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http://hdl.handle.net/10553/24128
| 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. | URI: | http://hdl.handle.net/10553/24128 | 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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