Anti-integrin αvβ6 autoantibodies in patients with primary sclerosing cholangitis.

Background: Patients with primary sclerosing cholangitis (PSC) possess autoantibodies against biliary epithelial cells. However, the target molecules remain unknown.

Methods: The sera of patients with PSC and controls were subjected to enzyme-linked immunosorbent assays to detect autoantibodies using recombinant integrin proteins.

Immobilization of a flavin molecule onto poly(methacrylic acid)s and its application in aerobic oxidation catalysis: effect of polymer stereoregularity.

The isoalloxazine ring system, called flavin, was successfully immobilized on poly(methacrylic acid)s, PMAAs, with different tacticity post-polymerization modification under suitable conditions. The resulting flavin-containing polymers showed catalytic activity for aerobic oxidation reactions, in which the polymer stereoregularity clearly influenced their catalytic activity.

Factor VII Deficiency Due to Compound Heterozygosity for the p.Leu13Pro Mutation and a Novel Mutation in the HNF4 Binding Region (-58G>C) in the F7 Promoter.

We investigated the molecular basis of factor VII (FVII) deficiency in a Japanese patient and identified compound heterozygous mutations. Factor VII activity and antigen levels in the patient were less than 5.0% and 6.

Ulnar Artery Perforator Adiposal Flap for Paraffinoma Treatment in a Patient With Leprosy: A Case Report.

Leprosy is a chronic infection in humans that mainly affects the peripheral nerves and skin. Paraffin filling has been previously used for muscle atrophy treatment. However, the formation of paraffin granulomas (paraffinomas) can occur over the long term.

Clinical features and symptoms of IgG4-related ophthalmic disease: a multicenter study.

Purpose: The aim of this study was to elucidate the clinical features and symptoms of IgG4-related ophthalmic disease (IgG4-ROD).

Study Design: Retrospective, multicenter study.

Methods: The medical charts of 378 patients with IgG4-ROD diagnosed at 9 hospitals in Japan were reviewed.

Essential role of Notch/Hes1 signaling in postnatal pancreatic exocrine development.

Background: Notch/Hes1 signaling has been shown to play a role in determining the fate of pancreatic progenitor cells. However, its function in postnatal pancreatic maturation is not fully elucidated.

Methods: We generated conditional Hes1 knockout and/or Notch intracellular domain (NICD) overexpression mice in Ptf1a- or Pdx1-positive pancreatic progenitor cells and analyzed pancreatic tissues.

Insertion of a Fogarty catheter through a slip joint section for neonatal and infantile one-lung ventilation : a report of two cases.

Here, we report two cases involving a neonate and child in which a slip joint section was used to thread a Fogarty catheter into the endotracheal tube for one-lung ventilation (OLV). Both the neonate and infant required OLV, and were placed under general anesthesia. A Fogarty catheter was used for OLV.

Identification of an Anti-Integrin αvβ6 Autoantibody in Patients With Ulcerative Colitis.

Background And Aims: Ulcerative colitis is the most frequent type of inflammatory bowel disease and is characterized by colonic epithelial cell damage. Although involvement of autoimmunity has been suggested in ulcerative colitis, specific autoantigens/antibodies have yet to be elucidated.

Methods: Using 23 recombinant integrin proteins, we performed enzyme-linked immunosorbent assays on sera from patients with ulcerative colitis and controls.

Digital artery perforator flap transfer for volar soft tissue defect due to dissociation from joint contracture in camptodactyly.

Camptodactyly is a condition defined by persistent flexion contracture of the proximal interphalangeal joints of the hand. Surgical treatment requires flaps for the dissection of the skin and the resulting soft tissue defect, soft tissue release, and sometimes reconstruction of the extension mechanism. Z-plasty and transposition flap have been reported as methods used in soft tissue defects.

Is Essential in Proliferating Ductal Cell-Mediated Development of Intrahepatic Cholangiocarcinoma.

Intrahepatic cholangiocarcinoma (ICC) is frequently driven by aberrant activation and develops in the liver with chronic inflammation. Although the Notch signaling pathway is critically involved in ICC development, detailed mechanisms of Notch-driven ICC development are still unknown. Here, we use mice whose Notch signaling is genetically engineered to show that the Notch signaling pathway, specifically the Notch/Hes1 axis, plays an essential role in expanding ductular cells in the liver with chronic inflammation or oncogenic activation.

A novel technique for mapping biopsy of bile duct cancer.

BACKGROUND : Accurate preoperative assessment of the longitudinal extension of perihilar cholangiocarcinoma (PHCC) is essential for treatment planning. Mapping biopsies for PHCC remain challenging owing to technical difficulties and insufficient sample amounts. The aim of this study was to investigate the usefulness of a novel technique for mapping biopsies of PHCC.

