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Drafting manuscript: KEE. Critical review and final approval of manuscript content: KEE, NP, JAC, YS, TAH, BCT, MS, and SRC. Statistical analysis: NP performed the statistical analyses and is independent of any commercial funder. She had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analyses. Critical review and final approval of manuscript content: AI. ""Paget disease of bone (PDB) is a common disorder characterized by increased bone turnover at one of more sites throughout the skeleton. Genetic factors play an important role in the pathogenesis of PDB, and the most important http://www.selleckchem.com/products/azd9291.html predisposing gene is SQSTM1, which is mutated in about 10% of patients. Here we investigated the relationship between SQSTM1 mutation status, disease severity, and clinical outcome in 737 patients who took part in a randomized study of two different management strategies for the disease. Mutations of SQSTM1 were detected in 80 of 737 (10.9%) patients. Mutation carriers had an earlier age at diagnosis (59.4 ��11.5 versus 65.0?��?10.4 years, p? http://www.selleck.cn/products/Everolimus(RAD001).html number of affected bones (3.2?��?1.2 versus 2.1?��?1.2, p? http://www.selleckchem.com/products/ch5424802.html these complications. ? 2010 American Society for Bone and Mineral Research. Paget disease of bone (PDB) is a common condition characterized by increased and disorganized bone remodeling affecting one or more sites throughout the skeleton. The abnormal remodeling processes disrupts normal bone architecture and can lead to the development of several complications, including bone pain, deformity, secondary osteoarthritis, nerve compression syndromes, and pathologic fractures.1 Some patients with PDB have severe disease that has a major impact on quality of life,2, 3 but others are completely asymptomatic.1 The determinants of clinical severity of PDB are poorly understood. Both genetic and environmental factors are thought to be involved in the pathogenesis of PDB.