BUPHENYL POWDER Rx
Generic Name and Formulations:
Sodium phenylbutyrate 940mg/gram; contains sodium 11.7g/100g of pwd.
Indications for BUPHENYL POWDER:
Adjunct in the chronic management 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.
Adults and Children:
<20kg: use powder. Take in equally divided doses with each meal or feeding. Mix powder with food or liquid. <20kg: 450–600mg/kg/day. ≥20kg: 9.9–13g/m2/day. Max 20g/day.
Restrict dietary protein, may need to supplement with essential amino acids. CHF. Sodium retention with edema. Renal or hepatic insufficiency. Inborn errors of beta oxidation. Monitor serum levels of phenylbutyrate and metabolites, phenylacetate and phenylacetylglutamine. Plasma levels of ammonia, arginine, branched-chain amino acids, serum proteins: maintain within normal levels; plasma glutamine: maintain at <1000μmol/L. Elderly. Pregnancy (Cat.C). Nursing mothers.
May be potentiated by probenecid. Corticosteroids may increase plasma ammonia levels. Hyperammonemia possible with haloperidol and valproic acid.
Amenorrhea/menstrual dysfunction, acidosis, hypoalbuminemia, anemia, alkalosis, hyperchloremia, hypophosphatemia, increased alkaline phosphatase, increased liver transaminases, leukopenia, leukocytosis, decreased appetite, body odor, bad taste, taste aversion, others.
Psychiatry Advisor Articles
- ACOG Update: Marijuana Use Discouraged During Pregnancy, Breastfeeding
- Continuing Research, Emerging Treatments Hold Promise for Treating Anorexia Nervosa
- Asenapine Prevents Recurrence of Mood Events in Bipolar Disorder
- Depression Profiles in Patients With Type 1 Diabetes vs Type 2 Diabetes
- Purpose, Questions of Social Interaction Lead Physicians to Delay Retirement
- CBT, Acceptance Commitment Therapy Helpful for Those With Chronic Pain
- Suicidal Behavior, Thoughts Associated With Perfectionist Tendencies
- Depression Reduced by Social Belonging, Feelings of Inclusion
- Sleep Disturbance May Be Causal Factor in Psychotic Experiences
- Is Antidepressant Use in Pregnancy Tied to Psychiatric Disorders in Offspring?
- Cardiovascular Risk Management May Slow Neurocognitive Decline in HIV
- Maintenance rTMS for Treatment-Resistant Depression
- New Study Compares Opioid Dependence Relapse Treatments
- Increase Use of Nursing Home for Patients With Cognitive Impairment Category
- Venlafaxine XR Safe, Effective in Treating Generalized Anxiety Disorder