The Relationship between Dietary Intake, Growth, and Body Composition in Inborn Errors of Intermediary Protein Metabolism
| Autor: | Helen Truby, Maureen Evans, Avihu Boneh |
|---|---|
| Rok vydání: | 2016 |
| Předmět: |
0301 basic medicine
Male medicine.medical_specialty Urea cycle disorder Adolescent Methylmalonic acidemia 030105 genetics & heredity Gastroenterology Body Mass Index 03 medical and health sciences 0302 clinical medicine Weight loss Internal medicine medicine Body Fat Distribution Humans Mass index Longitudinal Studies Propionic acidemia Child Amino Acid Metabolism Inborn Errors Retrospective Studies business.industry Body Weight food and beverages medicine.disease Body Height Endocrinology Organic acidemia Child Preschool Pediatrics Perinatology and Child Health Body Composition Median body Female Dietary Proteins medicine.symptom business Energy Intake Body mass index 030217 neurology & neurosurgery |
| Zdroj: | The Journal of pediatrics. 188 |
| ISSN: | 1097-6833 |
| Popis: | To examine relationships between dietary intake, growth and body composition patterns in patients with inborn errors of intermediary protein metabolism and to determine a safe protein:energy ratio (P:E ratio) associated with optimal growth outcomes.Retrospective longitudinal data of growth and dietary intake in patients (n = 75) with isovaleric acidemia (IVA; n = 7), methylmalonic acidemia/propionic acidemia (MMA/PA; n = 14), urea cycle defects (UCD; n = 44), classical maple syrup urine disease (MSUD; n = 10) were collected. Prospective longitudinal data of growth, dietary intake, and body composition from 21 patients: IVA (n = 5), MMA/PA (n = 6), UCD (n = 7), and MSUD (n = 3) were collected at clinic visits.Fifty-two of 75 (66%), 49 of 74 (68%), and 44 of 65 (68%) patients had a z-score of 0 (±1) for lifetime weight, height, and body mass index, respectively. Patients with MMA/PA had the lowest median height and weight z-scores, and MSUD patients had highest median body mass index z-score at all ages. In IVA, MMA/PA, and UCD, total natural protein intake met or exceeded the Food and Agriculture Organization of the United Nations (FAO)/World Health Organization (WHO)/United Nations University (UNU) recommended safe levels. Median percentage fat mass was 17.6% in IVA, 20.7% in MMA/PA, 19.4% in UCD, and 17.8% in MSUD. There was a significant negative correlation between percentage fat mass and total protein intake in IVA, MMA/PA, and UCD (r = -0.737; P = .010). The correlation between the P:E ratio and growth variables in IVA, MMA/PA, and UCD suggest a safe P:E ratio (1.5 to 2.9) g protein:100 kcal/day.Growth outcomes in inborn errors of intermediary protein metabolism are not always ideal. Most patients with IVA, MMA/PA, and UCD consume sufficient natural protein to meet FAO/WHO/UNU recommendations. A P:E ratio range of (1.5 to 2.9)g protein/100 kcal/day correlates with optimal growth outcomes. |
| Databáze: | OpenAIRE |
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