Volar Locking Plate Fixation for Distal Radius Fractures by Intraoperative Computed Tomographic-Guided Navigation.

Purpose: Unstable distal radius intra-articular fractures require restoration of alignment. Exact fixation of intra-articular fragments is ideal. Here, we employed intraoperative computed tomography (CT) navigation to insert screws accurately in the intra-articular dorsal fragments during treatment with a volar locking plate for distal radius intra-articular fractures.

Treatment outcomes of IgG4-producing marginal zone B-cell lymphoma: a retrospective case series.

IgG4-producing marginal zone B-cell lymphomas (MZLs) have been recently proposed as a subtype of MZLs. Despite the abundant literature on pathophysiological features of this type of lymphoma, only a few retrospective studies pertaining to the treatment outcomes have been reported, and its prognosis remains unclear. We retrospectively analyzed seven patients with IgG4-producing MZLs diagnosed at our institute, with specific reference to treatment and outcomes.

CXCR4 in Tumor Epithelial Cells Mediates Desmoplastic Reaction in Pancreatic Ductal Adenocarcinoma.

Pancreatic ductal adenocarcinoma (PDAC) features abundant stromal cells with an excessive extracellular matrix (ECM), termed the desmoplastic reaction. CXCR4 is a cytokine receptor for stromal cell-derived factor-1 (CXCL12) expressed in PDAC, but its roles in PDAC and the characteristic desmoplastic reaction remain unclear. Here, we generated a mouse model of PDAC with conditional knockout of Cxcr4 (KPC-Cxcr4-KO) by crossing Cxcr4 flox mice with Pdx1-Cre;KrasLSL-G12D/+;Trp53LSL-R172H/+ (KPC-Cxcr4-WT) mice to assess the development of pancreatic intraepithelial neoplasia (PanIN) and pancreatic cancers.

Rb and p53 Execute Distinct Roles in the Development of Pancreatic Neuroendocrine Tumors.

Pancreatic neuroendocrine tumors (PanNET) were classified into grades (G) 1 to 3 by the World Health Organization in 2017, but the precise mechanisms of PanNET initiation and progression have remained unclear. In this study, we used a genetically engineered mouse model to investigate the mechanisms of PanNET formation. Although pancreas-specific deletion of the gene ( ) in mice did not affect pancreatic exocrine cells, the α-cell/β-cell ratio of islet cells was decreased at 8 months of age.

Utility of laser-cut covered self-expandable metal stents for unresectable malignant distal biliary obstruction: a single-center experience.

Background: Few reports have evaluated the effectiveness of laser-cut, covered, self-expandable metal stents (LC-CSEMS) for unresectable malignant distal biliary obstruction (MDBO) and whether reintervention is feasible after placement. We describe our experience with LC-CSEMS placement for unresectable MDBO.

Methods: Patients undergoing LC-CSEMS placement for unresectable MDBO from November 2014 to December 2018 were reviewed.

Cutaneous Myxoma of Multiple Lesions.

Cutaneous myxoma is a rare neoplasm typically occurring as an asymptomatic nodule on the head, neck, and chest regions of adults. However, multiple cutaneous myxoma of the subungual space is even rarer. Here, we report a case of multiple cutaneous myxoma of the subungual space in which the tumor was removed by creating a mid-radial incision while preserving the nail matrix.

Hes1 plays an essential role in Kras-driven pancreatic tumorigenesis.

Most pancreatic ductal adenocarcinoma (PDAC) develops from pancreatic epithelial cells bearing activating mutant KRAS genes through precancerous lesions, i.e. acinar-to-ductal metaplasia (ADM) and pancreatic intraepithelial neoplasia (PanIN).

Platforms of in vivo genome editing with inducible Cas9 for advanced cancer modeling.

The emergence of clustered regularly interspaced short palindromic repeat (CRISPR)/Cas9 technology has dramatically advanced how we manipulate the genome. Regarding in vivo experiments, Cas9-transgenic animals could provide efficient and complex genome editing. However, this potential has not been fully realized partly due to a lack of convenient platforms and limited examples of successful disease modeling.

Laminin 511 is a target antigen in autoimmune pancreatitis.

Autoimmune pancreatitis (AIP), a major manifestation of immunoglobulin G4-related disease (IgG4-RD), is an immune-mediated disorder, but the target autoantigens are still unknown. We previously reported that IgG in patients with AIP induces pancreatic injuries in mice by binding the extracellular matrix (ECM). In the current study, we identified an autoantibody against laminin 511-E8, a truncated laminin 511, one of the ECM proteins, in patients with AIP.