O-3 Clinical features and outcome measures during 1 year enzyme replacement therapy in late onset GSD II patients

Influence of comorbidity and cognitive status on instrumental activities of daily living in amnestic mild cognitive impairment: results from the ReGAl project.

Objectives To investigate whether amnestic mild cognitive impairment (aMCI) is characterised by restriction in instrumental activities of daily living (IADL). Further, to examine the role of comorbidity and cognitive performance on IADL changes in aMCI subjects. Methods The study included 132 subjects with aMCI and 249 subjects with no cognitive impairment (NCI), consecutively enrolled as outpatients in a multicentric Italian clinical-based study, the ReGAl Project. All subjects underwent a comprehensive evaluation including clinical examination, laboratory screening, neuroimaging and cognitive and behavioral assessments. Functional status was evaluated by the Lawton's Instrumental Activiti…

Relationship between quality of life and behavioural disorders in children with persistent asthma: a Multiple Indicators Multiple Causes (MIMIC) model

AbstractKnowledge on multiple interdependences between quality of life (QoL) and behavioural problems in relation to asthma severity and control is undetermined. The aims of the study were: (i) to assess the relationship of QoL and behavioural problems with asthma severity and control (ii) to predict children’s “abnormal/borderline” status with variation in QoL. For these purposes a multicenter case-control study on 47 Severe Asthma (SA) and 94 Moderate Asthma (MA) children was performed. The MIMIC approach was applied to investigate the effect of SA and non-controlled asthma (NC) on QoL and behavioural disorders. Logistic regression was used to estimate probabilities of having an “abnormal…

Prioritization of high-cost new drugs for HCV: making sustainability ethical

Hepatitis C virus (HCV) infection is a major health problem worldwide. Chronic HCV infection may in the long run cause cirrhosis, hepatic decompensation and hepatocellular carcinoma, with an ultimate disease burden of at least 350,000 deaths per year worldwide. The new generation of highly effective direct acting antivirals (DAA) to treat HCV infection brings major promises to infected patients in terms of exceedingly high rates of sustained virological response (SVR) but also of tolerability, allowing even the sickest patients to be treated. Even in the face of the excellent safety and efficacy and wide theoretical applicability of these regimens, their introduction is currently facing cos…

Clinical strategies for the management of intestinal obstruction and pseudo-obstruction. A Delphi Consensus study of SICUT (Società Italiana di Chirurgia d'Urgenza e del Trauma)

Intestinal obstructions/pseudo-obstruction of the small/large bowel are frequent conditions but their management could be challenging. Moreover, a general agreement in this field is currently lacking, thus SICUT Society designed a consensus study aimed to define their optimal workout.The Delphi methodology was used to reach consensus among 47 Italian surgical experts in two study rounds. Consensus was defined as an agreement of 75.0% or greater. Four main topic areas included nosology, diagnosis, management and treatment.A bowel obstruction was defined as an obstacle to the progression of intestinal contents and fluids generally beginning with a sudden onset. The panel identified four major…