Endoscopic thoracic sympathicotomy for Raynaud's phenomenon

AbstractPurpose: For many years, thoracic sympathectomy via open surgery was not used to treat Raynaud's phenomenon because of the invasiveness of this procedure and the poor long-term outcomes associated with it. However, with the introduction of endoscopic surgery, thoracic sympathectomy (or sympathicotomy) has been performed by some surgeons as a less invasive surgical option for patients with Raynaud's phenomenon. The less invasive procedure has the possibility of emphasizing merits of sympathectomy. The purpose of this study was to reevaluate the efficacy of sympathicotomy for Raynaud's phenomenon with endoscopic technique and its range of applicability. Methods: Between December 1992 and August 2001, endoscopic thoracic sympathicotomy (ETS) was performed in 28 patients with Raynaud's phenomenon (of a total of 502 patients with autonomic disorders who underwent ETS) at National Kanazawa Hospital. We considered indications for surgical treatment of Raynaud's phenomenon to include severe chronic symptoms or nonhealing digital ulceration refractory to intensive medical therapy. All patients were mailed a self-assessment questionnaire after surgery to determine the immediate and long-term results of the procedure. Data from both initial and long-term follow-up examinations were obtained. Results: Fifty-four ETS procedures were performed in 28 patients. No operative mortality was seen, and no occurrence of major complications necessitated open surgery. Initial resolution or improvement of symptoms was achieved in 26 of 28 patients (92.9%). However, later in the postoperative period, symptoms recurred in 23 of 28 patients (82.1%), although no recurrence of digital ulceration was seen throughout our observation. At the final follow-up examination (median follow-up period, 62.5 months), 25 patients (89.3%) reported overall improvement of the frequency and severity of their symptoms. Conclusion: Despite the high rate of recurrence, ETS clearly produced a high rate of initial relief. ETS did indeed promote healing of digital ulcers, and the procedure shows potential for reducing the severity of refractory symptoms. We consider ETS to be the method of choice for treatment of severe or refractory Raynaud's phenomenon, and especially for Raynaud's involving digital ulcer, because of its safety and efficacy. (J Vasc Surg 2002;36:57-61.

帽子の構成に関する研究(第1報) : サイズ元線の形とブリムの傾斜角度について

帽子のサイズ元線は帽子の種類により,また好みにより種々のカーブをつける場合が多い。同じ形のブリムをつけても,サイズ元のカーブによってその形が非常に変化してくるので,この傾斜の変化をつかみ,より好みにあったブリムを構成するためこの研究をおこなった。はじめに,クラウンのサイズ元の変化によって同一のブリムの傾斜角度がどう変わるか,またブリム全体の傾斜のバランスはどうかということについて実験した。クラウンはごく一般的な大丸角クラウンの木型に0.5cm間隔の7種類のカーブを描いたものを用いた。ブリムは平らブリム,下がりブリム,急下がりブリム,特別急下がりブリムの木型にあわせて型紙をとり,画用紙でサイズ元1.5cm,バック2cmの止め代をつけたものを24枚用意した。このブリムを木型のサイズ元線のカーブにそれぞれ止めつけて傾斜角度を測った。各角度は第1表より第4表に示した通りであるが,全体的にみると,クラウンのサイズ元線が水平のもの,フロントからサイドにかけては水平でバックの0.5cm上がったものが,最も安定してバランスがとれており,次はフロントが0.5cm,バックが1cm上がったものがそれについで比較的安定している。サイズ元のカーブがそれ以上強くなると,これら4種類のブリムの形ではフロント,バックが上がりすぎ,サイドが下がりすぎてバランスがとれなくなることがわかる。フロント,バックとサイドの差がキャプリンのような平らブリム,下がりブリムでは5°位まで,クロッシェによく用いられる急下がりブリム,特別急下がりブリムでは約10°位までで,これ以上差が出てくるとバランスがとれにくくなる。ただしボンネクロッシェのような形ではクラウンのサイズ元のカーブが強く,サイドの下がりも強いので,ブリムの角度もそれにつれて急傾斜でも良いことがわかる。これら4種のブリムの形を,実験に使用した大丸角クラウンの底面をサイズ元原型として展開した製図法は,第2図から第6図の通りである。次に6種類のクラウンのサイズ元線のいずれの場合でも,帽子としてバランスの良いブリムの傾斜をフロント40°,サイド50°,バック35°に設定し,前記のような平らブリムにサイズ元2cm,エッジング5.2cm間隔に放射状の切り込みを入れ,これを6種のサイズ元カーブに従ってクラウンの木型に止めつけた。フロント40°,サイド50°,バック35°の傾斜になるまでエッジング側を重ね形作ってゆき,これを型紙に直した。6種類のクラウンと,ブリムのサイズ元線の変化の状態は第8図の通りである。クラウンのサイズ元線A～S～Gの場合が最もカーブがゆるやかで,E～S～Lの場合が最も強いカーブとなる。フロント寄り1/3とサイドの間で最もくりが深くなっており,その差は1.1cmである。バック寄り約1/3の位置で2つの線は交差し,バックで約2cmの開きが出てくる。A～S～H, B～S～I, C～S～J, D～S～Kのカーブのものはその中間に位置し,クラウンのサイズ元線のカーブが強いもの程,ブリムのカーブも強くなり,1段階ごとにP\u27S\u27間で約0.2cmくれ,バックでは約0.3cmずつそのカーブが強くなってゆくことがわかった。クラウンの底面の形が変わってくることにより,またブリムの表地,しん地の素材によっても,ブリムの形が複雑に変わってくると思われるので,それは今後の課題としたい

