Elastogenesis in cultured dermal fibroblasts from patients with lysosomal β-galactosidase, protective protein/cathepsin A and neuraminidase-1 deficiencies

The human GLB1 gene encodes a lysosomal β-galactosidase (β-Gal) and an elastinbinding protein(EBP). Defect of the EBP as a chaperon for tropoelastin and a component of receptor complex amongneuraminidase-1 (NEU1) and protective protein/ cathepsin A(PPCA)is suggested responsible for impaired elastogenesis in autosomal recessive β-Gal, PPCA and NEU1 deficiencies. The purpose of this study is to determine effects ofGLB1, PPCA and NEU1gene mutations on elastogenesis in skin fibroblasts. Elastic fiber formation and the EBP mRNA expression were examined by immunofluorescence with an anti-tropoelastin antibody and RT-PCR selective for EBP in skin fibroblasts with these lysosomal enzyme deficiencies. Apparently normal elastogenesis and EBP mRNA expression were observed for fibroblasts from Morquio B disease cases with the GLB1 gene alleles (W273L/W273L, W273L/R482H andW273L/W509C substitutions, respectively), a galactosialidosis case with the PPCA allele (IVS7+3A/IVS7+3A) and a sialidosis case with the NEU1 allele (V217M/G243R) as well as normal subject. In this study, theW273L substitution in the EBP could impossibly cause the proposed defect of elastogenesis, and the typical PPCA splicing mutation and the V217M/G243R substitutions in the NEU1 might hardly have effects on elastic fiber formation in the dermal fibroblasts

Characteristics of macroalgal vegetation along the coasts of Yashiro and Heigun islands, western Seto Inland Sea, Japan : especially, on the vertical distribution patterns of species of Sargassum and Ecklonia

瀬戸内海西部の伊予灘と広島湾の島嶼（屋代島，平郡島）で，大型褐藻のホンダワラ類（ヒバマタ目ホンダワラ科）とクロメ（コンブ目レッソニア科）により形成される藻場の特性を調べた。対象とした藻場は，自然岩礁域およびそれに付帯する礫集積域に形成されているものが8か所，投石による人工礁に形成されているものが3か所であり，ライントランセクト法により植生の垂直構造を明らかにし，そこにみられる法則性と環境要因との関係について考察した。調査では計85種の海藻を確認し，そのうちホンダワラ類についてはヒジキ，ノコギリモク，アカモク，ジョロモク，ホンダワラ等の計14種をみとめた。総じて，ホンダワラ類は潮間帯から水深4m 程度までで優占し，クロメは14mを下限にホンダワラ類より深所まで分布した。しかし，平郡島南岸や屋代島南岸では，浅所の岩盤上部にクロメ，その下部の漂砂影響域や礫集積域にホンダワラ類が生育し，植生の垂直構造が逆転している場合もみとめられた。人工礁上では，おおむね自然岩礁・礫集積域と類似した植生が形成されていたが，特に屋代島北岸（広島湾側）では深所で沈積浮泥の影響がみとめられ，クロメの生育は不良であるか植生から欠落していた。藻場の環境特性と植生の関係を解析した結果，平均水深が浅い藻場ではホンダワラ類の平均被度が大きくなり，海底傾斜が大きい藻場ではクロメの平均被度が大きくなる傾向がみとめられた。Characteristics of macroalgal beds composed of sargassaceous plants (Fucales, Phaeophyta) and Ecklonia kurome (Laminariales, Phaeophyta) were surveyed at Yashiro and Heigun islands in the western Seto Inland Sea (Iyo-nada Sea and Hiroshima Bay areas). The surveyed macroalgal beds were located on 8 natural rocky or boulder shores and 3 subtidal stone-built artificial reefs. A transect was set at each bed between the upper and lower limits of the macroalgal vegetation to investigate vertical distribution patterns of the constituent species. Eighty-five macroalgal species were recognized in total, and among those, 14 sargassaceous species, such as Sargassum fusiforme, S. macrocarpum, S. horneri, S. fulvellum and Myagropsis myagroides, were found. In general, sargassaceous plants were dominant from the intertidal to 4 m depth, and E. kurome distributed in deeper zones than sargassaceous plants, down to 14 m at the deepest. However, in some beds on the southern coasts of the islands, this vertical pattern was reversed. Ecklonia grows on rocky substrata which was stable and free from the effects of sand action, though sargassaceous plants grow on boulders distributed in zones deeper than the Ecklonia zones. On artificial reefs, similar vegetation with those on natural substrata was established. On reefs of the northern coast of Yashiro Is. (Hiroshima Bay area), however, sedimentation on substrata prevented the growth of Ecklonia. In the correlation analysis between physical characteristics and vegetation of the beds, there is a tendency that mean coverage of sargassaceous plants of the beds is higher as the mean depth of the beds becomes shallower, and that mean coverage of Ecklonia is higher as the slope of the beds becomes steeper.本現地調査は，農林水産省プロジェクト研究「地球温暖化が水産分野に与える影響評価と適応技術の開発」（平成22―24年度）および水産庁「藻場・干潟の炭素吸収源評価と吸収機能向上技術の開発」（平成23，24年度）の一環として実施し，とりまとめにあたってはJSPS科研費25450267の助成を受けたものである

Tokyo Guidelines 2018: initial management of acute biliary infection and flowchart for acute cholangitis

