Sodium Phenylbutyrate Granules for Urea Cycle Disorders Treatment

Sodium phenylbutyrate granules, also known as Hiper-Amonemix® 940 mg/g Granül, is a product from Assos Pharmaceuticals in Turkey. It is packaged in 266 g HDPE containers and is a white granular powder. Each 1 g of granule contains 940 mg of sodium phenylbutyrate. This medication is used to treat urea cycle disorders, a rare disorder characterized by the deficiency of certain liver enzymes that are not able to remove nitrogen waste from the body.

Uses of Sodium Phenylbutyrate Granules

Hiper-Amonemix® helps to reduce the amount of ammonia in the body and assists in the removal of nitrogen waste. It is indicated for newborns with full enzyme deficiencies that occur within the first 28 days of life, as well as for patients with a history of hyperammonemic encephalopathy with partial enzyme deficiencies that occur after the first month of life. The dosage for patients weighing less than 20 kg is 450-600 mg/kg/day, and for those weighing more than 20 kg, it is 9.9-13.0 g/m2/day. When taken orally, the measured dose should be mixed with solid or liquid food as recommended by your doctor. Before preparation, shake lightly. For patients with gastrostomy or nasogastric tubes, granules should be dissolved in water (until no dry granules remain). The dissolved liquid should be used immediately.

Benefits of Sodium Phenylbutyrate Granules

The use of sodium phenylbutyrate granules can provide many benefits for those suffering from urea cycle disorders. It reduces the amount of ammonia in the body, which in turn helps to decrease the symptoms associated with these conditions. Additionally, it can help to improve the elimination of nitrogen waste from the body, allowing for better overall health. Finally, this medication can also help to prevent further complications, such as hyperammonemic encephalopathy.

Conclusion

Sodium phenylbutyrate granules from Assos Pharmaceuticals in Turkey can be an effective treatment option for those who suffer from urea cycle disorders. By reducing the amount of ammonia in the body and assisting in the removal of nitrogen waste, this medication can help to alleviate symptoms and improve overall health. Patients should consult their doctor before starting any new medications, including sodium phenylbutyrate granules.