A multi-ethnic meta-analysis identifies novel genes, including ACSL5, associated with amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis (ALS) is a devastating progressive motor neuron disease that affects people of all ethnicities. Approximately 90% of ALS cases are sporadic and thought to have multifactorial pathogenesis. To understand the genetics of sporadic ALS, we conducted a genome-wide association study using 1,173 sporadic ALS cases and 8,925 controls in a Japanese population. A combined meta-analysis of our Japanese cohort with individuals of European ancestry revealed a significant association at the ACSL5 locus (top SNP p = 2.97 × 10−8). We validated the association with ACSL5 in a replication study with a Chinese population and an independent Japanese population (1941 ALS cases, 3821 controls; top SNP p = 1.82 × 10−4). In the combined meta-analysis, the intronic ACSL5 SNP rs3736947 showed the strongest association (p = 7.81 × 10−11). Using a gene-based analysis of the full multi-ethnic dataset, we uncovered additional genes significantly associated with ALS: ERGIC1, RAPGEF5, FNBP1, and ATXN3. These results advance our understanding of the genetic basis of sporadic ALS

Synaptic E3 Ligase SCRAPPER in Contextual Fear Conditioning: Extensive Behavioral Phenotyping of Scrapper Heterozygote and Overexpressing Mutant Mice

SCRAPPER, an F-box protein coded by FBXL20, is a subunit of SCF type E3 ubiquitin ligase. SCRAPPER localizes synapses and directly binds to Rab3-interacting molecule 1 (RIM1), an essential factor for synaptic vesicle release, thus it regulates neural transmission via RIM1 degradation. A defect in SCRAPPER leads to neurotransmission abnormalities, which could subsequently result in neurodegenerative phenotypes. Because it is likely that the alteration of neural transmission in Scrapper mutant mice affect their systemic condition, we have analyzed the behavioral phenotypes of mice with decreased or increased the amount of SCRAPPER. We carried out a series of behavioral test batteries for Scrapper mutant mice. Scrapper transgenic mice overexpressing SCRAPPER in the hippocampus did not show any significant difference in every test argued in this manuscript by comparison with wild-type mice. On the other hand, heterozygotes of Scrapper knockout [SCR (+/−)] mice showed significant difference in the contextual but not cued fear conditioning test. In addition, SCR (+/−) mice altered in some tests reflecting anxiety, which implies the loss of functions of SCRAPPER in the hippocampus. The behavioral phenotypes of Scrapper mutant mice suggest that molecular degradation conferred by SCRAPPER play important roles in hippocampal-dependent fear memory formation