Combined nutritional support and continuous extracorporeal removal therapy in the severe acute phase of maple syrup urine disease.
Intensive Care Med
; 27(11): 1798-806, 2001 Nov.
Article
em En
| MEDLINE
| ID: mdl-11810125
ABSTRACT
OBJECTIVE:
The authors assessed the efficiency, tolerance and outcome of neonates and children with maple syrup urine disease (MSUD) in acute decompensation managed by endogenous and extracorporeal removal of accumulated MSUD metabolites.DESIGN:
Single center cohort study.SETTING:
Pediatric and neonatal intensive care unit in a tertiary care hospital. PATIENTS Between January, 1991, and June, 1999, six neonates and six children in acute decompensation of MSUD were included in the study. Each of them had two of the three following criteria comatose state, gastrointestinal intolerance, leucine plasma levels over 1700 micromol/l.INTERVENTIONS:
Patients were treated by combined nutrition manipulation and continuous venovenous extracorporeal removal therapies (CECRT) including hemofiltration, hemodialysis or hemodiafiltration. A clinical and biological evaluation was performed before, during and following the treatment.RESULTS:
Eleven out of the 12 patients survived. One child had two acute episodes at 6.5 and 9 years old. Eight patients recovered a normal cerebral performance category score. In all cases, plasma leucine level decreased according to a logarithmic mode within 11-24 h hemodiafiltration combined with nutritional support whereas, with nutrition alone after stopping CECRT, the decrease in leucine plasma levels was slower, following a linear mode. Eight patients were supplemented with valine and isoleucine for mean plasma values of 177+/-92 and 68+/-66, respectively.CONCLUSION:
In severe acute decompensation of MSUD, CECRT combined with nutritional support limit central nervous system damage, by dramatically decreasing branched chain amino and keto acid levels.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Hemofiltração
/
Nutrição Enteral
/
Doença da Urina de Xarope de Bordo
Tipo de estudo:
Etiology_studies
/
Incidence_studies
/
Observational_studies
/
Risk_factors_studies
Limite:
Adolescent
/
Child
/
Child, preschool
/
Female
/
Humans
/
Male
/
Newborn
Idioma:
En
Revista:
Intensive Care Med
Ano de publicação:
2001
Tipo de documento:
Article