Control of Coagulation Abnormalities with Sodium Benzoate in Patients with Argininemia

Abstract

Aim: This study aimed to evaluate coagulation disorders in patients with argininemia associated with arginase 1 gene mutations and the control of these disorders with sodium benzoate treatment. Materials and Methods: Five argininemia patients followed up in the Pediatric Metabolism Clinic in University of Health Sciences Türkiye, Başakşehir Çam and Sakura City Hospital, were included in this study. The patients initially received protein-restricted diet treatment, while their ammonia, platelet count, liver enzymes and coagulation parameters were measured regularly. Later, sodium benzoate was added to the treatment and the same parameters were measured at 1-month intervals. Results: In the coagulation parameters measured after sodium benzoate treatment, one out of the five patients showed complete improvement, three had partial improvement, and one had no change. In the patients with coagulation disorders, factor VII and IX levels were low, while arginine levels remained above 250 µmol/L. Conclusion: The pathophysiology of coagulation disorders in patients with argininemia has not yet been fully elucidated, but it is thought that high arginine levels may affect the synthesis of short-lived coagulation factors. Adding sodium benzoate to dietary therapy may contribute to both the control of arginine levels and an improvement in coagulation parameters. This study demonstrates the importance of coagulation monitoring in those patients with argininemia and the potential therapeutic role of sodium benzoate.

Keywords

• argininemia
• coagulation disturbance
• factor deficiency
• sodium benzoate