
;
α-Ketoacids
;
Branched-chain amino acids
;
Restricted diet
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Abstract A patient with a deficiency of dihydrolipoyl dehydrogenase and neurological disease is described. The patients was placed on a branched-chain amino acid-restricted regimen. After the introduction of the regimen, there were some biochemical improvements and he achieved some developmental milestones, in contrast to previously reported patients whose neurological disease was progressive. Restriction of the branched-chain amino acids is worth trying among therapeutic measures for this disease, although restriction of the amino acids alone may not totally prevent progression of neurological disease.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00439399
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