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Baldacchino A.,University of St. Andrews | Crocamo C.,University of Pavia | Humphris G.,University of St. Andrews | Neufeind J.,University of St. Andrews | And 3 more authors.
Computer Methods and Programs in Biomedicine | Year: 2016

Background and objective: The application of e-health technology to the field of substance use disorders is at a relatively early stage, and methodological quality is still variable. Few have explored the extent of utilization of communication technology in exploring risk perception by patients enrolled in substance abuse services.The Overdose RIsk InfOrmatioN (ORION) project is a European Commission funded programme, aimed to develop and pilot an e-health psycho-educational tool to provide information to drug using individuals about the risks of suffering a drug overdose. Methods: In this article, we report on phase 1 (risk estimation), phase 2 (design), and phase 3 (feasibility) of the ORION project. Results: The development of ORION e-health tool underlined the importance of an evidence-based intervention aimed in obtaining reliable evaluation of risk. The ORION tool supported a decision making process aimed at influencing the substance users' self-efficacy and the degree to which the substance users' understand risk factors. Therefore, its innovative power consisted in translating risks combination into a clear estimation for the user who will then appear more likely to be interested in his/her risk perception. Conclusion: Exploratory field testing and validation confirmed the next stage of evaluation, namely, collection of routine patient samples in study clinics. The associations between risk perception of overdose, engagement with the ORION tool and willingness to alter overdose risk factors, in a clinical setting across various EU member states will further confirm the ORION tool's generalisability and effectiveness. © 2016 Elsevier Ireland Ltd.


Baldacchino A.,University of St. Andrews | Crocamo C.,University of Pavia | Humphris G.,University of St. Andrews | Neufeind J.,University of St. Andrews | And 3 more authors.
Computer Methods and Programs in Biomedicine | Year: 2016

Background and objective: The application of e-health technology to the field of substance use disorders is at a relatively early stage, and methodological quality is still variable. Few have explored the extent of utilization of communication technology in exploring risk perception by patients enrolled in substance abuse services.The Overdose RIsk InfOrmatioN (ORION) project is a European Commission funded programme, aimed to develop and pilot an e-health psycho-educational tool to provide information to drug using individuals about the risks of suffering a drug overdose. Methods: In this article, we report on phase 1 (risk estimation), phase 2 (design), and phase 3 (feasibility) of the ORION project. Results: The development of ORION e-health tool underlined the importance of an evidence-based intervention aimed in obtaining reliable evaluation of risk. The ORION tool supported a decision making process aimed at influencing the substance users' self-efficacy and the degree to which the substance users' understand risk factors. Therefore, its innovative power consisted in translating risks combination into a clear estimation for the user who will then appear more likely to be interested in his/her risk perception. Conclusion: Exploratory field testing and validation confirmed the next stage of evaluation, namely, collection of routine patient samples in study clinics. The associations between risk perception of overdose, engagement with the ORION tool and willingness to alter overdose risk factors, in a clinical setting across various EU member states will further confirm the ORION tool's generalisability and effectiveness. © 2016 Elsevier Ireland Ltd.


Harmatz P.R.,UCSF Benioff Childrens Hospital Oakland | Mengel K.E.,University of Mainz | Giugliani R.,Federal University of Rio Grande do Sul | Valayannopoulos V.,Institut Universitaire de France | And 13 more authors.
Molecular Genetics and Metabolism | Year: 2015

