157 research outputs found

    Congenital Portal Vein Aneurysm Associated with Peliosis Hepatis and Intestinal Lymphangiectasia

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    Portal vein aneurisym (PVA), peliosis hepatis (PH) and intestinal lymphangiectasia (IL) all are very uncommon entities. Herein, we presented a unique patient with these three rare entities who was admitted to our hospital because of portal hypertensive ascites rich in protein and lymphocyte. PVA was extrahepatic and associated with coronary vein aneurysm. Peliosis hepatis was of microscopic form. Lymphangiectasia was present in peritoneum and small intestine. Diagnoses of these rare entities were made by imaging techniques and histopathological findings. Patient also had hydronephrosis caused by ureteropelvic junction narrowing. Best of our knowledge, there is no such a case reported previously with the association of PVA, PH and IL. Therefore, we propose PVAPHIL syndrome to define this novel association

    Type 1 Autoimmune Pancreatitis in Europe: Clinical Profile and Response to Treatment.

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    Background and aimsAutoimmune pancreatitis (AIP) is an immune-mediated disease of the pancreas with distinct pathophysiology and manifestations. Our aims were to characterize type 1 AIP in a large pan-European cohort and study the effectiveness of current treatment regimens.MethodsWe retrospectively analyzed adults diagnosed since 2005 with type 1 or not-otherwise-specified AIP in 42 European university hospitals. Type 1 AIP was uniformly diagnosed using specific diagnostic criteria. Patients with type 2 AIP and those who had undergone pancreatic surgery were excluded. The primary endpoint was complete remission, defined as the absence of clinical symptoms and resolution of the index radiological pancreatic abnormalities attributed to AIP.ResultsWe included 735 individuals with AIP (69% male; median age 57 years; 85% White). Steroid treatment was started in 634 patients, of whom 9 (1%) were lost to follow-up. The remaining 625 had a 79% (496/625) complete, 18% (111/625) partial, and 97% (607/625) cumulative remission rate, while 3% (18/625) did not achieve remission. No treatment was given in 95 patients, who had a 61% complete (58/95), 19% partial (18/95), and 80% cumulative (76/95) spontaneous remission rate. Higher (≥0.4 mg/kg/day) corticosteroid doses were no more effective than lower ( 2 weeks (OR 0.908; 95%CI 0.818-1.009). Elevated IgG4 levels were independently associated with a decreased chance of complete remission (OR 0.639; 95%CI 0.427-0.955). Relapse occurred in 30% of patients. Relapses within 6 months of remission induction were independent of the steroid tapering duration, induction treatment duration, and total cumulative dose.ConclusionPatients with type 1 AIP and elevated IgG4 level may need closer monitoring. For remission induction, a starting dose of 0.4 mg/kg/day for 2 weeks followed by a short taper period seems effective. This study provides no evidence to support more aggressive regimens

    Diagnostic and Therapeutic Capability of Double-Balloon Enteroscopy in Clinical Practice

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    Advances in technology have facilitated the common use of small-bowel imaging. Intraoperative enteroscopy was the gold standard method for small-bowel imaging. However, noninvasive capsule endoscopy and invasive balloon enteroscopy are currently the main endoscopic procedures that are routinely used for small-bowel pathologies, and the indications for both techniques are similar. Although obstruction is a contraindication for capsule endoscopy, it is not considered to be problematic for double-balloon enteroscopy. The most important advantage of double-balloon enteroscopy is the applicability of therapeutic interventions during the procedure; however, double-balloon enteroscopy has certain advantages as well as disadvantages

    Diagnostic Capability of Capsule Endoscopy in Small Bowel Diseases

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    What is the effective clinical use of small bowel capsule endoscopy in real life?

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    Capsule endoscopy (CE) is a noninvasive and easy method for evaluating the gastrointestinal (GI) tract. Since the wireless CE system was first developed, many new technical improvements have been made in order to gain maximum benefit from thisprocedure. However, at this stage, it remains a diagnostic modality, the main indication for its use being obscure GI bleeding. CE is only contraindicated in symptomatic intestinal obstruction. New indications for use and therapeutic options may become possible with the further development of nanotechnologies

    Non-alcoholic fatty liver disease: What has changed in the treatment since the beginning?

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    Non-alcoholic fatty liver disease (NAFLD) is an umbrella term to describe the entire spectrum of this common liver disease. In patients with NAFLD, especially those with non-alcoholic steatohepatitis (NASH), most often have one or more components of the metabolic syndrome, but this is not universal. Although most patients with NAFLD share many clinical features, only a subset of patients develops significant liver inflammation and progressive fibrosis. On the other hand, not all patients with NASH exhibit insulin resistance. NASH can be seen in patients who are lean and have no identifiable risk factors. Many clinical studies have tried numerous drugs and alternative medicine, however, investigators have failed to identify a safe and effective therapy for patients with NASH. As summarized, the heterogeneity of pathogenic pathways in individual patients with NASH may warrant the development of an individualized treatment according to the underlying pathogenic pathway. The differentiation of pathogenetic targets may require the development of diagnostic and prognostic biomarkers, and the identification of genetic susceptibilities. At present, evidence-based medicine provides only a few options including life-style modifications targeting weight loss, pioglitazone and vitamin E in non-diabetic patients with biopsy-proven NASH. (C) 2014 Baishideng Publishing Group Inc. All rights reserved
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