Management
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MSUD Nutrition Management Guidelines
First Edition
February 2013, v.1.46
Current version: v.2.0
Updated: May 2014
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Nutrition Recommendations
Question
5. For the individual with MSUD undergoing liver transplantation, what specific nutritional interventions result in optimal nutritional, medical and quality of life outcomes?
Conclusion Statement
Derived from evidence and consensus based clinical practice

For patients with MSUD to be candidates for liver transplantation, they need to be in good metabolic control, through dietary management of their branched chain amino acids (BCAA). In the perioperative period, continuous glucose infusion is necessary to prevent catabolism, sodium/water homeostasis maintained to avoid brain edema, and plasma amino acid analyses availability to monitor the BCAA levels. Studies report that after transplantation, these patients can consume a diet with no BCAA restrictions and no longer experience metabolic decompensation. There are no published studies reporting nutritional counseling during the transition to an unrestricted diet, nor the monitoring of growth and nutritional status to ensure that their dietary intake is appropriate.

Recommendation 5.1

Consider liver transplantation as a viable treatment option for individuals with MSUD.

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
Recommendation 5.2

Attempt to bring candidates for liver transplant into good metabolic control (prior to surgery) through dietary management of their BCAA.

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
Recommendation 5.3

Prevent metabolic decompensation In the perioperative period.

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
Recommendation 5.4

Allow a relaxation of the BCAA-restricted diet and lift precautions for severe metabolic decompensation for individuals with MSUD who have had successful liver transplantation.

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
Recommendation 5.5

Provide nutritional counseling to assist in dietary transition, and monitor the anthropometric and nutritional status of individuals with MSUD who have had successful liver transplantation.

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
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