Treatment of acute decompensation of maple syrup urine disease in adult patients with a new parenteral amino-acid mixture.
J Inherit Metab Dis
; 36(6): 939-44, 2013 Nov.
Article
en En
| MEDLINE
| ID: mdl-23250513
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.CONCLUSION:
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.
Texto completo:
1
Bases de datos:
MEDLINE
Asunto principal:
Nutrición Parenteral
/
Aminoácidos
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Insuficiencia Cardíaca
/
Enfermedad de la Orina de Jarabe de Arce
Tipo de estudio:
Etiology_studies
Límite:
Adult
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Female
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Humans
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Male
Idioma:
En
Revista:
J Inherit Metab Dis
Año:
2013
Tipo del documento:
Article