Objectives: Baseline data from the Morquio A Clinical Assessment Program (MorCAP) revealed that individuals with Morquio A syndrome show substantial impairment in multiple domains including endurance and respiratory function (Harmatz et al., Mol Genet Metab, 2013). Here, 1- and 2-year longitudinal endurance and respiratory function data are presented. Methods: Endurance was assessed using the 6-minute walk test (6MWT) and the 3-minute stair climb test (3MSCT). Respiratory function was evaluated by measuring forced vital capacity (FVC) and maximum voluntary ventilation (MVV). Data were analyzed using repeated measures ANCOVA models. Annualized estimates of change were determined using model estimates and interpolation. Results: 353, 184, and 78 subjects were assessed at Year 0 (baseline), Year 1, and Year 2, respectively. The overall annualized estimate of change (SE) in 6MWT distance was - 4.86 ± 3.25. m; a larger decline of - 6.84 ± 5.38. m was observed in the subset of subjects meeting the inclusion/exclusion criteria of the Phase 3 clinical trial of elosulfase alfa (≥. 5. years of age with baseline 6MWT distance ≥. 30 and ≤. 325. m). In contrast, little change (- 0.14 ± 0.60. stairs/min) was observed in 3MSCT. Annualized changes (SE) in FVC and MVV were 2.44 ± 0.68% and 1.01 ± 2.38%, respectively. FVC and MVV increased in patients aged ≤. 14. years, but decreased in older patients. Conclusions: The natural history of Morquio A syndrome is characterized by progressive impairment of endurance as measured by the 6MWT. Longitudinal trends in FVC and MVV showing increase in younger patients, but decrease in older patients, are likely to be influenced by growth. Changes in 6MWT may represent a sensitive measure of disease progression in ambulatory Morquio A patients. © 2014.


Cairoli R.,Niguarda Hospital | Cairoli R.,Valduce Hospital | Beghini A.,University of Milan | Turrini M.,Niguarda Hospital | And 11 more authors.
American Journal of Hematology | Year: 2013

Acute myeloid leukemia (AML) with deranged core-binding factor beta (CBFβ) is usually associated with a favorable prognosis with 50-70% of patients cured using contemporary treatments. We analyzed the prognostic significance of clinical features on 58 patients with CBFβ-AML aged ≤60 years. Increasing age was the only predictor for survival (P <0.001), with an optimal cut-point at 43 years. White blood cells (WBCs) at diagnosis emerged as an independent risk factor for relapse incidence (P = 0.017), with 1.1% increase of hazard for each 1.0 × 109/L WBC increment. KIT mutations lacked prognostic value for survival and showed only a trend for relapse incidence (P = 0.069). © 2013 Wiley Periodicals, Inc.


Aldenhoven M.,University Utrecht | Wynn R.F.,Royal Manchester Childrens Hospital | Orchard P.J.,University of Minnesota | O'Meara A.,Our Ladys Childrens Hospital | And 17 more authors.
Blood | Year: 2015

Mucopolysaccharidosis type I-Hurler syndrome (MPS-IH) is a lysosomal storage disease characterized by multisystem morbidity and death in early childhood. Although hematopoietic cell transplantation (HCT) has been performed in these patients for more than 30 years, large studies on the long-term outcome of patients with MPS-IH after HCT are lacking. The goal of this international study was to identify predictors of the long-term outcome of patients with MPS-IH after successful HCT. Two hundred seventeen patients with MPS-IH successfully engrafted with a median follow-up age of 9.2 years were included in this retrospective analysis. Primary endpoints were neurodevelopmental outcomes and growth. Secondary endpoints included neurologic, orthopedic, cardiac, respiratory, ophthalmologic, audiologic, and endocrinologic outcomes. Considerable residual disease burden was observed in the majority of the transplanted patients with MPS-IH, with high variability between patients. Preservation of cognitive function at HCT andayoungerageat transplantationweremajorpredictors for superior cognitivedevelopmentposttransplant. Anormal a-L-iduronidase enzyme level obtained post-HCT was another highly significant predictor for superior long-term outcome in most organ systems. The long-termprognosisofpatientswithMPS-IH receivingHCTcanbe improvedbyreducingtheage atHCTthroughearlierdiagnosis, aswell as using exclusively noncarrier donors and achieving complete donor chimerism. (Blood. 2015;125(13):2164-2172) © 2015 by The American Society of Hematology.


