116 research outputs found

    X-linked primary ciliary dyskinesia due to mutations in the cytoplasmic axonemal dynein assembly factor PIH1D3

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    By moving essential body fluids and molecules, motile cilia and flagella govern respiratory mucociliary clearance, laterality determination and the transport of gametes and cerebrospinal fluid. Primary ciliary dyskinesia (PCD) is an autosomal recessive disorder frequently caused by non-assembly of dynein arm motors into cilia and flagella axonemes. Before their import into cilia and flagella, multi-subunit axonemal dynein arms are thought to be stabilized and pre-assembled in the cytoplasm through a DNAAF2–DNAAF4–HSP90 complex akin to the HSP90 co-chaperone R2TP complex. Here, we demonstrate that large genomic deletions as well as point mutations involving PIH1D3 are responsible for an X-linked form of PCD causing disruption of early axonemal dynein assembly. We propose that PIH1D3, a protein that emerges as a new player of the cytoplasmic pre-assembly pathway, is part of a complementary conserved R2TP-like HSP90 co-chaperone complex, the loss of which affects assembly of a subset of inner arm dyneins

    Laparoscopic cholecystectomy for acute calculous cholecystitis : a retrospective study assessing risk factors for conversion and complications

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    Background: The purpose of the study was to identify risk factors for conversion of laparoscopic cholecystectomy and risk factors for postoperative complications in acute calculous cholecystitis. The most common complications arising from cholecystectomy were also to be identified. Methods: A total of 499 consecutive patients, who had undergone emergent cholecystectomy with diagnosis of cholecystitis in Meilahti Hospital in 2013-2014, were identified from the hospital database. Of the identified patients, 400 had acute calculous cholecystitis of which 27 patients with surgery initiated as open cholecystectomy were excluded, resulting in 373 patients for the final analysis. The Clavien-Dindo classification of surgical complications was used. Results: Laparoscopic cholecystectomy was initiated in 373 patients of which 84 (22.5%) were converted to open surgery. Multivariate logistic regression identified C-reactive protein (CRP) over 150 mg/l, age over 65 years, diabetes, gangrene of the gallbladder and an abscess as risk factors for conversion. Complications were experienced by 67 (18.0%) patients. Multivariate logistic regression identified age over 65 years, male gender, impaired renal function and conversion as risk factors for complications. Conclusions: Advanced cholecystitis with high CRP, gangrene or an abscess increase the risk of conversion. The risk of postoperative complications is higher after conversion. Early identification and treatment of acute calculous cholecystitis might reduce the number of patients with advanced cholecystitis and thus improve outcomes.Peer reviewe

    Management of intra-abdominal infections : recommendations by the WSES 2016 consensus conference

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    This paper reports on the consensus conference on the management of intra-abdominal infections (IAIs) which was held on July 23, 2016, in Dublin, Ireland, as a part of the annual World Society of Emergency Surgery (WSES) meeting. This document covers all aspects of the management of IAIs. The Grading of Recommendations Assessment, Development and Evaluation recommendation is used, and this document represents the executive summary of the consensus conference findings.Peer reviewe

    2016 WSES guidelines on acute calculous cholecystitis

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    Credit Rationing with Symmetric Information

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    Without denying the importance of asymmetric information, this article purports the view that credit rationing may also originate from a lender's inability to classify loan applicants in proper risk categories. This effect is particularly strong when novel technologies are involved. Furthermore, its relevance may increase with the importance assigned to internal rating systems by the Basel accord. This article presents a measure of the inadequacy of a lender's classification criteria to the qualitative features of prospective borrowers. Even without information asymmetries, credit rationing may occur if this quantity reaches too high a value. Furthermore, some general principles are outlined, that may be used by lenders in order to change their classification criteria

    2013 WSES guidelines for management of intra-abdominal infections

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