Tags

Type your tag names separated by a space and hit enter

Combined nutritional support and continuous extracorporeal removal therapy in the severe acute phase of maple syrup urine disease.
Intensive Care Med. 2001 Nov; 27(11):1798-806.IC

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.

Authors+Show Affiliations

Fédération de Pédiatrie Médicale, Service de Réanimation Pédiatrique, Hôpital Necker Enfants Malades, 149 rue de Sèvres, 75743 Paris cedex 15, France. philippe.jouvet@nck.ap-hop-paris.frNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

11810125

Citation

Jouvet, P, et al. "Combined Nutritional Support and Continuous Extracorporeal Removal Therapy in the Severe Acute Phase of Maple Syrup Urine Disease." Intensive Care Medicine, vol. 27, no. 11, 2001, pp. 1798-806.
Jouvet P, Jugie M, Rabier D, et al. Combined nutritional support and continuous extracorporeal removal therapy in the severe acute phase of maple syrup urine disease. Intensive Care Med. 2001;27(11):1798-806.
Jouvet, P., Jugie, M., Rabier, D., Desgrès, J., Hubert, P., Saudubray, J. M., & Man, N. K. (2001). Combined nutritional support and continuous extracorporeal removal therapy in the severe acute phase of maple syrup urine disease. Intensive Care Medicine, 27(11), 1798-806.
Jouvet P, et al. Combined Nutritional Support and Continuous Extracorporeal Removal Therapy in the Severe Acute Phase of Maple Syrup Urine Disease. Intensive Care Med. 2001;27(11):1798-806. PubMed PMID: 11810125.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Combined nutritional support and continuous extracorporeal removal therapy in the severe acute phase of maple syrup urine disease. AU - Jouvet,P, AU - Jugie,M, AU - Rabier,D, AU - Desgrès,J, AU - Hubert,P, AU - Saudubray,J M, AU - Man,N K, Y1 - 2001/10/25/ PY - 2000/12/07/received PY - 2001/09/04/accepted PY - 2002/1/26/pubmed PY - 2002/3/29/medline PY - 2002/1/26/entrez SP - 1798 EP - 806 JF - Intensive care medicine JO - Intensive Care Med VL - 27 IS - 11 N2 - 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. SN - 0342-4642 UR - https://www.unboundmedicine.com/medline/citation/11810125/Combined_nutritional_support_and_continuous_extracorporeal_removal_therapy_in_the_severe_acute_phase_of_maple_syrup_urine_disease_ L2 - https://dx.doi.org/10.1007/s00134-001-1124-2 DB - PRIME DP - Unbound Medicine ER -