Effects of L-arginine suplementation in individuals with Sickle Cell Disease

Abstract

Sickle cell disease is a group of hereditary disorders resulting from a monogenic mutation in the β-globin chain of hemoglobin. Currently, the proposed non- pharmacological treatment consists of amino acid supplementation, administered orally or intravenously. Objective: To evaluate the effects of arginine supplementation in individuals with sickle cell disease across different age groups. Methodology: A narrative review was conducted with a literature search in the PubMed, Scopus, Scielo, and Lilacs databases, covering publications from the last 11 years. In vitro studies, animal studies, undergraduate theses, editorials, dissertations, theses, and reviews were excluded. Results: A total of 528 publications were identified, of which only 10 met the inclusion criteria after analysis. Different treatment protocols were identified, involving the exclusive use of L-arginine or its combination with hydroxyurea. The first protocol was applied to hospitalized children and adolescents for 5 to 7 days, with oral and/or intravenous administration. The second protocol was used for 6 weeks to 4 months in adults and children with stable clinical conditions, administered orally. The effects of the first protocol included a 54% reduction in pain levels and opioid use, along with improvements in mitochondrial function and cardiopulmonary performance. The second protocol resulted in a reduction in oxidative stress, improved liver function, fasting blood glucose levels, and decreased inflammation. These effects were observed with both routes of administration. Conclusion: L-arginine supplementation, whether oral or intravenous, demonstrated potential as an adjuvant therapy for sickle cell disease. However, further studies with larger patient cohorts and longer treatment durations are necessary to better understand the effects, safety, and efficacy of L-arginine supplementation.