Other Names for this Disease
- ARG1 deficiency
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Individuals with arginase deficiency should regularly visit a doctor who is familiar with metabolic disorders to monitor the levels of nitrogen in the blood; this is done by testing for a compound called ammonia, which is made from nitrogen. Routine treatment for this condition includes limiting the amount of protein in the diet. Special dietary formulas low in protein may be used in place of a normal diet. Several medications, such as sodium benzoate and sodium phenylbutyrate, also may be taken to remove excess ammonia from the blood. If the amount of ammonia in the blood becomes too high, individuals with arginase deficiency may need to go to the hospital for other treatments, such as intravenous (IV) fluids or, in severe cases, dialysis.
Last updated: 9/13/2012
- Cederbaum A and Crombez EA. Arginase Deficiency. GeneReviews. February 2012; http://www.ncbi.nlm.nih.gov/books/NBK1159/. Accessed 9/12/2012.
- Arginase deficiency. Genetics Home Reference. October 2006; http://ghr.nlm.nih.gov/condition/arginase-deficiency. Accessed 9/12/2012.
- Finding Treatment Information - A video developed by GARD Information Specialists that explains how you can find information about treatment for a rare disease.
- GeneReviews provides a current, expert-authored, peer-reviewed, full-text article urea cycle disorders in general that you may find helpful. GeneReview articles describe the application of genetic testing to the diagnosis, management, and genetic counseling of patients with specific inherited conditions.
- GeneReviews provides current, expert-authored, peer-reviewed, full-text articles describing the application of genetic testing to the diagnosis, management, and genetic counseling of patients with specific inherited conditions. Click on the link to view the article on this topic.
- The NORD Physician Guide for Arginase deficiency was developed as a free service of the National Organization for Rare Disorders (NORD) and it's medical advisors. The guides provide a resource for clinicians about specific rare disorders to facilitate diagnosis and treatment of their patients with this condition.
- The U.S. National Institutes of Health, through the National Library of Medicine, developed ClinicalTrials.gov to provide patients, family members, and members of the public with current information on clinical research studies. There is a study titled Study of Treatment and Metabolism in Patients With Urea Cycle Disorders which may be of interest to you. To find this trial, click on the link above.
|Generic Name||Glycerol phenylbutyrate|
(Hyperion Therapeutics, Inc.)
The FDA has approved this product to be used in this manner.
|Use as a nitrogen-binding adjunctive therapy for chronic management of adult and pediatric patients > or = to 2 years of age with urea cycle disorders (UCDs) that cannot be managed by dietary protein restriction and/or amino acid supplementation alone. RAVICTI must be used with dietary protein restriction and, in some cases, dietary supplements (eg, essential amino acids, arginine, citrulline, protein-free calorie supplements).|
|More Information about this product||Drug Information Portal|