EXAMINING
SPECIAL
NUTRITIONAL
REQUIREMENTS
IN DISEASE STATES
PROCEEDINGS OF A WORKSHOP
Anne Brown Rodgers, Rapporteur
Food and Nutrition Board
Health and Medicine Division
THE NATIONAL ACADEMIES PRESS
Washington, DC
www.nap.edu
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This activity was supported by contracts between the National Academy of Sciences and the Academy of Nutrition and Dietetics; American Society for Nutrition; Crohn’s & Colitis Foundation; Health Canada; National Institutes of Health; and U.S. Food and Drug Administration. Any opinions, findings, conclusions, or recommendations expressed in this publication do not necessarily reflect the views of any organization or agency that provided support for the project.
International Standard Book Number-13: 978-0-309-47837-3
International Standard Book Number-10: 0-309-47837-5
Digital Object Identifier: https://doi.org/10.17226/25164
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Copyright 2018 by the National Academy of Sciences. All rights reserved.
Printed in the United States of America
Suggested citation: National Academies of Sciences, Engineering, and Medicine. 2018. Examining special nutritional requirements in disease states: Proceedings of a workshop. Washington, DC: The National Academies Press. doi: https://doi.org/10.17226/25164.
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PLANNING COMMITTEE ON EXAMINING SPECIAL NUTRITIONAL REQUIREMENTS FOR DISEASE STATES—A WORKSHOP1
BARBARA O. SCHNEEMAN (Chair), Professor Emerita, University of California, Davis
PATSY M. BRANNON, Professor, Division of Nutritional Sciences, Cornell University, Ithaca, NY
STEVEN K. CLINTON, Professor, Department of Internal Medicine, Division of Medical Oncology, The Ohio State University, Columbus
ALEX R. KEMPER, Chief of the Division of Ambulatory Pediatrics, Nationwide Children’s Hospital, Columbus, OH
ERIN MACLEOD, Director of Metabolic Nutrition, Division of Genetics and Metabolism, Children’s National Health System, Washington, DC
BERNADETTE P. MARRIOTT, Professor, Division of Gastroenterology and Hepatology, Department of Medicine, and Professor, Military Division, Department of Psychiatry, Medical University of South Carolina, Charleston
PATRICK J. STOVER, Vice Chancellor and Dean for Agriculture and Life Sciences at Texas A&M AgriLife, College Station
DAVID L. SUSKIND, Professor of Pediatrics, Division of Gastroenterology, Seattle Children’s Hospital, Washington
GARY D. WU, Ferdinand G. Weisbrod Professor in Gastroenterology, Perelman School of Medicine, University of Pennsylvania, Philadelphia
Health and Medicine Division Staff
MARIA ORIA, Study Director
ALICE VOROSMARTI, Research Associate
CYPRESS LYNX, Senior Program Assistant
ANN L. YAKTINE, Director, Food and Nutrition Board
___________________
1 The National Academies of Sciences, Engineering, and Medicine’s planning committees are solely responsible for organizing the workshop, identifying topics, and choosing speakers. The responsibility for the published Proceedings of a Workshop rests with the workshop rapporteur and the National Academies.
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Reviewers
This Proceedings of a Workshop was reviewed in draft form by individuals chosen for their diverse perspectives and technical expertise. The purpose of this independent review is to provide candid and critical comments that will assist the National Academies of Sciences, Engineering, and Medicine in making each published proceedings as sound as possible and to ensure that it meets the institutional standards for quality, objectivity, evidence, and responsiveness to the charge. The review comments and draft manuscript remain confidential to protect the integrity of the process.
We thank the following individuals for their review of this proceedings:
JOHN W. ERDMAN, Emeritus, University of Illinois at Urbana-Champaign
DENISE M. NEY, University of Wisconsin–Madison
ROBERT M. RUSSELL, Emeritus, Tufts University School of Medicine
DAVID L. SUSKIND, Seattle Children’s Hospital
Although the reviewers listed above provided many constructive comments and suggestions, they were not asked to endorse the content of the proceedings nor did they see the final draft before its release. The review of this proceedings was overseen by A. CATHERINE ROSS, The Pennsylvania State University. She was responsible for making certain that an independent examination of this proceedings was carried out in accordance with standards of the National Academies and that all review comments were carefully considered. Responsibility for the final content rests entirely with the rapporteur and the National Academies.
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Contents
1 INTRODUCTION AND WORKSHOP OVERVIEW
Organization of This Proceedings of a Workshop
What Defines a Special Nutritional Requirement?
The Underlying Biological Processes of Special Nutritional Requirements
Moderated Panel Discussion and Q&A
2 ADDRESSING NUTRIENT NEEDS DUE TO LOSS OF FUNCTION IN GENETIC DISEASES
Understanding the Basis of Nutritional Needs in Phenylketonuria
Nutritional Inadequacies in Mitochondrial-Associated Metabolic Disorders
Contribution of Nutrients in Complex Inborn Errors of Metabolism: The Case of Methylmalonic Acidemia
Lessons Learned: What Is Known About Nutrition Management for Inborn Errors of Metabolism
3 DISEASE-INDUCED LOSS OF FUNCTION AND TISSUE REGENERATION
Examples of Gastrointestinal Dysfunction and Malabsorption of Nutrients: Intestinal Failure
Examples of Gastrointestinal Dysfunction and Malabsorption of Nutrients: Cystic Fibrosis
Nutritional Requirements for Inflammatory Bowel Disease
Blood–Brain Barrier Dysfunction and Resulting Brain Nutrient Deficiencies
Macro- and Micronutrient Homeostasis in the Setting of Chronic Kidney Disease
Moderated Panel Discussion and Q&A
4 DISEASE-INDUCED DEFICIENCY AND CONDITIONALLY ESSENTIAL NUTRIENTS IN DISEASE
Arginine as an Example of a Conditionally Essential Nutrient: Sickle Cell Anemia and Surgery
Traumatic Brain Injury: Pathophysiological Mechanisms and Potential Nutrient Needs
Metabolic Turnover, Inflammation, and Redistribution: Impact on Nutrient Requirements
Moderated Panel Discussion and Q&A
5 BUILDING THE EVIDENCE BASE: RESEARCH APPROACHES FOR NUTRIENTS IN DISEASE STATES
Type and Strength of Evidence Needed for Determining Special Nutrient Requirements
Identification and Validation of Biomarkers in Disease States
Innovative Causal Designs for Efficacy: What Type of Evidence Is Needed?
Examples of a Complex Disease: Inflammatory Bowel Disease
Examples of a Complex Disease: Cancer
Moderated Panel Discussion and Q&A
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Boxes, Figures, and Tables
BOXES
1-2 What Constitutes a Distribution Outside of the Dietary Reference Intakes?
FIGURES
1-2 Classifying and evaluating human nutrient needs
2-1 Mitochondrial disease: Molecular pathways affected by genetic disorders
2-2 Novel treatment strategies in mitochondrial disease
2-3 Daily protein intake by patients with methylmalonic acidemia mut subtype, sorted by age
3-1 Prevalence of micronutrient deficiencies during parenteral nutrition weaning
3-2 Survival in children with cystic fibrosis at various weight-for-age percentiles
3-3 Influence of disease on whole-body nutrient status and tissue-specific nutrient status
4-2 Mechanisms of vasculopathy and endothelial dysfunction
5-1 Risk assessment approach to setting Dietary Reference Intake values
5-2 Establishing adequacy: Biomarkers of exposure on the causal pathway, using folate as an example
5-3 Nutrient requirements in disease states
5-4 Example of complex relationships in cystic fibrosis
5-5 The relationship among diet, the gut microbiota, and inflammatory bowel disease
TABLES
1-1 Comparison of Dietary Reference Intakes and Special Nutritional Needs
3-1 Summary of Nutrient Requirements in Chronic Kidney Disease
Acronyms and Abbreviations
AI |
Adequate Intake |
AND |
Academy of Nutrition and Dietetics |
ASN |
American Society for Nutrition |
ATP |
adenosine triphosphate |
BBB |
blood–brain barrier |
BMI |
body mass index |
BOND |
Biomarkers of Nutrition for Development |
BRINDA |
Biomarkers Reflecting Inflammation and Nutritional Determinants of Anemia |
CF |
cystic fibrosis |
CFD |
cerebral folate deficiency |
CFTR |
cystic fibrosis transmembrane conductance regulator |
CHOP |
Children’s Hospital of Pennsylvania |
CKD |
chronic kidney disease |
CRP |
C-reactive protein |
CSF |
cerebrospinal fluid |
DHA |
docosahexaenoic acid |
DNA |
deoxyribonucleic acid |
DoD |
Department of Defense |
DRI |
Dietary Reference Intake |
EAR |
Estimated Average Requirement |
EEN |
exclusive enteral nutrition |
EPA |
eicosapentaenoic acid |
FARMM |
Food and Resulting Microbial Metabolites |
FDA |
U.S. Food and Drug Administration |
FODMAP |
fermentable oligosaccharides, disaccharides, monosaccharides, and polyols |
GFR |
glomerular filtration rate |
GI |
gastrointestinal |
GMP |
glycomacropeptide |
GRADE |
Grading of Recommendations Assessment, Development and Evaluation |
IBD |
inflammatory bowel disease |
IOM |
Institute of Medicine |
MELAS |
mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes |
MMA |
methylmalonic aciduria |
NAD+ |
nicotinamide adenine dinucleotide |
NADH |
nicotinamide adenine dinucleotide + hydrogen |
NADPH |
nicotinamide adenine dinucleotide phosphate |
NHANES |
National Health and Nutrition Examination Survey |
NIH |
National Institutes of Health |
NOS |
nitric oxide synthase |
PAH |
phenylalanine hydroxylase |
PEW |
protein energy wasting |
Phe |
phenylalanine |
PICO |
Population, Intervention, Comparisons, Outcomes |
PKU |
phenylketonuria |
PLP |
pyridoxal phosphate |
RCT |
randomized controlled trial |
RD |
registered dietitian |
RDA |
Recommended Dietary Allowance |
TBI |
traumatic brain injury |
UL |
Tolerable Upper Intake Level |
WCRF-AICR |
World Cancer Research Fund and the American Institute for Cancer Research |
WHO |
World Health Organization |
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