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Title: Profile of sodium phenylbutyrate granules for the treatment of urea-cycle disorders: Patient perspectives
Authors: Peña Quintana, Luis 
Llarena, M.
De los Reyes Rosales Medina, D. 
Reyes Súarez, D. 
Aldámiz-Echevarria, L.
UNESCO Clasification: 32 Ciencias médicas
3206 Ciencias de la nutrición
Keywords: Sodium phenylbutyrate granules
Urea-cycle disorders
Treatment adherence
Quality of life
Issue Date: 2017
Journal: Patient Preference and Adherence 
Abstract: Urea-cycle disorders are a group of rare hereditary metabolic diseases characterized by deficiencies of one of the enzymes and transporters involved in the urea cycle, which is necessary for the removal of nitrogen produced from protein breakdown. These hereditary metabolic diseases are characterized by hyperammonemia and life-threatening hyperammonemic crises. Pharmacological treatment of urea-cycle disorders involves alternative nitrogen-scavenging pathways.
ISSN: 1177-889X
DOI: 10.2147/PPA.S136754
Source: Patient Preference and Adherence [ISSN 1177-889X], v. 11, p. 1489-1496
Rights: by-nc-nd
Appears in Collections:Reseña
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