Heterozygous B beta-chain C-terminal 12 amino acid elongation variant, B beta X462W (Kyoto VI), showed dysfibrinogenemia

A heterozygous patient with dysfibrinogenemia with slight bleeding and no thrombotic complications was diagnosed with fibrinogen Kyoto VI (K-VI). To elucidate the genetic mutation(s) and characterize the variant protein, we performed the following experiments and compared with identical and similar variants that have already been reported. The proposita's PCR-amplified DNA was analyzed by sequencing and her purified plasma fibrinogen underwent SDS-PAGE followed by immunoblotting, fibrin polymerization, and scanning electron microscopic observation of fibrin clot and fibers. Sequence analyses showed that K-VI fibrinogen substituted W (TGG) for terminal codon (TAG), resulting in 12 amino acid elongation 462-473 (WSPIRRFLLFCM) in the B beta-chain. Protein analyses indicated that the presence of some albumin-binding variant fibrinogens and a dimeric molecule of variant fibrinogens reduced fibrin polymerization, with a thinner fiber and aberrant fibrin network. These results are almost the same as for the identical variant of Magdeburg, however, different from the similar variant of Osaka VI [ 12 amino acid elongation 462-473 (KSPIRRFLLFCM) in the B beta-chain] in the presence of variant forms and clot structure. We speculate the side-chain difference at 462 residues, W in K-VI, K in Osaka VI, and/or the difference in the presence of disulfide bridged forms of variant fibrinogens, led to the notable difference in the fibrin bundle network. Although a strong evolutional and structural association between B beta-chain and gamma-chain molecules is established, the corresponding recombinant 15 residue elongation variants of the fibrinogen gamma-chain showed reduced assembly and secretion.ArticleBLOOD COAGULATION & FIBRINOLYSIS. 23(1):87-90 (2012)journal articl

A Multicenter, Open-label, Clinical Trial to Assess the Effectiveness and Safety of Allogeneic Hematopoietic Stem Cell Transplantation Using Reduced-intensity Conditioning in Relapsed/refractory Anaplastic Large-cell Lymphoma in Children

No standard treatment for relapsed or refractory anaplastic large-cell lymphoma (ALCL) has been established. This study is a multicenter, open-label trial to examine the effectiveness and safety of transplantation with reduced-intensity conditioning (RIC) for patients under 20 years old with relapsed or refractory ALCL. We defined RIC as the administration of fludarabine (30 mg/m2/day) for five days plus melphalan (70 mg/m2/day) for two days and total body irradiation at 4 Gy, followed by allogeneic hematopoietic stem cell transplantation

医療的ケアを必要とする在宅療養児のきょうだいへの支援

本研究は、医療的ケアを必要とする在宅療養児のきょうだいが抱えた思いや悩み、在宅療養児の疾患認識、きょうだい支援の現状やニーズを明らかにし、それらの結果から今後の支援のあり方を考察することを目的とした。対象は医療的ケアが必要な在宅療養児をきょうだいにもつ青年２名であり、インタビューガイドに基づいて半構成的面接法を実施した。その結果、８つのカテゴリーが抽出され、きょうだいが在宅療養児を通して良い影響を大きく受けていた一方で、課題として在宅療養児の疾患の共有時期・理解度、必然的に医療的ケアに関わらざるを得ない環境、ライフ・イベント時に直面する問題、将来きょうだいが在宅療養児を支える際の問題などが挙がった。きょうだいへの支援においては親の受容状況なども大切な視点であり、保健福祉医療の各側面からのきょうだい支援が必要であることが示唆された

Results of the search for inspiraling compact star binaries from TAMA300's observation in 2000-2004

We analyze the data of TAMA300 detector to search for gravitational waves from inspiraling compact star binaries with masses of the component stars in the range 1-3Msolar. In this analysis, 2705 hours of data, taken during the years 2000-2004, are used for the event search. We combine the results of different observation runs, and obtained a single upper limit on the rate of the coalescence of compact binaries in our Galaxy of 20 per year at a 90% confidence level. In this upper limit, the effect of various systematic errors such like the uncertainty of the background estimation and the calibration of the detector's sensitivity are included.Comment: 8 pages, 4 Postscript figures, uses revtex4.sty The author list was correcte

The Japanese space gravitational wave antenna; DECIGO

DECi-hertz Interferometer Gravitational wave Observatory (DECIGO) is the future Japanese space gravitational wave antenna. DECIGO is expected to open a new window of observation for gravitational wave astronomy especially between 0.1 Hz and 10 Hz, revealing various mysteries of the universe such as dark energy, formation mechanism of supermassive black holes, and inflation of the universe. The pre-conceptual design of DECIGO consists of three drag-free spacecraft, whose relative displacements are measured by a differential Fabry– Perot Michelson interferometer. We plan to launch two missions, DECIGO pathfinder and pre- DECIGO first and finally DECIGO in 2024

DECIGO pathfinder

DECIGO pathfinder (DPF) is a milestone satellite mission for DECIGO (DECi-hertz Interferometer Gravitational wave Observatory) which is a future space gravitational wave antenna. DECIGO is expected to provide us fruitful insights into the universe, in particular about dark energy, a formation mechanism of supermassive black holes, and the inflation of the universe. Since DECIGO will be an extremely large mission which will formed by three drag-free spacecraft with 1000m separation, it is significant to gain the technical feasibility of DECIGO before its planned launch in 2024. Thus, we are planning to launch two milestone missions: DPF and pre-DECIGO. The conceptual design and current status of the first milestone mission, DPF, are reviewed in this article

認知症高齢者の在宅療養継続を目指した訪問看護師の支援－対象理解に焦点をあてて－

本研究は、対象理解に焦点をあてた認知症高齢者の在宅療養継続を目指した訪問看護師の支援について明らかにすることを目的とした。対象は訪問看護師３名で、半構成的面接法を用いてインタビューした内容を質的記述的に分析した。その結果、訪問看護師は、【培ってきた人生に敬意を払い療養者の尊厳を守る】という信念に立ち、【言葉に頼らず全身を使って気遣いを表現し焦らず徐々に心を通わせ】、【観察とケアチームとの連携により療養者が自ら表現できない状態をアセスメント】して療養者の世界に身を置いていた。その上で、【療養者の状態を経時的に捉え起こりうるリスクをアセスメントし予防】し、【療養者が言葉にできない症状を感知し適切な治療につなげ】、【療養者のできる力を最大限に引き出し生活に取り入れ】回復を助ける支援を行っており、カテゴリーの１つ１つが連動して実施されることが、認知症高齢者への支援に重要であることが示唆された