Original Article

Journal of Inherited Metabolic Disease

, Volume 29, Issue 1, pp 58-63

Effect of docosahexaenoic acid administration on plasma lipid profile and metabolic parameters of children with methylmalonic acidaemia

  • L. Aldámiz-EchevarríaAffiliated withDepartamento de Pediatría, Hospital de CrucesDivision of Metabolism, Department of Pediatrics, Hospital de Cruces and Basque University School of Medicine Email author 
  • , P. SanjurjoAffiliated withDivision of Metabolism, Department of Pediatrics, Hospital de Cruces and Basque University School of Medicine
  • , J. ElorzAffiliated withDivision of Metabolism, Department of Pediatrics, Hospital de Cruces and Basque University School of Medicine
  • , J. A. PrietoAffiliated withDivision of Metabolism, Department of Pediatrics, Hospital de Cruces and Basque University School of Medicine
  • , C. PérezAffiliated withDivision of Metabolism, Department of Pediatrics, Hospital de Cruces and Basque University School of Medicine
  • , F. AndradeAffiliated withDivision of Metabolism, Department of Pediatrics, Hospital de Cruces and Basque University School of Medicine
  • , J. Rodríguez-SorianoAffiliated withDivision of Metabolism, Department of Pediatrics, Hospital de Cruces and Basque University School of Medicine

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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.