Rate and duration of hospitalisation for acute pulmonary embolism in the real-world clinical practice of different countries : Analysis from the RIETE registry

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Description of clinical and genetic features of 122 patients included in the Spanish Pompe registry

\ua9 2023Pompe disease is a rare genetic disorder with an estimated prevalence of 1:60.000. The two main phenotypes are Infantile Onset Pompe Disease (IOPD) and Late Onset Pompe Disease (LOPD). There is no published data from Spain regarding the existing number of cases, regional distribution, clinical features or, access and response to the treatment. We created a registry to collect all these data from patients with Pompe in Spain. Here, we report the data of the 122 patients registered including nine IOPD and 113 LOPD patients. There was a high variability in how the diagnosis was obtained and how the follow-up was performed among different centres. Seven IOPD patients were still alive being all treated with enzymatic replacement therapy (ERT) at last visit. Ninety four of the 113 LOPD patients had muscle weakness of which 81 were receiving ERT. We observed a progressive decline in the results of muscle function tests during follow-up. Overall, the Spanish Pompe Registry is a valuable resource for understanding the demographics, patient\u27s journey and clinical characteristics of patients in Spain. Our data supports the development of agreed guidelines to ensure that the care provided to the patients is standardized across the country

Cheese consumption and prevalence of overweight and obesity in a Basque adult population: a cross-sectional study

[EN] Studies have reported a negative association between dairy product consumption and weight status. However, not as much research has focused on cheese; therefore, the aim of this study was to study the association between cheese intake and overweight and obesity in a representative Basque adult population. A food frequency questionnaire (FFQ) was obtained from a random sample of 1081 adults (530 males and 551 females, 17–96 years old). Cheese consumption data were expressed as g/1000 kcal/day. The prevalence of overweight/obesity was higher in men (55.1%) than in women (35.4%) (p50.001). Participants with low or moderate intake of fresh and processed cheese demonstrated a higher prevalence of excess weight, compared with those with higher consumption. The confounding variables selected in multivariate analysis were occupational status and age in both genders; and place of residence in men. In conclusion, negative associations were found between consumption of some types of cheese and overweight and obesity in this population