PMID: 15243994Jul 13, 2004Paper

Acute metabolic crisis with extreme deficiency of glutathione in combination with decreased levels of leukotriene C4 in a patient with glutathione synthetase deficiency

Journal of Inherited Metabolic Disease
E MayatepekH Gröbe

Abstract

A 32-year-old man with glutathione synthetase deficiency developing an acute metabolic crisis is described. During this acute episode, intracellular glutathione content in erythrocytes was below the detection limit (<0.3 mmol/L). Leukotriene C4 in CSF and urinary leukotriene E4 were massively decreased, indicating an imparied synthesis of cysteinyl leukotrienes. Clinical recovery after one week was accompanied by a clear improvement of these biochemical parameters. The highly disturbed glutathione synthesis is postulated to be the reason for a deficient synthesis of cysteinyl leukotrienes, which may at least in part be responsible for the severe clinical symptoms.

References

Nov 15, 1976·Clinica Chimica Acta; International Journal of Clinical Chemistry·A LarssonU Porath
Jun 2, 1978·Deutsche medizinische Wochenschrift·U Porath, K Schreier
Jul 11, 2001·The Journal of Pediatrics·E RistoffA Larsson

❮ Previous
Next ❯

Related Concepts

Related Feeds

Anxiety Disorders

Discover the latest research on anxiety disorders including agoraphobia, panic disorder, obsessive-compulsive disorder, and post-traumatic stress disorder here.

CSF & Lymphatic System

This feed focuses on Cerebral Spinal Fluid (CSF) and the lymphatic system. Discover the latest papers using imaging techniques to track CSF outflow into the lymphatic system in animal models.