Treatment
Support while transport to a tertiary
center is arranged
Several actions, including the insertion of deep vein catheters and intubation,
prior to transportation improve the prognosis by aiding the administration of
fluids and medications and preventing the development of hypoxia.
Treatment of Acute Hyperammonemia
Acute hyperammonemia is a life threatening event and requires prompt treatment.
Ammonul® (sodium benzoate/sodium phenylacetate)
which is an intravenous solution is FDA approved as adjunctive therapy for the
treatment of acute hyperammonemia in patients with uera cycle disorders.
(http://www.Ammonul.com)
Removal of ammonia by dialysis
Hemodialysis is the only way to remove ammonia from the bloodstream rapidly.
Peritoneal dialysis is not sufficient in this acute phase of the disorder.
Maintenance of ammonia removal by
using drugs
The buildup of ammonia can be prevented by the use of ammonia-scavenging drugs
such as Buphenyl®. More information on this
medication can be found by clicking (here).
Reversing the net breakdown of protein (catabolism)
Reducing nitrogen turnover reduces the amount of ammonia that is produced. The
use of a combination of intravenous glucose and Intralipid® to
maximize parenteral calories is often require as these patients are frequently
undernourished on admission.
Drug Interactions and Dosage
Buphenyl is an oral medication and should be taken by mouth,
.
| Drug Interactions |
|---|
| Probenecid is known to inhibit the renal transport of many organic compounds, including hippuric acid, and may affect renal excretion of the conjugation product of buphenyl as well as it's metabolite. |
| Use of intravenous corticosteroids may cause the breakdown of body protein and increase plasma ammonia. |
Dosing Table |
|
| Patient Weight | Dose |
< 20 kgs |
450-600mg/kg/day |
>20kgs |
9-13grams/M2/day |
Medication should be taken in equally divided doses with meals three to six times per day.
|
|
Buphenyl powder is recommended for patients weighing <20kgs. Buphenyl powder is indicated for oral use (via mouth, gastrostomy, or nasogastric tube only).
|
|
The powder is to be mixed with food (solid or liquid). Sodium phenylbutyrate is very soluble in water (5 grams per 10 mL). When Buphenyl Powder is added to a liquid, only sodium phenylbutyrate will dissolve, the excipients will not. The effect of food on sodium phenylbutyrate has not been determined.
|
|
Each level teaspoon (enclosed) dispenses 3.2 grams of powder and 3.0 grams of sodium phenylbutyrate.
|
|
Each level tablespoon (enclosed) dispenses 9.1 grams of powder and 8.6 grams of sodium phenylbutyrate.
|
|
Shake lightly before use.
|
|
The safety or efficacy of doses in excess of 20 grams (40 tablets) per day has not been established.
|
|
Full Prescribing Information PDF