Sodium Phenylbutyrate 100g Oral Powder

Product Introduction

Sodium Phenylbutyrate oral powder helps control ammonia in urea-cycle disorders, where the body cannot dispose of nitrogen normally. It is used with a protein-managed diet for long-term control, rather than as emergency treatment for a sudden ammonia crisis.

How to use

Measure each dose with a calibrated measuring device or an accurate weighing method. Divide the daily amount between meals or feeds, commonly three to six times daily, and mix with a small amount of suitable food or liquid for immediate use. The daily amount is calculated from body weight or body-surface area.

How it works

Sodium phenylbutyrate is converted to phenylacetate, which binds glutamine to form phenylacetylglutamine. Excretion of this compound in urine removes nitrogen by a pathway that bypasses the defective urea cycle.

Side effects

Unpleasant taste or body odour, reduced appetite, nausea, vomiting, abdominal discomfort and menstrual changes can occur. Excess exposure can affect alertness and nervous-system function. New lethargy or confusion can also indicate rising ammonia and requires urgent testing.

Important information

Ammonia, glutamine and nutritional markers guide ongoing treatment. The sodium burden matters in heart failure, oedema or significant kidney disease. Valproate and corticosteroids can worsen ammonia control, while probenecid can affect metabolite elimination. Acute hyperammonaemia needs an emergency treatment plan, not extra powder alone.