The Case | Ectopic calcifications in a child

The case A nine year old boy, born of third degree consanguinity, presented with a hard left scapular swelling for 6 months. There was no history of fever, trauma or weight loss. There was no pain, redness or discharge. This mass was excised but recurred over the next 6 months (Left panel of Figure 1). A similar swelling had been excised from the left knee one year before presentation, but had recurred over 6 months. Family history was negative for similar lesions. Development was normal for age and he had no other medical problems. On examination, pulse was 84 per minute, blood pressure 96/68 mmHg, weight 22 kg and height 122 cm (both between 10th and 25th percentile). A 6 × 4 cm mass was noted in the right scapular region and a 3 × 3 cm mass at the lateral aspect of the left knee. Both of these masses were firm to hard, globular, nontender, and fixed to the bone. The overlying skin was without erythema or local warmth, though scars from the previous resections were noted. Lymphadenopathy was absent. The rest of the examination was unremarkable. Radiographs of the knee showed a lobular, inhomogenously but densely calcified lesion in the anterolateral region of the left knee (Right panel of Figure 1). Serum calcium was 9.

X-linked hypophosphatemia caused by the prevailing North American PHEX variant c.*231A\u3eG; exon 13–15 duplication is often misdiagnosed as ankylosing spondylitis and manifests in both men and women

Inactivating mutations of the gene coding for phosphate-regulating endopeptidase homolog X-linked (PHEX) cause X-linked hypophosphatemia (XLH). A nove

Engaging Teachers in the Reflective Process: How one Massachusetts School District Facilitates and Sustains Teacher Growth

Thesis advisor: Lauri JohnsonThis qualitative case study examined the perceptions of teachers and leaders in one Massachusetts school district about the actions of school leaders that engaged teachers in the reflective process. Utilizing Lave and Wenger's (1991) "Communities of Practice" as a framework enabled the researcher to examine the extent to which a social process of learning existed and whether the district's current structures and conditions engaged teachers in the reflective process. The findings from this study drew upon interview, observation, and document data to explore teacher and leader views regarding specific leadership actions (or inactions) that supported and facilitated teacher engagement in the examination of their practice and whether teachers viewed those actions as impacting their ability to reflect. Results indicated that school leaders implemented anticipatory and ongoing actions to create necessary preconditions, which fostered teacher reflection. Data revealed a district belief that reflective dialogue in pairs (teacher to teacher or teacher to administrator) or in groups (faculty meetings, grade level or departmental meeting) was important to educator growth. Administrators' actions, which engaged teachers in a reflective process, were perceived to be those associated with being an instructional leader. Recommendations include helping district leaders understand the role and design of collaborative reflection as well as the role of the principal in order to plan and implement effective professional learning, which develops reflective practitioners.Thesis (EdD) — Boston College, 2014.Submitted to: Boston College. Lynch School of Education.Discipline: Educational Leadership and Higher Education

New Teacher Support: How One Massachusetts School District Facilitates and Sustains Teacher Growth

Thesis advisor: Lauri JohnsonThis qualitative case study examined the structures and conditions in one Massachusetts school district that supported new teachers. Data was gathered from semi-structured interviews with teachers and administrators, artifact analyses and participant observations of district meetings. While the existing literature on new teacher support focuses on mentoring as the primary means of induction, study results indicated that the district supported new teachers through building a collaborative atmosphere where new teachers regularly meet with veteran teachers in their subject area and grade level; weekly meetings with mentor coordinators; and strong principal support. However, new teachers were not formally assigned a mentor, which can leave them without a primary support person. Recommendations are made to formalize the mentoring program, to provide a mentor coordinator for each building, and to involve principals more formally in the induction program.Thesis (EdD) — Boston College, 2014.Submitted to: Boston College. Lynch School of Education.Discipline: Educational Leadership and Higher Education

Feedback: How one Massachusetts School District Facilitates and Sustains Teacher Growth

Thesis advisor: Lauri JohnsonThis qualitative case study examined teacher and administrator perceptions of how one Massachusetts school district used the feedback processes to facilitate teacher growth and development. Feedback was defined as any type of information about performance or progress towards a goal that is transferred from one individual or group to another individual or group. Data gathered from participant interviews, artifact analyses, and observations of district meetings found not only effective distribution and use of educator feedback, but also that teachers and administrators participated in feedback-seeking behavior. The district appeared to be successful in embedding a social learning culture that facilitated and conditioned the positive use of feedback as an activator for ongoing examination of teaching and learning as well as the development and progress monitoring of individual and collective district improvement goals.Thesis (EdD) — Boston College, 2014.Submitted to: Boston College. Lynch School of Education.Discipline: Educational Leadership and Higher Education

Building Leadership Capacity: How One Massachusetts School District Facilitates and Sustains Teacher Growth

Thesis advisor: Lauri JohnsonDistrict leadership has been found to have a measurable effect on student achievement by creating conditions within which teachers and administrators frame their daily work with children. The superintendent is uniquely poised to build the needed infrastructure of support and assure its alignment with the philosophy and mission of the district and, in turn, with the work of the school. By attending to the habits and conditions that allow a staff to work as a unit, superintendents are able to contribute to the development of a community of professional learners within and among district schools. This qualitative case study analyzed district leadership practices that support ongoing teacher growth in a Massachusetts school district by examining the work of the superintendent and the impact of his leadership on the ongoing development of a community of professional learners at the district and school level. Data included interviews with teachers and administrators, artifact analyses, and observations of district meetings. Findings reveal the superintendent's use of a PLC process to model and provide support to school-level leaders by encouraging broad-based participation in the skillful work of leadership; establishing a clear vision which resulted in program coherence; fostering a system of inquiry-based accountability that informed decision making and practice; and nurturing organizational relationships that involved high district engagement and low bureaucratization which supported school-based collaborative teacher growth.Thesis (EdD) — Boston College, 2014.Submitted to: Boston College. Lynch School of Education.Discipline: Educational Leadership and Higher Education

