RESUMEN

Background: Late-onset Pompe disease (LOPD) is an autosomal-recessive metabolic myopathy caused by deficiency of the lysosomal enzyme Acid Alpha-Glucosidase (GAA), leading to glycogen accumulation in proximal and axial muscles, and in the diaphragm. Enzyme Replacement Therapy (ERT) with recombinant GAA became available in 2006. Since then, several outcome measures have been investigated for the adequate follow-up of disease progression and treatment response, usually focusing on respiratory and motor function. Prognostic factors predicting outcome have not been identified till now. Methods: In this single Centre, prospective study, we evaluate the response to enzyme replacement therapy in 15 patients (7 males) with LOPD in different stages of disease, aged 49.4 ± 16.1, followed-up for 15 years. Treatment response was measured by the 6-min walking test, vital capacity in supine and upright position, respiratory muscle strength, muscle MRI, manual muscle testing. We investigated the usefulness of Body Impedance Vectorial Analysis for serial body composition assessment. Results: Although most patients with LOPD benefit from long-term treatment, some secondary decline may occur after the first 3-5 years. Some nutritional (lower body mass index, higher fat free mass, higher phase angle) and disease parameters (higher creatinine and shorter disease duration at the beginning of treatment) seem to predict a better motor outcome. Lower Phase Angle, possibly reflecting loss of integrity of skeletal muscle membranes and thus treatment mis-targeting, seems to correlate with worse treatment response on long-term follow-up. Conclusion: Body Impedance Vectorial Analysis is a fast, easily performed and cheap tool that may be able to predict long-term treatment response in patients with LOPD. Low Phase angle may serve as a marker of muscle quality and may be used to predict the response to a muscle-targeted intervention such as ERT, thus improving the identification of patients needing a closer follow-up due to higher fragility and risk of deterioration.

RESUMEN

OBJECTIVE: A few studies link out-of-home eating to higher energy consumption, overweight and obesity in both adults and children. The present study was undertaken to investigate the nutritional value of meals available in a university cafeteria, in order to develop a target nutritional tool to help consumers make a more conscious nutritional choice. DESIGN: A cross-sectional study. SETTING: In a university cafeteria in Pavia, northern Italy, the recipes and ingredients of each meal served during the whole year were obtained from the cooks. Energy, protein, fat, carbohydrate and fibre contents were computed for each meal standardized portion. Thirteen pyramid figures, subdivided into three coloured levels, were used to depict the energy and nutrient content of each meal. SUBJECTS: Four hundred randomly selected customers were interviewed on the cafeteria nutritional proposal. RESULTS: Foods available in the cafeteria consisted of 216 items and were distributed in the pyramids according to their energy content: the lowest ones at the bottom (green level) and the highest ones at the top (red level), passing through an orange level in the middle. Energy values ranged from 460 kJ (110 kcal) for a portion of dressed vegetables to 5021 kJ (1200 kcal) for a pizza. The depicted pyramids were displayed in the cafeteria, so that customers could choose their meal according to its nutritional value. The meals' nutritional content information was perceived very helpful for customers' nutritional choices. CONCLUSIONS: Availability of nutrition information in the cafeteria was well accepted by the customers who could plan their meals according to a more balanced diet.

Asunto(s)

RESUMEN

Our objective was to assess the reliability and relative validity of a food frequency questionnaire (FFQ) among adult people. In a cross-sectional study carried out in northern Italy, 112 adults were recruited. A total of 189 food and drink items were selected according to those typically consumed by Italians. FFQ reliability was assessed by two repeated administrations at 6 weeks. The FFQ was validated using four 24-h recalls repeated in the same period of time. For the validation study, classification into quartiles from the two methods and Bland-Altman plot were also performed. The reliability study showed a good correlation between the two methods. Bland-Altman plots showed that the two methods are very likely to agree for individual energy and macronutrient intakes. The reliability and relative validity of this FFQ was good, supporting its use in assessing dietary intakes of Italians in nutritional surveillance programs and in epidemiological dietary surveys.

Asunto(s)

RESUMEN

OBJECTIVE: The present study evaluates eating habits and behaviors, and nutritional and food safety knowledge of a group of Italian adolescents. DESIGN: A dietary questionnaire previously constructed and tested was self-administered during school time. Each section was evaluated using a separate score. SETTING: The study was carried out as a part of a nutritional surveillance project in the Aosta Valley Region, Northern Italy. SUBJECTS: Five hundred and thirty-two adolescent subjects, aged 15.4 +/- 0.7 years, attending the second year of secondary schools participated in the study. MEASURES: We evaluated eating habits, physical activity, meaning of healthy and unhealthy dietary habits and food, self-efficacy, barriers affecting healthy food choices, nutritional and food safety, weight, height, Body Mass Index (BMI). RESULTS: Only 37.0% of the sample have satisfactory eating habits; 18.5% have a very active lifestyle; only 8.6% have quite good nutritional knowledge, 2.4% have satisfactory food safety knowledge, although 43.7% have good hygiene practices. CONCLUSIONS: The results point out unhealthy behaviors influencing adolescents' eating habits and suggest which of these must be considered in order to develop tailored nutrition interventions, improving adolescents' consciousness aimed at adopting a healthy lifestyle.

Asunto(s)