HYPERORNITHINAEMIA AND GYRATE ATROPHY OF THE RETINA: IMPROVEMENT OF VISION DURING TREATMENT WITH A LOW-ARGININE DIET

A 15-year-old patient with hyperornithinaemia (0.6--1.2 mmol/l) and gyrate atrophy of the retina was given a low-arginine diet to reduce plasma ornithine to a concentration (0.15--0.25 mmol/l) near the normal range. After five weeks of treatment, there was improvement in the visual function of one eye which had been severely impaired without improvement for 3 years. This improved visual function was maintained until compliance with the diet …