Sclerosing

BACKGROUND: Lymphatic malformations (LMs) are low-flow congenital lesions that consist of cysts of varying size. Sclerotherapy with intralesional bleomycin and OK-432 has been reported to yield dramatically beneficial results for this disorder. However, inflammation-related symptoms are often seen after treatment with these sclerosing agents. On the other hand, polidocanol (POL) is reportedly associated with fewer allergic and inflammatory reactions. Up to now, however, very few reports have documented the use of POL microfoam for treatment of LMs...

PURPOSE: To determine the causes of lacrimal gland inflammation based on histopathology and systemic evaluation. METHODS: This is a retrospective case series study. From the University of British Columbia Orbit Clinic between January 1976 and December 2008, we reviewed the medical records of 60 patients who presented with inflammatory features of the lacrimal gland (i.e., erythema, edema, or tenderness) in which the diagnoses were not possible clinically and on imaging alone...

Lymphangioma is a rare, benign mesenchymal neoplasm, which is characterized by numerous intercommunicating cystic spaces containing lymphatic fluid. It is considered a congenital disease resulting from the obstruction of regional lymph drainage during the developmental period. Lymphangioma frequently occurs in the cervical neck and axilla, also in the retroperitoneum, mediastinum, mesentery, omentum, colon, and pelvis, rarely in the perirenal space. These tumors usually present in childhood, but infrequently, these also present in adults...

BACKGROUND: Detection of cholangiocarcinoma (CC) remains a diagnostic challenge particularly in patients with primary sclerosing cholangitis (PSC). We recently established diagnostic peptide marker models in bile and urine to detect CC. Our aim was to combine both models to reach a higher diagnostic accuracy of CC diagnosis. METHODS: Bile (BPA) and urine (UPA) proteome analysis by capillary electrophoresis mass spectrometry was performed in a case-control phase II study on 87 patients (36 CC including 13 with CC on top of PSC, 33 PSC and 18 other benign disorders)...

Fibrosing mediastinitis (FM), also known as sclerosing mediastinitis, is an uncommon but serious disease involving the mediastinal structures. A high index of suspicion is essential to establish the diagnosis of FM and starting the appropriate therapy for patients. Here, we report a case of a young female who presented with chest symptoms and subsequently underwent different laboratory and radiologic investigations and an excisional biopsy. The findings of these investigations were consistent with the diagnosis of idiopathic FM...

Familial hypercholesterolemia (FH) is an autosomal dominant disorder characterized by impaired clearance of low-density lipoprotein cholesterol. Given limitations in pharmacologic therapy and the significant morbidity and mortality associated with this disease, liver transplantation may be offered to select homozygous FH patients in childhood in an effort to slow progression of atherosclerotic cardiovascular disease. In rare cases, domino liver transplantation can be performed, transplanting the livers of patients with various metabolic disorders into elderly recipients whose projected survival precludes prolonged waiting on the transplant list...

OBJECTIVE: To evaluate the efficacy and safety of sclerotherapy as the treatment of infected postoperative lymphocele in gynecologic malignancy patients. MATERIALS AND METHODS: Percutaneous catheter drainage (PCD) with or without sclerotherapy was performed for postoperative lymphocele in 75 patients from 2002 to 2014. Eighty-eight lymphoceles (43 non-infected as group A, 45 infected as group B) in 75 patients (mean age ± SD; 50.3 ± 11.3) were included. Sclerotherapy was performed in 17 (39...

Primary sclerosing cholangitis (PSC) is a rare disorder characterized by multi-focal bile duct strictures and progressive liver disease. Inflammatory bowel disease (IBD) is usually present and there is a high risk of cholangiocarcinoma and colorectal cancer. With no effective medical therapy, most patients ultimately require liver transplantation, after which disease recurrence may occur. With limited therapeutic options and lack of proven surveillance strategies, patients experience significant unmet needs...

Cholangiocyte senescence has been linked to primary sclerosing cholangitis (PSC). Persistent secretion of growth factors by senescent cholangiocytes leads to the activation of stromal fibroblasts (ASF), which are drivers of fibrosis. The activated phenotype of ASF is characterized by an increased sensitivity to apoptotic stimuli. Here, we examined the mechanisms of apoptotic priming in ASF and explored a combined targeting strategy to deplete senescent cholangiocytes and ASF from fibrotic tissue to ameliorate liver fibrosis...

BACKGROUND: Indwelling tunneled pleural catheters (TPCs) are increasingly being used to treat recurrent pleural effusions. There is also an increased interest in early pleurodesis in order to prevent infectious complications. We studied the time to removal and other outcomes for all the TPCs placed at our institution. METHODS: After institutional review board approval, records of patients who had had a TPC placed between July 2009 and June 2016 were reviewed; the catheters were placed in an endoscopy suite or during pleuroscopy with or without a sclerosant...

