Glutaric Aciduria Type I

What is Glutaric Aciduria Type I

Glutaric Aciduria Type I is a rare inherited disorder caused by a deficiency of glutaryl-CoA dehydrogenase, resulting in progressive neurological deterioration. The aim of dietary management is to limit lysine and tryptophan by means of a low protein diet. The protein requirements are met by supplementing the diet with a lysine free and low tryptophan amino acid mixture if necessary.

Carnitine is routinely used, and riboflavin as a vitamin cofactor. Baclofen has also been recommended.

Emergency regimes are critical in periods of decompensation and catabolism.

Bibliography

Baric I, Zschoke J, Christensen B, Duran M, Goodman SI, Leonard JV, Muller E,
Morton DIJ, Superti-Purga A, Hoffman GF. Diagnosis and management of glutaric
aciduria type I. J Inher Metab Dis. 1998;21:326-340

Monavari AA, Naughten ER. Prevention of cerebral palsy in Glutaric Aciduria type I
by dietary management. Arch Dis Child 200;82:67-70

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