Arngrimsson R.,University of Iceland | Barbey F.,University of Lausanne | Cecchi F.,University of Florence | Deegan P.B.,University of Cambridge | And 24 more authors.
Orphanet Journal of Rare Diseases | Year: 2015

Introduction: Fabry disease (FD) is a lysosomal storage disorder resulting in progressive nervous system, kidney and heart disease. Enzyme replacement therapy (ERT) may halt or attenuate disease progression. Since administration is burdensome and expensive, appropriate use is mandatory. We aimed to define European consensus recommendations for the initiation and cessation of ERT in patients with FD. Methods: A Delphi procedure was conducted with an online survey (n∈=∈28) and a meeting (n∈=∈15). Patient organization representatives were present at the meeting to give their views. Recommendations were accepted with 75% agreement and no disagreement. Results: For classically affected males, consensus was achieved that ERT is recommended as soon as there are early clinical signs of kidney, heart or brain involvement, but may be considered in patients of 16 years in the absence of clinical signs or symptoms of organ involvement. Classically affected females and males with non-classical FD should be treated as soon as there are early clinical signs of kidney, heart or brain involvement, while treatment may be considered in females with non-classical FD with early clinical signs that are considered to be due to FD. Consensus was achieved that treatment should not be withheld from patients with severe renal insufficiency (GFR < 45 ml/min/1.73 m2) and from those on dialysis or with cognitive decline, but carefully considered on an individual basis. Stopping ERT may be considered in patients with end stage FD or other co-morbidities, leading to a life expectancy of <1 year. In those with cognitive decline of any cause, or lack of response for 1 year when the sole indication for ERT is neuropathic pain, stopping ERT may be considered. Also, in patients with end stage renal disease, without an option for renal transplantation, in combination with advanced heart failure (NYHA class IV), cessation of ERT should be considered. ERT in patients who are non-compliant or fail to attend regularly at visits should be stopped. Conclusion: The recommendations can be used as a benchmark for initiation and cessation of ERT, although final decisions should be made on an individual basis. Future collaborative efforts are needed for optimization of these recommendations. © 2015 Biegstraaten et al.; licensee BioMed Central.


PubMed | University of Turku, University of Zürich, The Childrens Memorial Health Institute, General Hospital Slovenj Gradec and 21 more.
Type: | Journal: Orphanet journal of rare diseases | Year: 2015

Fabry disease (FD) is a lysosomal storage disorder resulting in progressive nervous system, kidney and heart disease. Enzyme replacement therapy (ERT) may halt or attenuate disease progression. Since administration is burdensome and expensive, appropriate use is mandatory. We aimed to define European consensus recommendations for the initiation and cessation of ERT in patients with FD.A Delphi procedure was conducted with an online survey (n=28) and a meeting (n=15). Patient organization representatives were present at the meeting to give their views. Recommendations were accepted with 75% agreement and no disagreement.For classically affected males, consensus was achieved that ERT is recommended as soon as there are early clinical signs of kidney, heart or brain involvement, but may be considered in patients of 16years in the absence of clinical signs or symptoms of organ involvement. Classically affected females and males with non-classical FD should be treated as soon as there are early clinical signs of kidney, heart or brain involvement, while treatment may be considered in females with non-classical FD with early clinical signs that are considered to be due to FD. Consensus was achieved that treatment should not be withheld from patients with severe renal insufficiency (GFR < 45 ml/min/1.73m(2)) and from those on dialysis or with cognitive decline, but carefully considered on an individual basis. Stopping ERT may be considered in patients with end stage FD or other co-morbidities, leading to a life expectancy of <1year. In those with cognitive decline of any cause, or lack of response for 1year when the sole indication for ERT is neuropathic pain, stopping ERT may be considered. Also, in patients with end stage renal disease, without an option for renal transplantation, in combination with advanced heart failure (NYHA class IV), cessation of ERT should be considered. ERT in patients who are non-compliant or fail to attend regularly at visits should be stopped.The recommendations can be used as a benchmark for initiation and cessation of ERT, although final decisions should be made on an individual basis. Future collaborative efforts are needed for optimization of these recommendations.


