caa a22 4500 467932522 CHVBK 20180406152947.0 cr unu---uuuuu 170328e20060201xx s 000 0 eng 10.1007/s10545-006-0182-6 doi (NATIONALLICENCE)springer-10.1007/s10545-006-0182-6 Effect of docosahexaenoic acid administration on plasma lipid profile and metabolic parameters of children with methylmalonic acidaemia [Elektronische Daten] [L. Aldámiz-Echevarría, P. Sanjurjo, J. Elorz, J. Prieto, C. Pérez, F. Andrade, J. Rodríguez-Soriano] Summary: Aim. To evaluate the effect of administration of docosahexaenoic acid (DHA) on dyslipidaemia, plasma fatty acid composition and metabolic parameters of children with isolated methylmalonic acidaemia (MMA) (McKusick 25100). Methods. Four children (3 male, 1 female) with MMA (mut(0)), participated in a crossover, randomized study of DHA administration (25 mg/kg per day, divided into three daily doses). The control group comprised 56 healthy children, aged 10± 2.7 years, (51 male, 5 female), who were followed in our clinic owing to possible familial risk of cardiovascular disease. Results. The comparison of plasma fatty acid composition of children with MMA versus control children demonstrated that the patients had significantly higher values for oleic acid (p = 0.004) and linolenic acid (p = 0.008). No differences were observed in the levels of DHA and arachidonic acid. Plasma concentrations of insulin, glycine, ammonia, total cholesterol and cholesterol fractions did not change with DHA administration. No significant changes were observed in urinary excretion of methylmalonic acid. As expected, the percentage of DHA and n−3 fatty acids in plasma increased significantly after therapy (p = 0.005 and 0.014, respectively). The most remarkable result was a decrease of plasma levels of triglycerides after DHA therapy (p = 0.014). Conclusion. As previously found in normal children, dietary supplementation with DHA decreases the triglyceride levels, normalizing the hypertriglyceridaemia of these children without any evidence of short-term adverse effects. SSIEM and Springer, 2006 Aldámiz-Echevarría L. Departamento de Pediatría, Hospital de Cruces, Plaza de Cruces s/n, 48903, Baracaldo, Vizcaya, Spain aut Sanjurjo P. Division of Metabolism, Department of Pediatrics, Hospital de Cruces and Basque University School of Medicine, Bilbao, Spain aut Elorz J. Division of Metabolism, Department of Pediatrics, Hospital de Cruces and Basque University School of Medicine, Bilbao, Spain aut Prieto J. Division of Metabolism, Department of Pediatrics, Hospital de Cruces and Basque University School of Medicine, Bilbao, Spain aut Pérez C. Division of Metabolism, Department of Pediatrics, Hospital de Cruces and Basque University School of Medicine, Bilbao, Spain aut Andrade F. Division of Metabolism, Department of Pediatrics, Hospital de Cruces and Basque University School of Medicine, Bilbao, Spain aut Rodríguez-Soriano J. Division of Metabolism, Department of Pediatrics, Hospital de Cruces and Basque University School of Medicine, Bilbao, Spain aut Journal of Inherited Metabolic Disease Kluwer Academic Publishers 29/1(2006-02-01), 58-63 0141-8955 29:1<58 2006 29 10545 https://doi.org/10.1007/s10545-006-0182-6 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s10545-006-0182-6 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Aldámiz-Echevarría L. Departamento de Pediatría, Hospital de Cruces, Plaza de Cruces s/n, 48903, Baracaldo, Vizcaya, Spain aut NATIONALLICENCE 700 1- Sanjurjo P. Division of Metabolism, Department of Pediatrics, Hospital de Cruces and Basque University School of Medicine, Bilbao, Spain aut NATIONALLICENCE 700 1- Elorz J. Division of Metabolism, Department of Pediatrics, Hospital de Cruces and Basque University School of Medicine, Bilbao, Spain aut NATIONALLICENCE 700 1- Prieto J. Division of Metabolism, Department of Pediatrics, Hospital de Cruces and Basque University School of Medicine, Bilbao, Spain aut NATIONALLICENCE 700 1- Pérez C. Division of Metabolism, Department of Pediatrics, Hospital de Cruces and Basque University School of Medicine, Bilbao, Spain aut NATIONALLICENCE 700 1- Andrade F. Division of Metabolism, Department of Pediatrics, Hospital de Cruces and Basque University School of Medicine, Bilbao, Spain aut NATIONALLICENCE 700 1- Rodríguez-Soriano J. Division of Metabolism, Department of Pediatrics, Hospital de Cruces and Basque University School of Medicine, Bilbao, Spain aut NATIONALLICENCE 773 0- Journal of Inherited Metabolic Disease Kluwer Academic Publishers 29/1(2006-02-01), 58-63 0141-8955 29:1<58 2006 29 10545 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer