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Protein and calorie intakes in adult and pediatric subjects with urea cycle disorders participating in clinical trials of glycerol phenylbutyrate()
BACKGROUND: Little prospectively collected data are available comparing the dietary intake of urea cycle disorder (UCD) patients to UCD treatment guidelines or to healthy individuals. OBJECTIVE: To examine the protein and calorie intakes of UCD subjects who participated in clinical trials of glycero...
Autores principales: | , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elsevier
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4789342/ https://www.ncbi.nlm.nih.gov/pubmed/27014577 http://dx.doi.org/10.1016/j.ymgmr.2015.11.002 |
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author | Hook, Debra Diaz, George A. Lee, Brendan Bartley, James Longo, Nicola Berquist, William Le Mons, Cynthia Rudolph-Angelich, Ingrid Porter, Marty Scharschmidt, Bruce F. Mokhtarani, Masoud |
author_facet | Hook, Debra Diaz, George A. Lee, Brendan Bartley, James Longo, Nicola Berquist, William Le Mons, Cynthia Rudolph-Angelich, Ingrid Porter, Marty Scharschmidt, Bruce F. Mokhtarani, Masoud |
author_sort | Hook, Debra |
collection | PubMed |
description | BACKGROUND: Little prospectively collected data are available comparing the dietary intake of urea cycle disorder (UCD) patients to UCD treatment guidelines or to healthy individuals. OBJECTIVE: To examine the protein and calorie intakes of UCD subjects who participated in clinical trials of glycerol phenylbutyrate (GPB) and compare these data to published UCD dietary guidelines and nutritional surveys. DESIGN: Dietary data were recorded for 45 adult and 49 pediatric UCD subjects in metabolic control during participation in clinical trials of GPB. Protein and calorie intakes were compared to UCD treatment guidelines, average nutrient intakes of a healthy US population based on the National Health and Nutrition Examination Survey (NHANES) and Recommended Daily Allowances (RDA). RESULTS: In adults, mean protein intake was higher than UCD recommendations but lower than RDA and NHANES values, while calorie intake was lower than UCD recommendations, RDA and NHANES. In pediatric subjects, prescribed protein intake was higher than UCD guidelines, similar to RDA, and lower than NHANES data for all age groups, while calorie intake was at the lower end of the recommended UCD range and close to RDA and NHANES data. In pediatric subjects height, weight, and body mass index (BMI) Z-scores were within normal range (− 2 to 2). CONCLUSIONS: Pediatric patients treated with phenylbutyrate derivatives exhibited normal height and weight. Protein and calorie intakes in adult and pediatric UCD subjects differed from UCD dietary guidelines, suggesting that these guidelines may need to be reconsidered. |
format | Online Article Text |
id | pubmed-4789342 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | Elsevier |
record_format | MEDLINE/PubMed |
spelling | pubmed-47893422016-03-24 Protein and calorie intakes in adult and pediatric subjects with urea cycle disorders participating in clinical trials of glycerol phenylbutyrate() Hook, Debra Diaz, George A. Lee, Brendan Bartley, James Longo, Nicola Berquist, William Le Mons, Cynthia Rudolph-Angelich, Ingrid Porter, Marty Scharschmidt, Bruce F. Mokhtarani, Masoud Mol Genet Metab Rep Research Paper BACKGROUND: Little prospectively collected data are available comparing the dietary intake of urea cycle disorder (UCD) patients to UCD treatment guidelines or to healthy individuals. OBJECTIVE: To examine the protein and calorie intakes of UCD subjects who participated in clinical trials of glycerol phenylbutyrate (GPB) and compare these data to published UCD dietary guidelines and nutritional surveys. DESIGN: Dietary data were recorded for 45 adult and 49 pediatric UCD subjects in metabolic control during participation in clinical trials of GPB. Protein and calorie intakes were compared to UCD treatment guidelines, average nutrient intakes of a healthy US population based on the National Health and Nutrition Examination Survey (NHANES) and Recommended Daily Allowances (RDA). RESULTS: In adults, mean protein intake was higher than UCD recommendations but lower than RDA and NHANES values, while calorie intake was lower than UCD recommendations, RDA and NHANES. In pediatric subjects, prescribed protein intake was higher than UCD guidelines, similar to RDA, and lower than NHANES data for all age groups, while calorie intake was at the lower end of the recommended UCD range and close to RDA and NHANES data. In pediatric subjects height, weight, and body mass index (BMI) Z-scores were within normal range (− 2 to 2). CONCLUSIONS: Pediatric patients treated with phenylbutyrate derivatives exhibited normal height and weight. Protein and calorie intakes in adult and pediatric UCD subjects differed from UCD dietary guidelines, suggesting that these guidelines may need to be reconsidered. Elsevier 2016-01-21 /pmc/articles/PMC4789342/ /pubmed/27014577 http://dx.doi.org/10.1016/j.ymgmr.2015.11.002 Text en © 2015 The Authors http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). |
spellingShingle | Research Paper Hook, Debra Diaz, George A. Lee, Brendan Bartley, James Longo, Nicola Berquist, William Le Mons, Cynthia Rudolph-Angelich, Ingrid Porter, Marty Scharschmidt, Bruce F. Mokhtarani, Masoud Protein and calorie intakes in adult and pediatric subjects with urea cycle disorders participating in clinical trials of glycerol phenylbutyrate() |
title | Protein and calorie intakes in adult and pediatric subjects with urea cycle disorders participating in clinical trials of glycerol phenylbutyrate() |
title_full | Protein and calorie intakes in adult and pediatric subjects with urea cycle disorders participating in clinical trials of glycerol phenylbutyrate() |
title_fullStr | Protein and calorie intakes in adult and pediatric subjects with urea cycle disorders participating in clinical trials of glycerol phenylbutyrate() |
title_full_unstemmed | Protein and calorie intakes in adult and pediatric subjects with urea cycle disorders participating in clinical trials of glycerol phenylbutyrate() |
title_short | Protein and calorie intakes in adult and pediatric subjects with urea cycle disorders participating in clinical trials of glycerol phenylbutyrate() |
title_sort | protein and calorie intakes in adult and pediatric subjects with urea cycle disorders participating in clinical trials of glycerol phenylbutyrate() |
topic | Research Paper |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4789342/ https://www.ncbi.nlm.nih.gov/pubmed/27014577 http://dx.doi.org/10.1016/j.ymgmr.2015.11.002 |
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