Sodium 4-Phenylbutyrate

Sodium phenylbutyric acid is metabolized in the body by beta-oxidized phenyl acetate, and the phenyl acetate is then conjugated with glutamyl aminoacylol, and the two reactions are relatively rapid and complete. Benzene acetyl glutamine ammonia as an alternative waste nitrogen urea product, renal excretion through, compared with urea at Moore level, both per mole all contains 2 Moore nitrogen (i.e., 1 mole phenyl butyric acid sodium can also remove 2 Moore source of amino acid nitrogen). According to the report, the use of sodium phenylbutyric acid in the treatment of urea circulatory disorders (the combined control of dietary nitrogen intake), the blood ammonia and haematine levels can be reduced or even normal.

The pharmacological action of sodium benzoate

Sodium phenylbutyric acid is metabolized in the body by beta-oxidized phenyl acetate, and the phenyl acetate is then conjugated with glutamyl aminoacylol, and the two reactions are relatively rapid and complete. Benzene acetyl glutamine ammonia as an alternative waste nitrogen urea product, renal excretion through, compared with urea at Moore level, both per mole all contains 2 Moore nitrogen (i.e., 1 mole phenyl butyric acid sodium can also remove 2 Moore source of amino acid nitrogen). According to the report, the use of sodium phenylbutyric acid in the treatment of urea circulatory disorders (the combined control of dietary nitrogen intake), the blood ammonia and haematine levels can be reduced or even normal.

Sodium phenylbutyric acid

Treatment for urea cycle disorders.

Sodium phenylbutyric acid

Allergic to phenylbutyric acid or phenyl acetate.

The emergency treatment is banned for acute hyperamemia.

Severe hypertension, heart failure, kidney failure.

Matters needing attention

(1) low, moderate hypertension, heart failure, insufficiency of kidney function, or other edema associated with sodium retention. (2) the liver is not fully functional. (3) obese patients.

The effects of drugs on children: it is reported that children taking phenylbutyric acid can produce unpredictable adverse reactions; Child medication, its own severe symptoms and brain damage may continue to worsen.

The effect of the drug on pregnancy: there is no evidence of pregnant women in the country, and the U.S. drug and food administration (FDA) classifications for the safety of sodium benzoate in grade C.

The effect of drugs on breastfeeding: the study of drug use by women is not sufficient and should be taken into consideration.

(1) routine monitoring of serum protein and plasma levels of ammonia, glutamate, and arginine should be monitored. (2) if necessary, the liver and kidney work should be monitored, serum albumin, total protein, arterial blood gas analysis, blood electrolyte concentration, white blood cell classification and counting, platelet count and urine analysis.

Storage: avoid light and keep at room temperature.

Sodium phenylbutyric acid

Cardiovascular system: ankle and peripheral edema, abnormal blood pressure changes, rare arrhythmias and fainting.

Nervous system: headaches and depression.

3. Metabolic/endocrine system: can cause low serum albumin leels (about 10%), metabolic acidosis (14%), (7%), high blood chlorine alkali poisoning (7%), also can cause high uric acid blood disease, hypokalemia, low phosphorus, hyperphosphatemia, high sodium (incidence of less than 6%).

Urogenital system: often causes irregular periods of menstruation of women, and even menopause (about 23%). Rare phenylbutyric acid is the cause of renal tubular acidosis.

Liver: can cause the serum aminotransferase, bilirubin, alkaline phosphatase level to rise, there is no report on the toxicity of phenylbutyric acid.

6. The gastrointestinal tract: common lack of appetite (about 4%), also can cause poor taste, abdominal pain, nausea, vomiting, gastritis, unusual sodium phenyl butyric acid peptic ulcer, rectal bleeding, constipation, pancreatitis.

Blood: may cause anemia (including aplastic anemia), leukocyte decrease or increase, platelets decrease, rare phenylbutyric acid can cause platelets to increase.

Skin: even rash.

Other: after sodium benzoate is taken, it may cause a bad smell.

Sodium phenylbutyric acid

Oral medicine: usually 4 to 6 times a day, the daily dosage of medicine is less than 20g. The safety and effectiveness of more than 20 grams of drugs per day has not been determined. Children's normal dosage: oral medicine: body weight is less than 20kg children, 450 ~ 600mg/kg a day, 4 ~ 6 times to the medicine, taking the medicine at mealtime, the total amount of medicine is less than 20g per day. It is recommended to use sodium phenylbutyric acid, not recommended for sodium benzoate. Weigh more than 20 kg and use the same amount as adults. Dosage of foreign usage: oral medicine: 9.9 ~ 13g/m2, the safety and effectiveness of more than 20g a day are not determined. Experiments show that liver function is full-time dose: have not been urea cycle disorder of hepatic insufficiency, phenyl butyric acid metabolism and excretion of sodium is not affected, there is no need to adjust the dose of data suggest that hepatic insufficiency, but needs further research. Children's regular doses: oral medicine: less than 20kg of children, 450 to 600 mg/kg a day; Weight is greater than 20k, use the same as adults.

Drug interaction

The combination of haloperidol, valproate, corticosteroid, and corticosteroids, may lead to high amenemia and should be used with caution.

The renal excretion of the metabolite of ben-butyric acid is affected by the use of acetsu-sulfonate, which should be used with caution.