Corrao G.,University of Milan Bicocca | Mazzola P.,University of Milan Bicocca | Monzio Compagnoni M.,University of Milan Bicocca | Rea F.,University of Milan Bicocca | And 5 more authors.
Drugs and Aging | Year: 2015

Objective: Our objective was to assess the relationship between antihypertensive drugs, loop diuretics, and the risk of hospitalization for hip fracture (HF). Design: We conducted a population-based study in a cohort of 81,617 patients from Lombardy (Italy) aged 70–90 years who were newly treated with antihypertensive agents or loop diuretics between 2005 and 2009. Cases were the 2153 patients who experienced the outcome (hospitalization for HF before 31 December 2012). For each case, up to three controls were randomly selected from the cohort to be matched for sex, age at cohort entry, and date of initial prescription. The case–control and case-crossover designs and the logistic regression for matched sets were used to measure the strength of the association between current use of an antihypertensive drug (within 30 days before the HF hospitalization) and the risk of outcome. Results: Case–control and case-crossover odds ratios (ORs) for current use of loop diuretics were 1.67 (95 % confidence interval [CI] 1.28–2.18) and 1.49 (95 % CI 1.05–2.10), respectively. Among patients aged 81–90 years, case–control and case-crossover ORs were 1.52 (95 % CI 1.04–2.21) and 1.82 (95 % CI 1.10–3.00) for current use of loop diuretics and 1.86 (95 % CI 1.03–3.35) and 1.88 (95 % CI 1.01–3.48) for α-blockers. No other agent was associated with the outcome. Conclusions: Evidence that loop diuretics and α-blockers are associated with a higher risk of HF was consistent in the two observational approaches. Clinicians should carefully consider the risk of falls in their selection of drugs for hypertension and in the clinical use of loop diuretics. © 2015, Springer International Publishing Switzerland.


Bellelli G.,University of Milan Bicocca | Bellelli G.,San Gerardo University Hospital | Bellelli G.,Geriatric Research Group | Guerini F.,Geriatric Research Group | And 5 more authors.
Aging Clinical and Experimental Research | Year: 2012

Early diagnosis of infections is often a challenge in older patients, since this age group may have atypical presentation. We report here the cases of 3 old patients, in whom a sudden decline in mobility status occurred shortly before the onset of the classically recognized features of infection. The decline was interpreted as an anticipatory marker of imminent infection. We discuss the possible implications of our findings and the opportunity that they offer to improve routine clinical practice in older patients. ©2012, Editrice Kurtis.


Pioli G.,ASMN IRCCS Hospital | Barone A.,Galliera Hospital | Mussi C.,University of Modena and Reggio Emilia | Tafaro L.,University of Rome La Sapienza | And 5 more authors.
Aging Clinical and Experimental Research | Year: 2014

This document is a Joint Position Statement by Gruppo Italiano di OrtoGeriatria (GIOG) supported by Società Italiana di Gerontologia e Geriatria (SIGG), and Associazione Italiana Psicogeriatria (AIP) on management of hip fracture older patients. Orthogeriatric care is at present the best model of care to improve results in older patients after hip fracture. The implementation of orthogeriatric model of care, based on the collaboration between orthopaedic surgeons and geriatricians, must take into account the local availability of resources and facilities and should be integrated into the local context. At the same time the programme must be based on the best available evidences and planned following accepted quality standards that ensure the efficacy of the intervention. The position paper focused on eight quality standards for the management of hip fracture older patients in orthogeriatric model of care. The GIOG promotes the development of a clinic database with the aim of obtaining a qualitative improvement in the management of hip fracture. © 2014, Springer International Publishing Switzerland.

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