The 6 May 1976 Friuli earthquake: re-evaluating and consolidating

The aim of this paper is to propose the creation, in terms of European Macroseismic Scale (EMS-98), of the entire macroseismic fi eld of the 6 May 1976 Friuli earthquake. Only forty odd years have passed, and nothwithsatnding that there is a huge quantity of existing data, it was still disturbing to fi nd that much of the original data are missing and probably lost forever Efforts have therefore been made to fi nd additional and still unknown primary data. For the majority of the collected national data sets, a reevaluation was then possible. This study presents the comprehensive macroseismic data set for 14 European countries. It is, to our knowledge, one of the largest European data sets, consisting of 3423 intensity data points (IDPs). The earthquake was felt from Rome to the Baltic Sea, and from Belgium to Warsaw. The maximum intensity 10 EMS-98 was reached in eight localities in Friuli (Italy). Compared to previous studies, the Imax values have changed from country to country, in some cases being lowered due to methodological differences, but in the case of three among the most hit countries, Imax is now higher than in the previous studies, mainly due to the new data.Published417-4444T. Sismicità dell'ItaliaJCR Journa

Management of phenylketonuria in Europe: Survey results from 19 countries

To gain better insight in the most current diagnosis and treatment practices for phenylketonuria (PKU) from a broad group of experts, a European PKU survey was performed. The questionnaire, consisting of 33 questions, was sent to 243 PKU professionals in 165 PKU centers in 23 European countries. The responses were compiled and descriptive analyses were performed. One hundred and one questionnaires were returned by 93/165 centers (56%) from 19/23 European countries (83%). The majority of respondents (77%) managed patients of all age groups and more than 90% of PKU teams included physicians or dieticians/nutritionists. The greatest variability existed especially in the definition of PKU phenotypes, therapeutic blood phenylalanine (Phe) target concentrations, and follow-up practices for PKU patients. The tetrahydrobiopterin (BH4; sapropterin) loading test was performed by 54% of respondents, of which 61% applied a single dose test (20mg/kg over 24h). BH4 was reported as a treatment option by 34%. This survey documents differences in diagnostic and treatment practices for PKU patients in European centers. In particular, recommendations for the treatment decision varied greatly between different European countries. There is an urgent need to pool long-term data in PKU registries in order to generate an evidence-based international guideline

Management of phenylketonuria in Europe: Survey results from 19 countries

To gain better insight in the most current diagnosis and treatment practices for phenylketonuria (PKU) from a broad group of experts, a European PKU survey was performed. The questionnaire. consisting of 33 questions, was sent to 243 PKU professionals in 165 PKU centers in 23 European countries. The responses were compiled and descriptive analyses were performed. One hundred and one questionnaires were returned by 93/165 centers (56%) from 19/23 European countries (83%). The majority of respondents (77%) managed patients of all age groups and more than 90% of PKU teams included physicians or dieticians/nutritionists. The greatest variability existed especially in the definition of PKU phenotypes, therapeutic blood phenylalanine (Phe) target concentrations, and follow-up practices for PKU patients. The tetrahydrobiopterin (BH4: sapropterin) loading test was performed by 54% of respondents, of which 61% applied a single dose test (20 mg/kg over 24 h). BH4 was reported as a treatment option by 34%. This survey documents differences in diagnostic and treatment practices for PKU patients in European centers. In particular, recommendations for the treatment decision varied greatly between different European countries. There is an urgent need to pool long-term data in PKU registries in order to generate an evidence-based international guideline