Estimating the clinical prevalence of Wilson’s disease in the UK

Background and AimThe clinical prevalence of Wilson’s disease (WD) in the UK remains unknown. The estimated genetic prevalence in the UK, 142/million, is higher than the clinical prevalence (15/million) reported in other European studies. The aim of this study was to estimate the clinical prevalence of WD utilising readily available laboratory and clinical data.MethodPatients with WD who attended Nottingham University Hospital NHS Trust (NUH) between 2011 and 2018 were identified using multiple sources of case ascertainment: serum ceruloplasmin, 24-hour urinary copper, ‘Wilson’ in liver biopsy report, hospital prescription for penicillamine/trientine/zinc and admission coded with ICD-10 Code E83.0 (disorder of copper metabolism). Potential cases were identified using the Leipzig score, diagnosis was confirmed in hospital records and the point prevalence was calculated using the Office for National Statistics mid-2017 population estimates.ResultsA total of 1,794 patients were identified from ≥1 source; 19 patients had WD, of which 11 were from within the study catchment area and alive at the time of point prevalence estimation. Twentynine patients had Leipzig score ≥2 without a diagnosis of WD, but none had WD on screening (n=16). The overall prevalence of WD was 15.5/million; males 16.9/million and females 14.1/million.ConclusionThis is the first UK population-based study of WD clinical prevalence. This is lower than the UK genetic prevalence, but comparable to European clinical prevalence. The case ascertainment approach used in this study may be a cost-effective, and similar practises could be adopted nationally

An Assessment of the UK’s Trade with Developing Countries under the Generalised System of Preferences

The European Union (EU) Generalised System of Preferences (GSP Scheme) grants preferential treatment to 88 eligible countries. There are, however, concerns that the restrictive features (such as Rules of Origin, Low Preference Margin and Low Coverage) of the existing scheme indicate gravitation towards commercial trade agenda to which efficiency imperatives appear subordinated. Whether these concerns are genuine is an empirical question whose answer largely determines whether, after Brexit, the UK continues with the existing specifics of the EU scheme or develops a more inclusive UK-specific GSP framework. This study quantitatively examines the efficiency of the EU GSP as it relates to UK beneficiaries from 2014 to 2017. We draw on the descriptive efficiency estimation (The utilisation Rate, Potential Coverage Rate, and the Utility Rate) using import data across 88 beneficiary countries and agricultural products of the Harmonised System Code Chapter 1 to 24. Asides the Rules of Origin that, generally, harm the uptake of GSP, low preference margin is found to cause low utilisation rates in a non-linear manner. Essentially, a more robust option (such that allows “global Cumulation” or broader product coverage) could, substantially, lower the existing barriers to trade and upsurge the efficiency of the GSP scheme

Progress toward standardized diagnosis of vascular cognitive impairment: Guidelines from the Vascular Impairment of Cognition Classification Consensus Study

INTRODUCTION: Progress in understanding and management of vascular cognitive impairment (VCI) has been hampered by lack of consensus on diagnosis, reflecting the use of multiple different assessment protocols. A large multinational group of clinicians and researchers participated in a two-phase Vascular Impairment of Cognition Classification Consensus Study (VICCCS) to agree on principles (VICCCS-1) and protocols (VICCCS-2) for diagnosis of VCI. We present VICCCS-2. METHODS: We used VICCCS-1 principles and published diagnostic guidelines as points of reference for an online Delphi survey aimed at achieving consensus on clinical diagnosis of VCI. RESULTS: Six survey rounds comprising 65-79 participants agreed guidelines for diagnosis of VICCCS-revised mild and major forms of VCI and endorsed the National Institute of Neurological Disorders-Canadian Stroke Network neuropsychological assessment protocols and recommendations for imaging. DISCUSSION: The VICCCS-2 suggests standardized use of the National Institute of Neurological Disorders-Canadian Stroke Network recommendations on neuropsychological and imaging assessment for diagnosis of VCI so as to promote research collaboration

Crime analytics : analysis of crimes through newspaper articles

Crime analysis is one of the most important activities of the majority of the intelligent and law enforcement organizations all over the world. Generally they collect domestic and foreign crime related data (intelligence) to prevent future attacks and utilize a limited number of law enforcement resources in an optimum manner. A major challenge faced by most of the law enforcement and intelligence organizations is efficiently and accurately analyzing the growing volumes of crime related data. The vast geographical diversity and the complexity of crime patterns have made the analyzing and recording of crime data more difficult. Data mining is a powerful tool that can be used effectively for analyzing large databases and deriving important analytical results. This paper presents an intelligent crime analysis system which is designed to overcome the above mentioned problems. The proposed system is a web-based system which comprises of crime analysis techniques such as hotspot detection, crime comparison and crime pattern visualization. The proposed system consists of a rich and simplified environment that can be used effectively for processes of crime analysis

Relevance of heat stress and dehydration to chronic kidney disease (CKDu) in Sri Lanka.

Chronic kidney disease in the absence of hypertension and diabetes is a growing problem among agricultural laborers in tropical and subtropical regions. It is unclear if heat stress and dehydration are risk factors for this form of chronic kidney disease (CKDu). To investigate this relationship, agricultural workers in four villages (n = 261) in North Central Province, Sri Lanka completed the US National Institute for Occupational Safety and Health (NIOSH) health hazard evaluation of heat stress, translated into Sinhalese (July 2017). We constructed a heat stress/dehydration index based on the frequency of 16 symptoms (range 0-32; reliability, 0.84). Workers provided a urine sample for dipstick assessment of urine albumin-creatinine ratio (ACR) and refractometer analysis of urine concentration. Of 261 respondents, 41 participants reported diabetes or chronic kidney disease. They scored higher on the heat stress-dehydration index (10.78 vs. 8.03, p < .01) and were more likely to have ACR > 30 (85.4% vs. 69.4%, p < .05). Among 216 non-pregnant agricultural workers without diabetes or kidney disease (mean age, 46.6; 37% male), villagers in the high-CKDu prevalence area were more likely to show signs of dehydration (for example, greater urine concentration, 1.015 vs. 1.012, p < .05, among males); however, the heat stress-dehydration index overall was not associated with ACR or urine concentration. Because an elevated ACR (proteinuria) is not a reliable marker of early CKDu, additional studies are needed to assess the association between heat stress-dehydration symptoms and risk of CKDu