Clinical trial of vitamin B6 for gyrate atrophy of the choroid and retina

Richard Weleber, N. G. Kennaway

Research output: Contribution to journalArticle

47 Citations (Scopus)

Abstract

Seven patients with gyrate atrophy and deficiency of ornithine-δ-aminotransferase were studied for in vivo pyridoxine responsiveness; three responded to oral vitamin B6 with over 50% reduction of serum ornithine levels and return to normal of serum lysine levels. Electrophysiologic studies were performed on two B6-responsive patients and one B6-nonresponder over various time periods with and without pyridoxine supplementation. Electroretinogram (ERG) amplitudes improved 100% in one patient when initially given high doses of vitamin B6. Electro-oculogram light-to-dark ratio also improved for this patient. Withdrawal followed by resumption of B6 supplementation was associated with mild worsening followed by improvement of ERG responses respectively in both patients. Long-term follow-up will be needed to assess whether pyridoxine treatment will slow or halt the progression of the disease.

Original languageEnglish (US)
Pages (from-to)316-324
Number of pages9
JournalOphthalmology
Volume88
Issue number4
StatePublished - 1981

Fingerprint

Gyrate Atrophy
Vitamin B 6
Pyridoxine
Clinical Trials
Ornithine
Serum
Lysine
Disease Progression
Light

ASJC Scopus subject areas

  • Ophthalmology

Cite this

Clinical trial of vitamin B6 for gyrate atrophy of the choroid and retina. / Weleber, Richard; Kennaway, N. G.

In: Ophthalmology, Vol. 88, No. 4, 1981, p. 316-324.

Research output: Contribution to journalArticle

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