PD532 - INCREASED ADIPOSITY DESPITE LOW ENERGY AND PROTEIN INTAKE IN ADULTS WITH UREA CYCLE DISORDERS AND METHYLMALONIC ACIDAEMIA/PROPIONIC ACIDAEMIA ON LONG-TERM LOW-PROTEIN DIETS: A SECONDARY ANALYSIS
PD532
INCREASED ADIPOSITY DESPITE LOW ENERGY AND PROTEIN INTAKE IN ADULTS WITH UREA CYCLE DISORDERS AND METHYLMALONIC ACIDAEMIA/PROPIONIC ACIDAEMIA ON LONG-TERM LOW-PROTEIN DIETS: A SECONDARY ANALYSIS
G. Gugelmo1,*, F. Francini Pesenti2, L. Lenzini3, G. P. Fadini1, L. Busetto2, A. Burlina4, N. Vitturi1
1Division of Metabolic Diseases, 2Division of Clinical Nutrition, 3Department of Medicine, 4Division of Inherited Metabolic Diseases, Department of Woman's and Child's Health, Azienda Ospedale Università Padova, Padova, Italy
Rationale: Low-protein diets (LPDs) are the cornerstone of treatment in adults with urea cycle disorders (UCDs), methylmalonic acidaemia (MMA) and propionic acidaemia (PA). While dietary management focuses on metabolic stability, the long-term impact of chronic protein restriction on body composition remains poorly defined.
Methods: A secondary analysis of 14 adults (28.6±7.9 years;11 UCD,2 MMA,1 PA) was performed from a previously published cohort (1), evaluating dietary intake (7-day food diary) and body composition by bioimpedance analysis.
Results: Mean body mass index was 25.3±3.9 kg/m² (54% overweight). Mean energy intake (EI) was 24.2±5 kcal/kg/day; mean total protein intake (TPI) was 0.56±0.16 g/kg/day, below WHO safe levels and inversely associated with fat mass (FM) index (r=−0.61, p=0.03) and FM% (r=−0.68, p=0.01). On univariate analysis, both EI and TPI were associated with FM index, unlike the P:E ratio (2.26±0.45 g/100 kcal/day) (Table 1).
Model |
Predictor |
β |
p-value |
R² |
|
Univariate |
TPI |
−12.5 |
0.05 |
0.33 |
|
Multivariate (adj. age+sex) |
TPI |
−9.54 |
0.11 |
0.57 |
|
Univariate |
P:E ratio |
−0.92 |
0.70 |
0.01 |
|
Full multivariable model |
TPI+EI+Age+Sex |
No independent predictors |
— |
0.69 |
|
Univariate |
EI |
−0.43 |
0.01 |
0.46 |
Table 1.
Conclusion: Despite low reported EI, overweight was high. Absolute TPI and EI were more informative for body composition than the P:E ratio, unlike paediatric data where P:E ratio (1.5–2.9 g/100 kcal/day) was indicative of optimal growth outcomes (2). Physical inactivity, due to fear of metabolic decompensation, may represent an unmeasured determinant of altered body composition. Routine long-term monitoring (also by DXA and muscle and visceral fat ultrasound) is warranted for adiposity and sarcopenic features and guide nutritional interventions to reduce cardiometabolic risk.
References: 1. Gugelmo G,et al.Nutrients(2022)
2. Evans M,et al.J Pediatr.(2017)
Disclosure of Interest: None declared