Activated phosphoinositide 3-kinase (PI3K) δ Syndrome (APDS), caused by autosomal dominant mutations in PIK3CD (APDS1) or PIK3R1 (APDS2), is a heterogeneous primary immunodeficiency. While initial cohort-descriptions summarized the spectrum of clinical and immunological manifestations, questions about long-term disease evolution and response to therapy remain. The prospective European Society for Immunodeficiencies (ESID)-APDS registry aims to characterize the disease course, identify outcome predictors, and evaluate treatment responses. So far, 77 patients have been recruited (51 APDS1, 26 APDS2). Analysis of disease evolution in the first 68 patients pinpoints the early occurrence of recurrent respiratory infections followed by chronic lymphoproliferation, gastrointestinal manifestations, and cytopenias. Although most manifestations occur by age 15, adult-onset and asymptomatic courses were documented. Bronchiectasis was observed in 24/40 APDS1 patients who received a CT-scan compared with 4/15 APDS2 patients. By age 20, half of the patients had received at least one immunosuppressant, but 2–3 lines of immunosuppressive therapy were not unusual before age 10. Response to rapamycin was rated by physician visual analog scale as good in 10, moderate in 9, and poor in 7. Lymphoproliferation showed the best response (8 complete, 11 partial, 6 no remission), while bowel inflammation (3 complete, 3 partial, 9 no remission) and cytopenia (3 complete, 2 partial, 9 no remission) responded less well. Hence, non-lymphoproliferative manifestations should be a key target for novel therapies. This report from the ESID-APDS registry provides comprehensive baseline documentation for a growing cohort that will be followed prospectively to establish prognostic factors and identify patients for treatment studies.

Abstract:Two departments in a secondary school in England participated in 'lesson study' projects over a five-month period to explore its usefulness as a vehicle for professional development. Through a cycle of two research lessons, conducted separately in each department, teachers identified challenges that inhibited the learning of their students and collaboratively prepared innovative approaches to address the learning challenges. The process yielded multiple sets of data: DVD-recorded lessons, lesson plans and resources, transcripts of preparation and evaluation meetings and individual interviews at the end of the project. This paper draws principally on detailed qualitative analysis of end-of-project interviews about teacher experiences of learning and practice development in the two contexts. While the teachers encountered some logistical challenges to the implementation of lesson study, a number of important gains were reported: collaboration in lesson study reduced feelings of professional isolation; teachers reported a sharper focus on pupil learning and more confidence to take risks with approaches to teaching, which led to greater opportunities for pupils to engage in interactive activities, for example involving problem-solving and peer teaching in groups.Keywords: secondary school, lesson study, collaboration, observation of learning, teacher development IntroductionThe purpose of lesson study is improvement in the quality of teaching through a 'reflexive, recursive and collaborative' process (Dudley 2011, 5). Translated from the term 'kenkyu jugyou' meaning lesson study, the process originates from Japan where it has been widely used since the 1950s (Abiko 2011). A lesson study cycle involves small groups of teachers collaboratively planning a 'research lesson' that is taught by one member of the group while the others observe one or two 'case' pupils (Dudley 2011). The lesson is then evaluated in light of the reflections of the teacher and the insights of those who observed their respective case pupils. Subsequently, the lesson may be revised for further teaching with other classes (Hiebert and Stigler 2000). What distinguishes this from other forms of professional development is the planning of jointly conceived research lessons to address particular problems with learning, rather than focusing on the performance of an individual teacher.The process requires an investment of time and patience: 'Lesson study is a process of improvement that is expected to produce small, incremental improvements in teaching over long periods of time ' (Stigler and Hiebert 1999, 121). Consequently, it may not be attractive 2 in school systems that are hungry for quick-fix impacts, for example on examination results. Despite this, its use is growing and ever larger numbers of research investigations have explored its effectiveness in different contexts (Lewis, Perry and Murata 2006;Fernandez 2002;Fernandez, Cannon and Chokshi 2003;Lawrence and Chong 2010;Dudley , 2013). According to Lewis (2004, 19), teachers in Japa...

Abstract'Lesson Study' (LS) case studies were conducted in two secondary school teaching practice placements in England. Using Dudley's framework (2011), Geography and Modern Languages trainees and school-based colleagues collaboratively planned a 'research lesson'.This was taught by the mentor while the trainee and other teachers observed the learning of three 'focus' students. The lesson was reviewed and revised for teaching to a parallel group by the trainee and the cycle of observation and evaluation was repeated. In post-lesson study interviews, analysed from a Communities of Practice perspective, mentors claimed that LS facilitated rapid integration of the prospective teacher into departmental working practices while trainees claimed they benefited from the team approach inherent in LS. The process enabled participants to explore collaboratively the 'pedagogic black-box' enriching the experience and learning of both trainees and mentors. Successfully integrated, LS improves support for teacher development in teaching practice placements.

Lesson study is recommended to schools as a powerful approach to the development of teacher learning. This paper reports the outcomes of a 'Lesson Study' project conducted in a Mathematics department with four serving teachers in a secondary school in England. Using Dudley's lesson study framework and, drawing on Hargreaves and Fullan's notion of professional capital, the feasibility and value of collaborative lesson study as a vehicle for the development of teacher learning was explored. Planning and evaluation meetings as well as end-of-project interviews were analysed to investigate how teachers planned research lessons together and how these were evaluated. Despite time constraints, teachers who engaged in lesson study reported that the process improved understanding of their students, that collaboration helped them to develop less-teacher-centred approaches and created a stronger sense of teacher community. The project demonstrated that lesson study has potential as an alternative or complementary model of teachers' learning, but it also throws up substantive organisational challenges if its use is to expand.

Abstract• Purpose -A structured review of literature on Lesson Study (LS) in initial teacher education (ITE) was conducted. The focus was on how learning and observation were discussed in studies of LS in ITE.• Design -Each national team (in Norway and England) undertook independent searches of published peer-reviewed articles. The resulting articles were then combined, screened and collaboratively reviewed, the focus being on two areas of enquiry: 1) How learning is represented and discussed;2) The extent to which observation is described and used to capture evidence of learning.• Findings -The literature review indicated that there was no universally held understanding of, or explanation for, the process of observation, how it should be conducted, and who or what should be the principal focus of attention. There was also a lack of clarity in the definition of learning and the use of learning theory to support these observations.• Research limitations -This study was limited to a review of a selection of peerreviewed journal articles, published in English. It arrives at some tentative conclusions, but its scope could have been broadened to include more articles and other types of published material e.g. theses and book chapters.• Practical implications -Research that investigates the use of LS in ITE needs to be more explicit about how learning is defined and observed. Furthermore, LS research papers need to assure greater clarity and transparency about how observations are conducted in their studies. 2• Originality/Value -This literature review suggests that discussion of both learning and observation in ITE lesson study research papers should be strengthened. The review highlights three principal challenges that ITE LS researchers should consider: a) how to prepare student-teachers to observe (professional noticing being a promising option), b) the wide variation in the focus of classroom observation in ITE lesson studies c) discussion of what is understood by learning needs to stand at the heart of preparation for lesson studies in ITE.