Sustained Efficacy and Safety of Burosumab, a Monoclonal Antibody to FGF23, in Children With X-Linked Hypophosphatemia

PURPOSE: In X-linked hypophosphatemia (XLH), excess fibroblast growth factor-23 causes hypophosphatemia and low calcitriol, leading to musculoskeletal disease with clinical consequences. XLH treatment options include conventional oral phosphate with active vitamin D, or monotherapy with burosumab, a monoclonal antibody approved to treat children and adults with XLH. We have previously reported outcomes up to 64 weeks, and here we report safety and efficacy follow-up results up to 160 weeks from an open-label, multicenter, randomized, dose-finding trial of burosumab for 5- to 12-year-old children with XLH. METHODS: After 1 week of conventional therapy washout, patients were randomized 1:1 to burosumab every 2 weeks (Q2W) or every 4 weeks (Q4W) for 64 weeks, with dosing titrated based on fasting serum phosphorus levels between baseline and week 16. From week 66 to week 160, all patients received Q2W burosumab. RESULTS: Twenty-six children were randomized initially into each Q2W and Q4W group and all completed treatment to week 160. In 41 children with open distal femoral and proximal tibial growth plates (from both treatment groups), total Rickets Severity Score significantly decreased by 0.9 ± 0.1 (least squares mean ± SE; P < 0.0001) from baseline to week 160. Fasting serum phosphorus increases were sustained by burosumab therapy throughout the study, with an overall population mean (SD) of 3.35 (0.39) mg/dL, within the pediatric normal range (3.2-6.1 mg/dL) at week 160 (mean change from baseline P < 0.0001). Most adverse events were mild to moderate in severity. MAIN CONCLUSIONS: In children with XLH, burosumab administration for 160 weeks improved phosphate homeostasis and rickets and was well-tolerated. Long-term safety was consistent with the reported safety profile of burosumab. CLINICALTRIALS.GOV: NCT0216357

Cooperative learning in the first year of undergraduate medical education

<p>Abstract</p> <p>Background</p> <p>Despite extensive research data indicating that cooperative learning promotes higher achievement, the creation of positive relationships, and greater psychological health for students at all levels in their education, cooperative learning as a teaching strategy is still underutilized in undergraduate medical education.</p> <p>Methods</p> <p>A cooperative learning task was introduced as part of the mandatory first Year undergraduate Pathology course. The task was to create an 8.5" × 11" poster summary of pre-assigned content in self-chosen groups of four or five students. On the designated "Poster Day," the posters were displayed and evaluated by the students using a group product evaluation. Students also completed an individual group process reflection survey. An objective evaluation of their understanding was gauged at the midterm examination by specific content-related questions.</p> <p>Results</p> <p>Majority (91–96%) of students judged the group products to be relevant, effective, easy-to-understand, and clearly communicated. The majority of the students (90–100%) agreed that their group process skills of time management, task collaboration, decision-making and task execution were effective in completing this exercise. This activity created a dynamic learning environment as was reflected in the students' positive, professional discussion, and evaluation of their posters. The content-related questions on the midterm examination were answered correctly by 70–92% of the students. This was a mutually enriching experience for the instructor and students.</p> <p>Conclusion</p> <p>These findings demonstrate that cooperative learning as a teaching strategy can be effectively incorporated to address both content <it>and </it>interpersonal skill development in the early years of undergraduate medical education.</p

Mapping the categories of the Swedish primary health care version of ICD-10 to SNOMED CT concepts: Rule development and intercoder reliability in a mapping trial

<p>Abstract</p> <p>Background</p> <p>Terminologies and classifications are used for different purposes and have different structures and content. Linking or mapping terminologies and classifications has been pointed out as a possible way to achieve various aims as well as to attain additional advantages in describing and documenting health care data.</p> <p>The objectives of this study were:</p> <p>• to explore and develop rules to be used in a mapping process</p> <p>• to evaluate intercoder reliability and the assessed degree of concordance when the 'Swedish primary health care version of the International Classification of Diseases version 10' (ICD-10) is matched to the Systematized Nomenclature of Medicine, Clinical Terms (SNOMED CT)</p> <p>• to describe characteristics in the coding systems that are related to obstacles to high quality mapping.</p> <p>Methods</p> <p>Mapping (interpretation, matching, assessment and rule development) was done by two coders. The Swedish primary health care version of ICD-10 with 972 codes was randomly divided into an allotment of three sets of categories, used in three mapping sequences, A, B and C. Mapping was done independently by the coders and new rules were developed between the sequences. Intercoder reliability was measured by comparing the results after each set. The extent of matching was assessed as either 'partly' or 'completely concordant'</p> <p>Results</p> <p>General principles for mapping were outlined before the first sequence, A. New mapping rules had significant impact on the results between sequences A - B (p < 0.01) and A - C (p < 0.001). The intercoder reliability in our study reached 83%. Obstacles to high quality mapping were mainly a lack of agreement by the coders due to structural and content factors in SNOMED CT and in the current ICD-10 version. The predominant reasons for this were difficulties in interpreting the meaning of the categories in the current ICD-10 version, and the presence of many related concepts in SNOMED CT.</p> <p>Conclusion</p> <p>Mapping from ICD-10-categories to SNOMED CT needs clear and extensive rules. It is possible to reach high intercoder reliability in mapping from ICD-10-categories to SNOMED CT. However, several obstacles to high quality mapping remain due to structure and content characteristics in both coding systems.</p