Management
Guidelines
Portal
DNDF Model
MSUD Nutrition Management Guidelines
First Edition
February 2013, v.1.54
Current version: v.2.0
Updated: April 2018
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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.

Summary
 
Details
Recommendation 4.1

Provide individualized pregnancy planning information to all women with MSUD of child-bearing age and update when pregnancy is confirmed.

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

Adjust dietary intake to allow blood LEU concentrations to be in the 100-300µM range and VAL and ILE in the upper range of normal (200-400µM) through out pregnancy

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

4.3: Provide individualized dietary guidance to assure nutrient intake meets the changing needs of each phase of pregnancy and the postpartum period.

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

Provide nutrient, clinical and biochemical monitoring during all phases of pregnancy and the postpartum period.

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

Prevent catabolism during particularly vulnerable periods including: pregnancy-related nausea and emesis, intercurrent illness, labor and delivery, and post-partum recovery.

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

For women with MSUD who wish to breast feed, closely monitor her nutrient intake, clinical and  biochemical markers and her infant’s growth and development.

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
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