Management
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PROP Nutrition Management Guidelines
First Edition
March 2017, v.1.2
Updated: September 2017
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Nutrition Recommendations
Question
7. For the individual with PROP undergoing liver transplantation, what nutrition interventions pre, during and post transplant result in optimal outcomes?
Conclusion Statement
Derived from evidence and consensus based clinical practice

Liver transplantation is recognized as a viable treatment modality for PROP, as the liver is the major site of branched-chain amino acid (BCAA) metabolism and propionic acid (PA) production. Indications for liver transplant have included neonatal onset, frequent metabolic decompensation, uncontrolled hyperammonemia, poor growth, and sibling death, with consideration of risks and benefits of medical treatment for the patient who is a potential candidate. The metabolic team input, including contributions from the metabolic RD, is integral in the pre-transplant planning, peri-transplant period, and the post-transplant era for the individual with PROP. 

The goal for nutritional management of the individual with PROP undergoing liver transplant is to establish and maintain good metabolic control prior to, during, and after surgery. The nutrient recommendations for the pre-transplant period do not change from the individual’s usual intake.  The transplant and metabolic teams should prepare for possible surgical and other complications that may impact metabolic control.  During the surgery, continuous IV access for appropriate D10 and sodium bicarbonate infusion is recommended.  Protein intake post-transplant is less restrictive when compared to pre-transplant, with the goal of meeting the DRI for protein with intact protein, and possibly advancing protein intake while monitoring individual tolerance. Use of medical food as a protein source post-transplant was infrequent.  Continuation of carnitine supplementation was common but not universal.

Liver transplantation in PROP is only partially curative.  As there are extrahepatic sources of PA production, some individuals continue to experience periods of acidosis and/or remain at risk of central nervous system metabolic crises. However, liver transplantation appears beneficial for severely affected individuals with PROP by significantly reducing the number of episodes of decompensation. Improved metabolic control following transplant has been shown to slow and stabilize neurological decline, improve cardiac function, growth and quality of life. 

Definitions of the protein terminology used throughout this guideline are listed in Appendix B.

Recommendation 7.1

Consider liver transplantation as a potential treatment modality for individuals with PROP

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
Recommendation 7.2

Pre-transplant period

Before transplant surgery, establish and maintain good metabolic control and continue individual's appropriate and usual medical nutrition therapy.

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
Recommendation 7.3

During transplant surgery, provide continuous D10 infusion with electrolytes, with close monitoring for metabolic decompensation or other complications.

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
Recommendation 7.4

After liver transplantation surgery, provide intact protein at approximately the DRI and advance protein intake beyond the DRI as tolerated.  Continue carnitine supplementation post-transplant as a precaution against metabolic decompensation. Continue clinical and biochemical monitoring in individuals post-transplant.

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
Recommendation 7.5

Continue lifelong routine biochemical, nutritional and clinical monitoring of individuals who have undergone liver transplant, with the understanding that liver transplantation does not fully correct the metabolic abnormality of PROP (see Question 4).

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
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