Abstract

OBJECTIVE: Special low-protein foods (SLPFs) are essential to achieving adequate caloric intake, improving dietary adherence, preventing catabolism and ensuring good metabolic control in patients with hereditary metabolic disease. This study aims to analyze the composition of SLPFs in Italy and compare them with similar regular foods (RFs).

MATERIALS AND METHODS: We collected data on the composition and ingredients of SLPFs distributed in Italy and on the composition of the corresponding RFs.

RESULTS: A total of 177 SLPFs were identified and compared with 100 RFs from the same product categories. The average energy content was 326 kcal (range: 49.9–565.5 kcal) for all SLPFs and 329 kcal (range: 48.7–543 kcal) for all RFs. The amount of phenylalanine (Phe) was declared on 81% of SLPFs (143/177 products); for 54% of SLPFs (n=77/143), the Phe content was ≤25 mg per 100 g; for 7% of SLPFs (n=10/143), the Phe content was ≥100 mg per 100 g. Total carbohydrate content was higher in 51.8% (14/27) of SLPF subgroups than in RFs; sugars were higher in 33.3% (9/27) of SLPF subgroups than in RFs; 51.8% (14/27) of SLPF subgroups had higher total fat content than RFs, while 37% (10/27) of SLPF subgroups had lower total fat content than RFs. The fiber amount was >0 in 85% (n=151/177) of SLPFs, while 76% (n=135/177) of SLPFs contained added fiber.

CONCLUSIONS: SLPFs provide an important part of the energy requirements in the diet of patients with hereditary metabolic diseases; since these patients must follow the diet for life, improving the nutritional quality of these products is mandatory to ensure metabolic compensation and maintenance of good health in adulthood.