Original ArticlesThe Relationship between Dietary Intake, Growth, and Body Composition in Inborn Errors of Intermediary Protein Metabolism
Section snippets
Methods
This study was approved by the Royal Children's Hospital Human Research Ethic Committee (HREC: 30066B).
We collected longitudinal data on dietary intake and growth of patients born between 1976 and December 2014 (n = 75; 30 males, 45 females) with IVA (n = 7), MMA/PA (n = 14), UCD (n = 44), and classical MSUD (n = 10). Data were collected from medical and dietetic clinic records when patients were metabolically stable. Dietary data consisted of dietary recall, food diaries, and dietary history.
Results
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 BMI, respectively (Table and Figure 1). Longitudinal growth patterns differed between the groups. Children with IVA had essentially normal growth patterns. In children with MMA/PA, median height z-scores were persistently less than −1 after 5 years of age. In children with UCD, growth patterns were normal, although male patients had significantly higher BMI z-scores than
Discussion
Growth patterns in inborn errors of intermediary protein metabolism may be affected by both disease pathophysiology and the nutritional adequacy of consumed or prescribed diets. In this study, we assessed dietary adequacy and explored associated nutritional outcomes to define an apparent safe P:E ratio to be used as an additional clinical tool in the management of these patients. A strength of our study is that the protein intake reported was the estimated amount actually consumed rather than
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Cited by (0)
Portions of this study were presented at the ICIEM, Barcelona, Spain, September, 2013 and at the Genetic Metabolic Dietitians International Conference, Phoenix, Arizona, April, 2016.
The authors declare no conflicts of interest.