The relationship between social deprivation and the quality of primary care: a national survey using indicators from the UK Quality and Outcomes Framework

BACKGROUND: The existence of health inequalities between least and most socially deprived areas is now well established. AIM: To use Quality and Outcomes Framework (QOF) indicators to explore the characteristics of primary care in deprived communities. DESIGN OF STUDY: Two-year study. SETTING: Primary care in England. METHOD: QOF data were obtained for each practice in England in 2004-2005 and 2005-2006 and linked with census derived social deprivation data (Index of Multiple Deprivation scores 2004), national urbanicity scores and a database of practice characteristics. Data were available for 8480 practices in 2004-2005 and 8264 practices in 2005-2006. Comparisons were made between practices in the least and most deprived quintiles. RESULTS: The difference in mean total QOF score between practices in least and most deprived quintiles was 64.5 points in 2004-2005 (mean score, all practices, 959.9) and 30.4 in 2005-2006 (mean, 1012.6). In 2005-2006, the QOF indicators displaying the largest differences between least and most deprived quintiles were: recall of patients not attending appointments for injectable neuroleptics (79 versus 58%, respectively), practices opening > or =45 hours/week (90 versus 74%), practices conducting > or = 12 significant event audits in previous 3 years (93 versus 81%), proportion of epileptics who were seizure free > or = 12 months (77 versus 65%) and proportion of patients taking lithium with serum lithium within therapeutic range (90 versus 78%). Geographical differences were less in group and training practices. CONCLUSIONS: Overall differences between primary care quality indicators in deprived and prosperous communities were small. However, shortfalls in specific indicators, both clinical and non-clinical, suggest that focused interventions could be applied to improve the quality of primary care in deprived areas.     

Complex care of individuals with multiple sulfatase deficiency: Clinical cases and consensus statement

Multiple sulfatase deficiency (MSD) is an ultra-rare neurodegenerative disorder that results in defective sulfatase post-translational modification. Sulfatases in the body are activated by a unique protein, formylglycine-generating enzyme (FGE) that is encoded by SUMF1. When FGE is absent or insufficient, all 17 known human sulfatases are affected, including the enzymes associated with metachromatic leukodystrophy (MLD), several mucopolysaccharidoses (MPS II, IIIA, IIID, IVA, VI), chondrodysplasia punctata, and X-linked ichthyosis. As such, individuals demonstrate a complex and severe clinical phenotype that has not been fully characterized to date. In this report, we describe two individuals with distinct clinical presentations of MSD. Also, we detail a comprehensive systems-based approach to the management of individuals with MSD, from the initial diagnostic evaluation to unique multisystem issues and potential management options. As there have been no natural history studies to date, the recommendations within this report are based on published studies and consensus opinion and underscore the need for future research on evidence-based outcomes to improve management of children with MSD.