Management
Guidelines
Portal
MSUD Nutrition Management Guidelines
First Edition
February 2013, v.1.50
Current version: v.1.58
Updated: August 2016
Feedback/Comments :: View Release Notes
Send us your Feedback & Comments
This version is not current. Click to view the most recent edition (v.1.58, August 2016).
Nutrition Recommendations
Question
4. For the woman with MSUD, what specific nutrition interventions must be initiated during pregnancy, at delivery and during the postpartum period to achieve optimal outcomes for her and her newborn infant?
Conclusion Statement
Derived from evidence and consensus based clinical practice

The woman with MSUD who is pregnant needs increased protein intake to support the proliferation of maternal tissues and growth of the fetus, while keeping the plasma BCAA within the treatment range to maintain metabolic control. Energy intake must also support increased needs associated with pregnancy. Supplemental vitamins and minerals may be needed for those nutrients not adequate in the medical food consumed. Assessment of plasma carnitine levels is recommended with provision of supplemental carnitine if the free carnitine falls below normal levels. Catabolism should be prevented or minimized in all stages of pregnancy and the postpartum period. Tube- or parenteral feeding may be needed, if oral intake is not adequate. Nutritional counseling will be needed for assisting the pregnant woman to achieve adequate intake during periods of nausea or decreased appetite.

Recommendation 4.1

Monitor closely and provide individualized dietary guidance to assure that intake is adequate to meet the increased protein, BCAA and energy requirements of pregnancy. See TABLE #5, Protein Recommendations during Pregnancy for MSUD, and TABLE #7, Recommendations for the Nutritional Monitoring of Individuals with MSUD.

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
Recommendation 4.2

Maintain plasma BCAA levels in the normal treatment range (LEU:75-300 µmol/L; ILE and VAL: 200-400 µmol/L) throughout pregnancy.

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
Recommendation 4.3

Treat pregnancy-related poor appetite, nausea and vomiting aggressively to prevent or minimize endogenous protein catabolism. See treatment strategies to prevent endogenous catabolism in Research Question 1.

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
Recommendation 4.4

Evaluate the need for vitamin and/or mineral supplementation based on the pregnant women's prescribed medical food, dietary adherence and laboratory assessment. See TABLE #7, Recommendations for the Nutritional Monitoring of Individuals with MSUD, and resources for dietary analysis and medical foods’ composition at: http://gmdi.org/index.php?option=com_content&view=article&id=110&Itemid=58

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
Recommendation 4.5

Pay special attention to the prevention of catabolism during the particularily vulnerable periods of labor/delivery and the first two weeks postpartum, as well as up to six weeks postpartum. See TABLE #5, Protein Recommendations during Pregnancy for MSUD, TABLE #7, Recommendations for the Nutritional Monitoring of Individuals with MSUD, and treatment strategies to prevent endogenous catabolism in Research Question 1.

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
Recommendation 4.6

Provide increased nutrient intake and close clinical and biochemical monitoring for the woman with MSUD who is breastfeeding. See TABLE #5, Protein Recommendations during Pregnancy for MSUD and TABLE #7, Recommendations for the Nutritional Monitoring of Individuals with MSUD.

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
© 2006-2015   —   SOUTHEAST REGIONAL GENETICS NETWORK   —   A HRSA SUPPORTED PARTNERSHIP   —   GRANT #UH7MC30772