Effect of ascorbate or N-acetylcysteine treatment in a patient with hereditary glutathione synthetase deficiency
| Autor: | Nancy G. Kennaway, Johannes Martensson, Peter A. M. Auld, Ajey Jain, Neil R. M. Buist, Berkley R. Powell |
|---|---|
| Rok vydání: | 1994 |
| Předmět: |
medicine.medical_specialty
Antioxidant medicine.medical_treatment Ascorbic Acid Glutathione Synthase Acetylcysteine chemistry.chemical_compound Internal medicine Blood plasma medicine Humans Vitamin E Lymphocytes biology business.industry Glutathione Glutathione synthetase deficiency medicine.disease Ascorbic acid Glutathione synthase Glutathione synthetase Endocrinology chemistry Child Preschool Pediatrics Perinatology and Child Health biology.protein Female business Metabolism Inborn Errors medicine.drug |
| Zdroj: | The Journal of pediatrics. 124(2) |
| ISSN: | 0022-3476 |
| Popis: | A 45-month-old girl with 5-oxoprolinuria (pyroglutamic aciduria), hemolysis, and marked glutathione depletion caused by deficiency of glutathione synthetase was followed before and during treatment with ascorbate or N-acetylcysteine. High doses of ascorbate (0.7 mmol/kg per day) or N-acetylcysteine (6 mmol/kg per day) were given for 1 to 2 weeks without any obvious deleterious side effects. Ascorbate markedly increased lymphocyte (4-fold) and plasma (8-fold) levels of glutathione. N-Acetylcysteine also increased lymphocyte (3.5-fold) and plasma (6-fold) levels of glutathione. After these treatments were discontinued, lymphocyte and plasma glutathione levels decreased rapidly to pretreatment levels. Ascorbate treatment was extended for 1 year, and lymphocyte (4-fold) and plasma (2- to 5-fold) glutathione levels remained elevated above baseline. In parallel, the hematocrit increased from 25.4% to 32.6%, and the reticulocyte count decreased from 11% to 4%. The results demonstrate that ascorbate and N-acetylcysteine can decrease erythrocyte turnover in patients with hereditary glutathione deficiency by increasing glutathione levels. (J P EDIATR 1994;124:229-33) |
| Databáze: | OpenAIRE |
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