PURPOSE: Sclerosing adenosis (SA), found in ¼ of benign breast disease (BBD) biopsies, is a histological feature characterized by lobulocentric proliferation of acini and stromal fibrosis and confers a two-fold increase in breast cancer risk compared to women in the general population. We evaluated a NanoString-based gene expression assay to model breast cancer risk using RNA derived from formalin-fixed, paraffin-embedded (FFPE) biopsies with SA. METHODS: The study group consisted of 151 women diagnosed with SA between 1967 and 2001 within the Mayo BBD cohort, of which 37 subsequently developed cancer within 10 years (cases) and 114 did not (controls)...

BACKGROUND: Pulmonary manifestation of Hodgkin's lymphoma is common, and recently, single and/or multiple lung cavities with air-fluid levels has been reported as a primary manifestation of Hodgkin's lymphoma. However, the unusual presentation of the lung cavity itself has not been previously reported for Hodgkin's lymphoma, especially as the first presentation of the disease. CASE PRESENTATION: We report a 16-year-old male who presented to the emergency department with chief complaints of fever, unintentional 10-kilogram weight loss, a productive cough and pulmonary cavity for investigation...

OBJECTIVE: The etiopathogenesis and risk for development of biliary neoplasia in primary sclerosing cholangitis (PSC) are largely unknown. Microbes or their metabolites have been suggested to play a role. To explore this potential microbial involvement, we evaluated the differences in biliary microbiota in PSC patients at an early disease stage without previous endoscopic retrograde cholangiography (ERC) examinations, advanced disease stage, and with biliary dysplasia or cholangiocarcinoma...

BACKGROUND AND AIMS: Inflammatory bowel disease (IBD) may be complicated by primary sclerosing cholangitis (PSC). We aimed to assess the characteristics of Danish PSC-IBD patients and to compare their prognosis with IBD patients without PSC. METHODS: A retrospective nationwide population-based cohort of 257 PSC-IBD patients was assessed through Danish national registries and manual scrutiny of patient files. RESULTS: For all PSC-IBD patients diagnosed after 1976 (n=222) and 8,231 IBD controls (i...

The CD28 locus is associated with susceptibility to a variety of autoimmune and immune-mediated inflammatory diseases including primary sclerosing cholangitis (PSC). Previously, we linked the CD28 pathway in PSC disease pathology and found that vitamin D could maintain CD28 expression. Here, we assessed whether the PSC-associated CD28 risk variant A (rs7426056) affects CD28 expression and T cell function in healthy individuals (n = 14 AA, n = 14 AG, n = 14 GG). Homozygotes for the PSC disease risk allele (AA) showed significantly lower CD28 mRNA expression ex-vivo than either GG or AG (p < 0...

BACKGROUND AND STUDY AIMS : Endoscopic retrograde cholangiopancreatography (ERCP) is often performed in patients with primary sclerosing cholangitis (PSC). Our aim was to validate a treatment approach with the objective of decreasing ERCP related adverse events (AEs). PATIENTS AND METHODS : All patients who had undergone ERCP for PSC during the period from 2002 - 2012 were identified (group I). This group had traditional ERCP (no bile aspiration prior to contrast injection with balloon dilation and stent placement for treatment of dominant strictures)...

Cholangiocarcinoma (CCA) is an aggressive cancer arising from epithelial cells of the bile duct. Most patients with CCA have an unresectable tumor at the time of diagnosis. In Western countries, the risk of CCA increases in patients with primary sclerosing cholangitis, whereas liver fluke infection appears to be the major risk factor for CCA in Asian countries. A diagnosis of liver fluke infection often relies on stool samples, including microscopic examination, polymerase chain reaction-based assays, and fluke antigen detection...

INTRODUCTION: Sclerosing angiomatoid nodular transformation (SANT) of spleen is a new entity defined as a benign pathologic lesion. Fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) shows weak accumulation, thereby ruling out the malignancy in preoperative diagnosis is difficult. Herein, we reported a case of shrinking SANT with increased FDG accumulation during a 5-year follow-up period, which was treated by laparoscopic splenectomy. PRESENTATION OF CASE: A 64-year-old female had been referred to our hospital for the evaluation of a splenic tumor...

BACKGROUND: Sclerosing peritonitis (SP) is a rare but potentially fatal complication following orthotopic liver transplantation (OLT). The definitive surgical management is via a laparotomy peritonectomy and enterolysis procedure, but this carries risks particularly in the immunosuppressed transplant patient population. The natural history of SP is known from a handful of case reports and series, which mostly report de novo cases arising early on following OLT. The aim of this study was to identify all cases of de novo SP following OLT and the outcomes of management...

The pathophysiology of immunoglobulin G4-related disease (IgG4-RD) and its most common manifestations, IgG4-associated (sclerosing) cholangitis and autoimmune pancreatitis, remains largely unknown, but IgG4 is presumably involved. IgG4 is a promiscuous antibody, which could be directly pathogenic, fulfill a protective role, or could just be a fortuitous marker of an aberrant inflammatory response. IgG4 antibodies possess exclusive structural and functional characteristics suggesting anti-inflammatory and tolerance-inducing effects...