title: Effect of docosahexaenoic acid administration on plasma lipid profile and metabolic parameters of children with methylmalonic acidaemia.
abstract: OBJECTIVE
  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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Departamento de Pediatría, Hospital de Cruces, Plaza de Cruces s/n, Baracaldo, 48903 Vizcaya, Spain. kaldamiz@hcru.osakidetza.net
authors.forename: L
authors.initials: L
authors.lastname: Aldámiz-Echevarría
authors.forename: P
authors.initials: P
authors.lastname: Sanjurjo
authors.forename: J
authors.initials: J
authors.lastname: Elorz
authors.forename: J A
authors.initials: JA
authors.lastname: Prieto
authors.forename: C
authors.initials: C
authors.lastname: Pérez
authors.forename: F
authors.initials: F
authors.lastname: Andrade
authors.forename: J
authors.initials: J
authors.lastname: Rodríguez-Soriano
mesh_terms: D000293:Adolescent; D016718:Arachidonic Acid / Q000097:blood; D016022:Case-Control Studies; D002648:Child; D018592:Cross-Over Studies; D004281:Docosahexaenoic Acids / Q000494:pharmacology*; D005260:Female; D006801:Humans; D008055:Lipids / Q000097:blood*; D008297:Male; D008661:Metabolism, Inborn Errors / Q000097:blood* / Q000188:drug therapy*; D008764:Methylmalonic Acid / Q000652:urine*; D019301:Oleic Acid / Q000097:blood; D017962:alpha-Linolenic Acid / Q000097:blood
chemical_list: D008055:Lipids; D017962:alpha-Linolenic Acid; D004281:Docosahexaenoic Acids; D016718:Arachidonic Acid; D019301:Oleic Acid; D008764:Methylmalonic Acid