title: Normal serum alanine concentration differentiates transient neonatal lactic acidemia from an inborn error of energy metabolism.
abstract: BACKGROUND
  Elevated blood lactate levels are common in the critically ill neonate; however, sometimes they are difficult to interpret. Persistent or recurrent lactic acidemia might point to an inborn error of metabolism, like disturbances of the oxidative phosphorylation. Chronic lactic acidemia results in increased serum alanine levels. Serum alanine levels in newborns with transient lactic acidemia have not yet been studied.
  
  
  OBJECTIVE
  We designed a pilot study to evaluate the use of serum alanine levels as an additional metabolic marker to differentiate the transient effect of circulatory failure from a possible mitochondrial dysfunction.
  
  
  METHODS
  We prospectively evaluated 10 newborns with transient lactic acidemia after mild dysoxia, and 10 newborns with recurrent lactic acidemia consecutively diagnosed with a disorder in oxidative phosphorylation.
  
  
  RESULTS
  No significant serum alanine level elevation was found in transient lactic acidemia. Increased serum alanine was a sensitive marker in mitochondrial dysfunction.
  
  
  CONCLUSIONS
  We propose to measure the serum alanine level in hypotonic newborns with lactic acidemia to facilitate the decision making in further diagnostics and management.
authors.affiliation: Department of Pediatrics, Nijmegen Centre for Mitochondrial Disorders, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands. E.Morava@cukz.umcn.nl
authors.forename: Eva
authors.initials: E
authors.lastname: Morava
authors.forename: Marije
authors.initials: M
authors.lastname: Hogeveen
authors.forename: Maaike
authors.initials: M
authors.lastname: De Vries
authors.forename: Wim
authors.initials: W
authors.lastname: Ruitenbeek
authors.forename: Willem-Pieter
authors.initials: WP
authors.lastname: de Boode
authors.forename: Jan
authors.initials: J
authors.lastname: Smeitink
mesh_terms: D000409:Alanine / Q000097:blood*; D004272:DNA, Mitochondrial / Q000235:genetics; D003937:Diagnosis, Differential; D004734:Energy Metabolism; D006801:Humans; D007231:Infant, Newborn; D019344:Lactic Acid / Q000097:blood*; D008661:Metabolism, Inborn Errors / Q000097:blood / Q000175:diagnosis*; D028361:Mitochondrial Diseases / Q000097:blood / Q000235:genetics; D009154:Mutation; D010865:Pilot Projects
chemical_list: D004272:DNA, Mitochondrial; D019344:Lactic Acid; D000409:Alanine