Scoliosis is a complication of fibrous dysplasia/McCune-Albright syndrome (FD/MAS); however, risk factors and long-term outcomes are unknown. Bisphosphonates are commonly used; however, it is unknown whether their use decrease the risk of progressive scoliosis. Clinical data from the National Institutes of Health (NIH) cohort study was reviewed. Cobb angles were measured, and variables associated with scoliosis progression were identified. Of 138 subjects with available radiographs, 84 (61%) had scoliosis, including 55 (65%) classified as mild (Cobb angle >10 to ≤30 degrees), 11 (13%) as moderate (>30 to ≤45 degrees), and 18 (22%) as severe (>45 degrees). Total skeletal disease burden was highly associated with scoliosis severity (p < 0.0001). Endocrinopathies associated with scoliosis included fibroblast growth factor 23 (FGF23)-mediated hypophosphatemia (p < 0.001) and hyperthyroidism (p < 0.001). Bone turnover markers, including osteocalcin and NTX-telopeptides, were associated with severe scoliosis (p < 0.01). Associations were identified between Cobb angle and functional metrics, including leg length discrepancy (p < 0.01), hip range of motion (p < 0.05), and strength of the gluteus medius and maximus (p < 0.01). Longitudinal analyses were conducted in 69 subjects who had serial radiographs over a median 4.9-year period (range, 0.9 to 14.7 years). Twenty-two subjects were treated with bisphosphonates; there was no difference in Cobb angle progression compared to untreated subjects (0.10 versus 0.53 degrees/year, p = 0.36). Longitudinal data was available for 10 of 12 subjects treated with spinal fusion; one had instrumentation failure, but in nine subjects Cobb angles were stable with 6.1 years of follow-up (range, 0.9 to 14.7 years). Two fatalities from scoliosis-associated restrictive lung disease occurred in subjects managed non-operatively. Scoliosis occurs frequently in patients with polyostotic FD, and may be potentially fatal. The primary risk factor for progressive scoliosis is total skeletal disease burden. Treatable features that contribute to scoliosis progression include leg length discrepancy, FGF23-mediated hypophosphatemia, and hyperthyroidism. Current data do not support routine use of bisphosphonates to prevent progression of spinal curvature. Spinal fusion is frequently effective in providing long-term stability, and may be lifesaving. Published 2018. This article is a U.S. Government work and is in the public domain in the USA.

Purpose Although organizational visions can guide everyday work, little is known about how visions relating to non-academic goals, such as social justice, might be integrated into educators’ technology practices. The purpose of this paper is to describe and analyze one school’s 1:1 iPad initiative, including the potential role played by the school’s social justice mission. Design/methodology/approach This mixed-methods case study drew upon data collected in a 1:1 school enrolling approximately 1,500 students. In total, approximately 138 educators responded to the online survey, and 18 educators participated in interviews. Social network analysis (SNA) techniques (i.e. faction analysis) and analysis of variance helped to describe educators’ instructional practices and attitudes involving iPads, including the extent to which devices were used in alignment with the school’s mission and vision. Findings Lacking a centralizing core of actors, the school was found to be divided into nine distinct, cohesive subgroups (i.e. factions). Statistically significant differences were found among these communities of discourse. Leaders’ lack of centrality in school change, especially as it related to helping teachers envision ways to connect mission with practices, may have hindered technology integration and instructional innovation. Originality/value Whereas prior research has described the centralizing role leaders may play in 1:1 initiatives, this study demonstrates how a lack of centralized leadership structures may adversely impact a sense of mission, and ultimately, technology integration. Moreover, this study advances the use of SNA methodologies in studies of leadership, especially the use of latent, underlying communities of discourse as categories for further analysis. As such, the authors discuss recommendations for leaders regarding the development of cohesion around issues of mission, vision and technology integration. Further, the authors point toward ways in which scholars might conceptualize about technologically supported educational change.

PurposeApplications for tracking and managing classroom behavior have become increasingly commonplace, thus making it possible to incorporate nonacademic data into collaborative problem-solving and school improvement. Whether or how these platforms might support such aims, however, is not known. Accordingly, this study explores practices involving these applications, focusing especially on problem-solving among educators and with students' families.Design/methodology/approachThis comparative case study took place in three schools. In total, 34 semistructured interviews were conducted with teachers and school leaders. Analysis included qualitative coding as well as the development of within- and cross-case summaries.FindingsSchools varied greatly when it came to using behavior management platforms as a part of problem-solving. At a basic level, it was not uncommon for educators to use behavioral data for classroom troubleshooting or check-ins with students and transactional communications with families. However, only two schools attempted to use behavioral data for more systemic, “big picture” problem-solving, such as to make discipline policies more equitable or to improve teacher practices. The richness of collaboration with families seemed especially shaped by how and how frequently data were shared (e.g. automated notifications and paper printouts).Originality/valueEmpirical research about behavior management applications has been limited and focused only at the classroom level. The present study contributes new knowledge about the school-level implications of these platforms, while also expanding conversations about how behavioral data may be incorporated into data-informed problem-solving. Implications for leadership and theory are also discussed.