Natural history of Upshaw-Schulman syndrome based on ADAMTS13 gene analysis in Japan

Upshaw–Schulman syndrome (USS) is an extremely rare hereditary deficiency of ADAMTS13 activity, termed congenital TTP. The clinical signs are usually mild during childhood, often with isolated thrombocytopenia. But their symptoms become more evident when patients have infections or get pregnant. We identified 43 USS-patients in Japan, who ranged in age from early childhood to 79 years of age. Analysing the natural history of these USS patients based on ADAMTS13 gene mutations may help characterise their clinical phenotypes. Severe neonatal jaundice that requires exchange blood transfusion, a hallmark of USS, was found in 18 of 43 patients (42%). During childhood, 25 of 43 patients were correctly diagnosed with USS without gender disparity. These 25 patients were categorised as having ‘the early-onset phenotype’. Between 15 and 45 years of age, 15 were correctly diagnosed, and, interestingly, they were all female. The remaining three patients were male and were diagnosed when they were older than 45 years of age, suggesting that they were ‘the late-onset phenotype’. Two of these three males developed sudden overt TTP when they were 55 and 63 years old, respectively. These two men had two different homozygous ADAMTS13 gene mutations, p.R193W/p.R193W and p.C1024R/p.C1024R, respectively. Both of which were not discovered in the US or Western countries. In vitro expression studies showed that these two proteins were consistently secreted into the culture medium but to a lesser extent and with reduced activity compared to the wild-type protein. Our results indicate that ‘the late-onset phenotype’ of USS is formed with ethnic specificity.・The definitive version is available at " http://dx.doi.org/10.1111/j.1538-7836.2011.04341.x "・State of the Art 2011 : XXIII Congress of the International Society on Thrombosis and Haemostasis Invited Reviewhttp://dx.doi.org/10.1111/j.1538-7836.2011.04341.

An annual report 2014 on campus wide Faculty Development programs at The University of Tokushima

FD promotion programs have started from 2002, promoting systematization, organization and routinization of Faculty Development. In 2014, based on the results of 12 years of FD, we have aimed to enhance the quality of higher education of our university, building new educational system for assurance of higher education. The Center for the Enhancement of Teaching & Learning was restructured into the Center of University Education. Thus, based on the guideline for educational innovation of our university, the organization for promoting FD was strengthened. Concretely, we carried out 3 programs; 1) educational innovation FD, 2) educational development FD, 3) comprehensive FD. As to each program, we showed their outlines and considered their results and problems shown from the questionnaire to their attendants

FD ファシリテーター ヨウセイ ケンシュウ ノ コウカ ニ カンスル イチコウサツ : トクシマ ダイガク ニオケル FD ファシリテーター ノ ヤクワリ ト イギ

徳島大学では2002年度より教育革新FDプログラムが立ち上った。現在までに多様な全学FDプログラムが開発・実施されてきている。その中でも部局FDの組織化を目指した研修プログラム として部局FDを実質的に担当する教員を養成するためのFDファシリテーター養成研修を実施している。2008年度からは部局FD組織が実質的に機能し始め、全学的なFD実施組織と部局FDの連携が活発に実施されるようになりつつある。部局FDを推進する担い手となるFDファシリテーターの役割や存在意義は部局FD組織にとって、ますます重要なものになりつつあると考えられる。 FDファシリテーターはFD組織の発達にも関わるコアメンバーであり、実質的なFDの実践を続けていくためには、FDの専門性に加え、組織に関わる正統的成員(1)としての高い当事者意識が必要である。本研究の目的は、徳島大学におけるFDファシリテーターの役割とその意義に注目し、FDファシリテーター養成研修でのアンケート調査をもとに、効果検証を試みるものである。特に、2009年度の研修については、FDファシリテーターにとって重要な知識やスキルの獲得と、FDファシリテーターとしての当事者意識に関わる意欲や自信の変化に注目したところ、スキルアップと当事者意識の変容に関する効果が見られた。Since 2002，educational innovation has started through FD programs at the University of Tokushima. Until now various university-wide FD programs have been developed and conducted. The FD facilitator training program has been carried out to develop teachers who are responsible for FD in their respective departments. The Aim is to build up FD organizations in each department. Depaetment FD organizations have been incorporaled substantlally since 2008. We see elaborated collaboration between the university-wide FD organization and the departmenta FD organizations. The roles of the FD facilitator of each department as the core member to promote their departmental FD are becoming more important. To keep practicing substantial FD，they need to be conscious of their identity as the FD facilitator. They need to be actively involved in facilitating FD programs for their departments while maintaining a sense of ownership as an authentic member of the department.The aim of this paper is to discuss the importance of the FD facilitator program based on the results of the questionnaire survey conducted at the beginning and the end of FD facilitator training. To evaluate this program，we pay close attention to whether the participants are able to acquire FD skills and knowledge which are considered necessary. Futhermore focus on whether they demonstrated any attitudinal change in their motivation and confidence as FD facilitators

Preparation and Use of "Authentic English" Web Materials at The University of Tokushima (Tokushima, Japan)

This paper outlines a pilot project funded by The President’s Fund of the University of Tokushima that was designed to create English educational material to motivate students to acquire “Authentic English.” Recording was at sites in Honolulu (Hawaii, USA), Storrs (Connecticut, USA), Montreal (Canada) and Brisbane (Australia). Material was designed for use in teaching EFL students at our university who are taking general English courses. Section One of this paper refers to the aims and process of making English educational material for the Web. Sections Two, Three, and Four refer to how the materials were created. Section Five introduces technical aspects of the Web application. Sections Six and Seven discuss merits and problems related to the “Authentic English” material and its production

An annual report 2015 on campus wide Faculty Development programs at The University of Tokushima

Tokushima University’s FD promotion programs have started since 2002, promoting systematization, organization and routinization of Faculty Development. In 2015, in addition to the previous FD promotion programs, we have aimed to enhance the quality of higher education of our university, supporting effective implementation of Ministry of Education’s AP (=Acceleration Program for University Education Rebuilding) & building new educational system “Numbering System” for the assurance of higher education. Concretely, we carried out 3 programs; 1) educational innovation FD, 2) educational development FD, 3) comprehensive FD. As to each program, we showed their outlines and considered their results and problems shown from the questionnaire to their attendants