Buphenyl tablets 500 mg for urea cycle disorders (sodium phenylbutyrate, Ammonaps, triButyrate)

General information on Japanese Buphenyl tablets 500 mg for urea cycle disorders (sodium phenylbutyrate, Ammonaps, triButyrate)

Package details: 250 tablets

Manufacturer: OrphanPacific Co. Ltd., Japan

Active ingredient: sodium phenylbutyrate (chemical formula C10H11NaO2)

Medical effect: Buphenyl tablets are effective for treatment of urea cycle disorders including following:

• deficiencies of carbamylphosphate synthetase,
• deficiencies of ornithine transcarbamylase,
• deficiencies of argininosuccinic acid synthetase,
• neonatal-onset deficiency and late-onset disease with a history of hyperammonemic encephalopathy.

Contraindications and precautions: do not use in pregnant or breastfeeding women.

If an allergic reaction occurs, stop using the medication and consult with your doctor. If you’re taking any other medication, please consult with your doctor before use.

This medicine requires dietary restrictions and the control of amino acid levels in blood. Plasma levels of ammonia, arginine, branched-chain amino acids, and serum proteins should be maintained within normal limits, and plasma glutamine should be maintained at levels less than 1,000 µmol/L. The treatment should be performed under the monitoring of the professionals.

Dosage and administration of Buphenyl tablets 500 mg from Japan for urea cycle disorders (sodium phenylbutyrate)

For adults and children weighting above 20 kg: 9.9–13.0 g of active ingredient/m2 of body per day. Divide the daily dosage into 3-6 times and take it with or right after each meal. The maximum dosage of 20 g per day should never be exceeded.

The dosage may be adjusted by the consulting doctor according to the blood ammonia level.

How effective are Japanese Buphenyl tablets 500 mg for urea cycle disorders (sodium phenylbutyrate)?

Buphenyl tablets contain sodium phenylbutyrate, a salt of an aromatic fatty acid. It compensates the lack of liver enzymes and helps the kidneys eliminate waste substances from the body. By this, Buphenyl tablets significantly reduce the blood ammonia level.

Who should take Buphenyl tablets 500 mg from Japan?

Buphenyl tablets are effective for treatment of urea cycle disorders involving deficiencies of carbamylphosphate synthetase, ornithine transcarbamylase, or argininosuccinic acid synthetase, including neonatal-onset deficiency and late-onset disease with a history of hyperammonemic encephalopathy. Neonatal-onset enzymatic deficiency used to be considered fatal. However, currently with hemodialysis, use of alternative waste nitrogen excretion pathways (sodium phenylbutyrate, sodium benzoate, and sodium phenylacetate), dietary protein restriction, and, in some cases, essential amino acid supplementation, the survival rate in newborns diagnosed after birth but within the first month of life is almost 80%. In patients diagnosed during gestation and treated prior to any episode of hyperammonemic encephalopathy, survival is 100%. Also, Buphenyl tablets are now studied as a potential treatment to various diseases, including cystic fibrosis, cancer, retinal ischemia and others (T. Iannitti, B. Palmieri. "Clinical and Experimental Applications of Sodium Phenylbutyrate". Drugs in R&D. 2011, September, 11 (3): 227–249).