title: Treatment of acute decompensation of maple syrup urine disease in adult patients with a new parenteral amino-acid mixture.
abstract: BACKGROUND
  Acute decompensation of maple syrup urine disease (MSUD) is usually treated by enteral feeding with an amino-acid mixture without leucine (Leu), valine or isoleucine. However, its administration is ineffective in cases of gastric intolerance and some adult patients refuse enteral feeding via a nasogastric tube. We developed a new parenteral amino-acid mixture for patients with MSUD.
  
  
  METHODS
  Seventeen decompensation episodes in four adult patients with MSUD treated with a parenteral amino-acid mixture (group P) were compared to 18 previous episodes in the same patients treated by enteral feeding (group E).
  
  
  RESULTS
  The mean Leu concentration at presentation was similar in the groups P and E (1196.9 μmol/L and 1212.2 μmol/L, respectively). The mean decrease in the Leu concentration during the first 3 days of hospitalisation was significantly higher in group P than group E (p = 0.0026); there were no side effects. The mean duration of hospitalisation was similar (4 vs. 4.5 days, p = NS). No patient in group P deteriorated whereas one patient in group E required dialysis.
  
  
  CONCLUSIONS
  This new parenteral amino-acid mixture is safe and allows efficient Leu concentration decrease during acute MSUD decompensation episodes in adults. Its use avoids the need for nasogastric tube insertion.
authors.affiliation: Department of Nephrology, Hôpital Necker-Enfants Malades AP-HP, Paris, France, aude.servais@nck.aphp.fr.
authors.forename: A
authors.initials: A
authors.lastname: Servais
authors.forename: J B
authors.initials: JB
authors.lastname: Arnoux
authors.forename: C
authors.initials: C
authors.lastname: Lamy
authors.forename: A
authors.initials: A
authors.lastname: Hummel
authors.forename: N
authors.initials: N
authors.lastname: Vittoz
authors.forename: I
authors.initials: I
authors.lastname: Katerinis
authors.forename: V
authors.initials: V
authors.lastname: Bazzaoui
authors.forename: S
authors.initials: S
authors.lastname: Dubois
authors.forename: C
authors.initials: C
authors.lastname: Broissand
authors.forename: M C
authors.initials: MC
authors.lastname: Husson
authors.forename: M P
authors.initials: MP
authors.lastname: Berleur
authors.forename: D
authors.initials: D
authors.lastname: Rabier
authors.forename: C
authors.initials: C
authors.lastname: Ottolenghi
authors.forename: V
authors.initials: V
authors.lastname: Valayannopoulos
authors.forename: P
authors.initials: P
authors.lastname: de Lonlay
mesh_terms: D000328:Adult; D000596:Amino Acids / Q000008:administration & dosage*; D005260:Female; D005526:Food, Formulated; D006333:Heart Failure / Q000178:diet therapy* / Q000209:etiology; D006760:Hospitalization; D006801:Humans; D008297:Male; D008375:Maple Syrup Urine Disease / Q000150:complications / Q000178:diet therapy*; D010288:Parenteral Nutrition; D010342:Patient Acceptance of Health Care; D055815:Young Adult
chemical_list: D000596:Amino Acids