Protein and calorie intakes in adult and pediatric subjects with urea cycle disorders participating in clinical trials of glycerol phenylbutyrate
| Autor: | Nicola Longo, Bruce F. Scharschmidt, Cynthia Le Mons, Masoud Mokhtarani, Marty Porter, James Bartley, Brendan Lee, Ingrid Rudolph-Angelich, William E. Berquist, George A. Diaz, Debra Hook |
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| Rok vydání: | 2016 |
| Předmět: |
0301 basic medicine
medicine.medical_specialty Calorie Urea cycle disorder Phenylbutyrate WHO World Health Organization Urea cycle disorders 03 medical and health sciences chemistry.chemical_compound 0302 clinical medicine Endocrinology NaPBA sodium phenylbutyrate Internal medicine Inherited metabolic disorders UCD urea cycle disorder Genetics medicine Glycerol phenylbutyrate Molecular Biology Dietary management lcsh:QH301-705.5 lcsh:R5-920 business.industry medicine.disease GPB glycerol phenylbutyrate Clinical trial 030104 developmental biology chemistry lcsh:Biology (General) RDA Recommended Daily Allowances Protein intake Urea cycle Healthy individuals NHANESs National Health and Nutrition Examination Surveys OTC ornithine transcarbamylase FAO Food and Agriculture Organization business lcsh:Medicine (General) 030217 neurology & neurosurgery Research Paper |
| Zdroj: | Molecular Genetics and Metabolism Reports, Vol 6, Iss C, Pp 34-40 (2016) Molecular Genetics and Metabolism Reports |
| ISSN: | 2214-4269 |
| DOI: | 10.1016/j.ymgmr.2015.11.002 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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