The initial management of patients with suspected acute biliary infection starts with the measurement of vital signs to assess whether or not the situation is urgent. If the case is judged to be urgent, initial medical treatment should be started immediately including respiratory/circulatory management if required, without waiting for a definitive diagnosis. The patient's medical history is then taken; an abdominal examination is performed; blood tests, urinalysis, and diagnostic imaging are carried out; and a diagnosis is made using the diagnostic criteria for cholangitis/cholecystitis. Once the diagnosis has been confirmed, initial medical treatment should be started immediately, severity should be assessed according to the severity grading criteria for acute cholangitis/cholecystitis, and the patient's general status should be evaluated. For mild acute cholangitis, in most cases initial treatment including antibiotics is sufficient, and most patients do not require biliary drainage. However, biliary drainage should be considered if a patient does not respond to initial treatment. For moderate acute cholangitis, early endoscopic or percutaneous transhepatic biliary drainage is indicated. If the underlying etiology requires treatment, this should be provided after the patient's general condition has improved; endoscopic sphincterotomy and subsequent choledocholithotomy may be performed together with biliary drainage. For severe acute cholangitis, appropriate respiratory/circulatory management is required. Biliary drainage should be performed as soon as possible after the patient's general condition has been improved by initial treatment and respiratory/circulatory management. Free full articles and mobile app of TG18 are available at: http://www.jshbps.jp/modules/en/index.php?content_id=47 . Related clinical questions and references are also include

Tokyo Guidelines 2018 diagnostic criteria and severity grading of acute cholecystitis (with videos)

The Tokyo Guidelines 2013 (TG13) for acute cholangitis and cholecystitis were globally disseminated and various clinical studies about the management of acute cholecystitis were reported by many researchers and clinicians from all over the world. The 1st edition of the Tokyo Guidelines 2007 (TG07) was revised in 2013. According to that revision, the TG13 diagnostic criteria of acute cholecystitis provided better specificity and higher diagnostic accuracy. Thorough our literature search about diagnostic criteria for acute cholecystitis, new and strong evidence that had been released from 2013 to 2017 was not found with serious and important issues about using TG13 diagnostic criteria of acute cholecystitis. On the other hand, the TG13 severity grading for acute cholecystitis has been validated in numerous studies. As a result of these reviews, the TG13 severity grading for acute cholecystitis was significantly associated with parameters including 30-day overall mortality, length of hospital stay, conversion rates to open surgery, and medical costs. In terms of severity assessment, breakthrough and intensive literature for revising severity grading was not reported. Consequently, TG13 diagnostic criteria and severity grading were judged from numerous validation studies as useful indicators in clinical practice and adopted as TG18/TG13 diagnostic criteria and severity grading of acute cholecystitis without any modification. Free full articles and mobile app of TG18 are available at: http://www.jshbps.jp/modules/en/index.php?content_id=47. Related clinical questions and references are also include

Delphi consensus on bile duct injuries during laparoscopic cholecystectomy:An evolutionary cul-de-sac or the birth pangs of a new technical framework?

Bile duct injury (BDI) during laparoscopic cholecystectomy remains a serious iatrogenic surgical complication. BDI most often occurs as a result of misidentification of the anatomy; however, clinical evidence on its precise mechanism and surgeons' perceptions is scarce. Surgeons from Japan, Korea, Taiwan, and the USA, etc. (n=614) participated in a questionnaire regarding their BDI experience and near-misses; and perceptions on landmarks, intraoperative findings, and surgical techniques. Respondents voted for a Delphi process and graded each item on a five-point scale. The consensus was built when 80% of overall responses were 4 or 5. Response rates for the first- and second-round Delphi were 60.6% and 74.9%, respectively. Misidentification of local anatomy accounted for 76.2% of BDI. Final consensus was reached on: (1) Effective retraction of the gallbladder, (2) Always obtaining critical view of safety, and (3) Avoiding excessive use of electrocautery/clipping as vital procedures; and (4) Calot's triangle area and (5) Critical view of safety as important landmarks. For (6) Impacted gallstone and (7) Severe fibrosis/scarring in Calot's triangle, bail-out procedures may be indicated. A consensus was reached among expert surgeons on relevant landmarks and intraoperative findings and appropriate surgical techniques to avoid BD

TG18 management strategies for gallbladder drainage in patients with acute cholecystitis: Updated Tokyo Guidelines 2018 (with videos)

Since the publication of the Tokyo Guidelines in 2007 and their revision in 2013, appropriate management for acute cholecystitis has been more clearly established. Since the last revision, several manuscripts, especially for alternative endoscopic techniques, have been reported; therefore, additional evaluation and refinement of the 2013 Guidelines is required. We describe a standard drainage method for surgically high-risk patients with acute cholecystitis and the latest developed endoscopic gallbladder drainage techniques described in the updated Tokyo Guidelines 2018 (TG18). Our study confirmed that percutaneous transhepatic gallbladder drainage should be considered the first alternative to surgical intervention in surgically high-risk patients with acute cholecystitis. Also, endoscopic transpapillary gallbladder drainage or endoscopic ultrasound-guided gallbladder drainage can be considered in high-volume institutes by skilled endoscopists. In the endoscopic transpapillary approach, either endoscopic naso-gallbladder drainage or gallbladder stenting can be considered for gallbladder drainage. We also introduce special techniques and the latest outcomes of endoscopic ultrasound-guided gallbladder drainage studies. Free full articles and mobile app of TG18 are available at: . Related clinical questions and references are also include