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BACKGROUND
L-arginine has been recently investigated and proposed to reduce neurological damage after various experimental models of neuronal cellular damage. In this study, we aim to evaluate the beneficial effects of L-arginine administration on the numerical density of dark neurons (DNs) in the
Five patients with gyrate atrophy of the choroid and retina showed a 60% of greater decline in plasma ornithine levels during a five week trial of a low protein (10--15 g/day), low arginine (0.50--0.75 g/day) diet supplemented with essential amino acids and pyridoxine administration. These declines
OBJECTIVE
Both arginine and asymmetric dimethylarginine (ADMA) play a crucial role in the arginine-nitric oxide pathway. Low arginine and high ADMA levels can be found in critically ill patients after major surgery. The aim of this study was to evaluate the effects of low arginine plasma
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
Introduction. Gyrate atrophy is a rare genetical metabolic disorder affecting vision. Here, we report a 9-year-old boy with gyrate atrophy associated with bilateral macular edema at the time of diagnosis and the effect of long term metabolic control on macular edema. Case Presentation. A 9-year-old
This study was aimed at comparing the diagnostic accuracy of the growth hormone (GH) response to clonidine, arginine and both combined in order to establish a more reliable test to differentiate parkinsonism type multiple system atrophy (MSA-p) from Parkinson's disease (PD). Twenty-four patients
OBJECTIVE
To investigate inducible nitric oxide synthase(iNOS) activity of retina and the effects of N(omega)-nitro-L-arginine(N-Arg) on photoreceptor apoptosis in inherited retinal degeneration of Royal College of Surgeons (RCS) rats.
METHODS
iNOS activity was assayed in the whole retinal
We evaluated the arginine growth hormone (GH) stimulation test for the diagnosis of cerebellar-type multiple system atrophy (MSAc) in patients with ataxia. Fourteen subjects with MSAc, 11 with idiopathic late-onset cerebellar ataxia (ILOCA), 10 with familial ataxia, and 10 healthy controls were
OBJECTIVE
Multiple system atrophy (MSA) may be difficult to distinguish from idiopathic Parkinson's disease (PD). Our aim was to evaluate the accuracy of the arginine growth hormone (GH) stimulation test in distinguishing between MSA and PD in large populations of patients.
METHODS
We measured the
In gyrate atrophy the blood ornithine is grossly elevated, due to deficiency of ornithine ketoacid transaminase, which converts ornithine towards glutamic acid. Two patients with gyrate atrophy have been treated with a low arginine diet and their blood ornithine levels have been reduced to near
OBJECTIVE
Multiple system atrophy (MSA) is difficult to distinguish from idiopathic Parkinson's disease (PD) and idiopathic late-onset cerebellar ataxia (ILOCA). This study aimed to evaluate GH response to three different GH stimulation tests in order to establish a reliable test to differentiate
OBJECTIVE
To quantify the effect of long-term reduction of plasma ornithine levels through adherence to an arginine-restricted diet on visual function in patients of all ages with gyrate atrophy of the retina and choroid.
METHODS
A long-term observational study was conducted on 27 patients with
Four patients with gyrate atrophy on low protein (20-35 g/day), low arginine diets and one on vitamin B6 (300 mgm/day) have maintained their plasma ornithine concentrations in the range of 30-60% of pre-therapeutic trial levels for about two years. After two years, three of the five patients have
Ornithine-delta-aminotransferase deficiency is the primary biochemical defect in gyrate atrophy of the choroid and retina and results in the characteristic accumulation of ornithine. An additional consequence of this inborn error is that arginine, the precursor of ornithine, becomes an essential
We report the clinical and genetic data obtained at a 17-year follow-up examination of a patient with gyrate atrophy, without an arginine-restricted diet. Patient examinations included visual acuity (VA), perimetry, biomicroscopy, funduscopy, fundus photography, fundus autofluorescence (FAF),