Attempted dietary treatment of a boy with hyperammonemia due to ornithine transferase deficiency.

Abstract

Dietary treatment of a male patient suffering from the delayed-onset type of OCT deficiency was attempted. Control of the hyperammonemia was attempted by restriction of protein intake, guided by monitoring the plasma ammonia and regular checking of the serum amino acid levels. The influence of supplementary citric acid or lactulose therapy on the plasma ammonia level was investigated and found to be negligible. The therapeutic effect of supplying ornithine and arginine (an essential amino acid in urea cycle disorders) is described. Despite intensive dietary treatment over two and a half years, a incorrigible hyperammonemic crisis resulted in the sudden death of our patient.

• Authors: C van der Heiden, H D Bakker, J Desplanque, M Brink, P K de Bree, S K Wadman
• Journal: European journal of pediatrics (Eur J Pediatr) Vol. 128 Issue 4 Pg. 261-72 (Jul 19 1978) ISSN: 0340-6199 [Print] Germany
• PMID: 668733 (Publication Type: Case Reports, Journal Article)

Chemical References

• Amino Acids
• Citrates
• Dietary Proteins
• Lactulose
• Ammonia
• Arginine
• Ornithine

Topics

• Amino Acid Metabolism, Inborn Errors (diet therapy)
• Amino Acids (blood)
• Ammonia (blood)
• Arginine (therapeutic use)
• Child, Preschool
• Citrates (therapeutic use)
• Dietary Proteins (administration & dosage)
• Humans
• Lactulose (therapeutic use)
• Male
• Ornithine (therapeutic use)
• Ornithine Carbamoyltransferase Deficiency Disease