PMID: 6111630Mar 7, 1981Paper

Hyperornithinaemia and gyrate atrophy of the retina: improvement of vision during treatment with a low-arginine diet

Lancet
R R McInnesJ C McCulloch

Abstract

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 deteriorated at eight months, after which visual function regressed towards pretreatment status. Overrestriction of dietary arginine produced hyperammonaemia, indicating that arginine is an essential aminoacid in ornithine transaminase deficiency. These results suggest that reduction of plasma ornithine may reverse a metabolic neuroretinopathy in this disease, and offer hope that progression of the retinal atrophy can be arrested as well.

References

Oct 1, 1979·Metabolism: Clinical and Experimental·S A ArshinoffE B Marliss

❮ Previous
Next ❯

Citations

Jan 1, 1982·Graefe's Archive for Clinical and Experimental Ophthalmology = Albrecht Von Graefes Archiv Für Klinische Und Experimentelle Ophthalmologie·W Behrens-BaumannU Langenbeck
Feb 3, 2000·Proceedings of the National Academy of Sciences of the United States of America·T WangD Valle
Sep 1, 1981·The British Journal of Ophthalmology·S A ArshinoffE B Marliss
May 26, 2004·Journal of Inherited Metabolic Disease·R SantinelliE Miraglia del Giudice

❮ Previous
Next ❯

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.