Original Title: Utilidad de los tratamientos dietéticos en los trastornos del metabolismo de la Lisina

Authors' objectives: To determine the safety and effectiveness of using Lysine-free formulae in Hyperlysinemia, the use of L-citrulline and l-lysine modules in the hereditary intolerance to Lysine due to the dibasic amino acid transporter disorder and of the use of L-citrulline modules in the case of Hiperammoniemia, in order to establish recommendations regarding their permanence, exclusion or inclusion in the portfolio of health services.

Authors' results and conclusions: The selected studies provide very low quality evidence to demonstrate the effectiveness and safety of interventions by means of diets and supplements in Lysine metabolic disorders. Contextualization / Discussion As it is a rare, low prevalence and serious disease, it would be appropriate to take into consideration the information deriving from studies of series of cases and single cases, as well as reviews based on the experience and knowledge of the authors, in order to decide whether to include these or not in the health services portfolio. More research effort is required into metabolic diseases and to increase the methodological quality of experimental studies. Conclusions It is not possible to make an unreserved recommendation about the need to maintain or remove these products from the health services portfolio when these are prescribed for that group of patients. From the clinical viewpoint, it will be possible to take decisions about the type of diets and supplements, adapted in each case to the individual conditions of patients.

Authors' methods: Systematic review of the scientific literature: Definition of clinical questions in PICO format, design of the search strategy, selection criteria of studies in accordance with the components in the PICO questions, evaluation of their quality and the synthesis of the evidence in tables.

Project Status: Completed

Year Published: 2013

URL for published report: https://www.euskadi.eus/contenidos/informacion/2013_osteba_publicacion/es_def/adjuntos/tratmetab_lisina.pdf

English language abstract: An English language summary is available

Publication Type: Rapid Review

Country: Spain

Organisation Name: Basque Office for Health Technology Assessment

Contact Address: C/ Donostia – San Sebastián, 1 (Edificio Lakua II, 4ª planta) 01010 Vitoria - Gasteiz

Contact Name: Lorea Galnares-Cordero

Contact Email: lgalnares@bioef.eus

Copyright: <p>Osteba (Basque Office for Health Technology Assessment) Health Department of the Basque Government</p>