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| 1 | Encapsulating peritoneal sclerosis显示文摘Encapsulating peritoneal sclerosis(EPS) is a debilitating condition characterized by a fibrocollagenous membrane encasing the small intestine, resulting in recurrent small bowel obstructions. EPS is most commonly associated with long-term peritoneal dialysis, though medications, peritoneal infection, and systemic inflammatory disorders have been implicated. Many cases remain idiopathic. Diagnosis is often delayed given the rarity of the disorder combined with non-specific symptoms and laboratory findings. Although cross-sectional imaging with computed tomography of the abdomen can be suggestive of the disorder, many patients undergo exploratory laparotomy for diagnosis. Mortality approaches 50% one year after diagnosis. Treatment for EPS involves treating the underlying condition or eliminating possible inciting agents(i.e. peritoneal dialysis, medications, infections) and nutritional support, frequently with total parenteral nutrition. EPSspecific treatment depends on the disease stage. In the inflammatory stage, corticosteroids are the treatment of choice, while in the fibrotic stage, tamoxifen may be beneficial. In practice, distinguishing between stages may be difficult and both may be used. Surgical intervention, consisting of peritonectomy and enterolysis, is timeconsuming and high-risk and is reserved for situations in which conservative medical therapy fails in institutions with surgical expertise in this area. Herein we review the available literature of the etiology, pathogenesis, diagnosis, and treatment of this rare, but potentially devastating disease. | Christopher J Danford Steven C Lin Martin P Smith Jacqueline L Wolf | 2018 | World Journal of Gastroenterology2018，24，28： | 13 |
| 2 | Osteoporosis in primary biliary cholangitis显示文摘Primary biliary cholangitis(PBC) is an autoimmune cholestatic liver disease with multiple debilitating complications. Osteoporosis is a common complication of PBC resulting in frequent fractures and leading to significant morbidity in this population, yet evidence for effective therapy is lacking. We sought to summarize our current understanding of the pathophysiology of osteoporosis in PBC, as well as current and emerging therapies in order to guide future research directions. A complete search with a comprehensive literature review was performed with studies from Pub Med, EMBASE, Web of Science, Cochrane database, and the Countway Library. Osteoporosis in PBC is driven primarily by decreased bone formation, which differs from the increased bone resorption seen in postmenopausal osteoporosis. Despite this fundamental difference, current treatment recommendations are based primarily on experience with postmenopausal osteoporosis. Trials specific to PBC-related osteoporosis are small and have not consistently demonstrated a benefit in this population. As it stands, prevention of osteoporosis in PBC relies on the mitigation of risk factors such as smoking and alcohol use, as well as encouraging a healthy diet and weight-bearing exercise. The primary medical intervention for the treatment of osteoporosis in PBC remains bisphosphonates though a benefit in terms of fracture reduction has never been shown. This review outlines what is known regarding the pathogenesis of bone disease in PBC and summarizes current and emerging therapies. | Christopher J Danford Hirsh D Trivedi Konstantinos Papamichael Elliot B Tapper Alan Bonder | 2018 | World Journal of Gastroenterology2018，24，31： | 13 |
| 3 | Risk factors for hepatocellular carcinoma in cirrhosis due to nonalcoholic fatty liver disease: A multicenter, case-control study显示文摘AIM To identify risk factors associated with hepatocellular carcinoma(HCC), describe tumor characteristics and treatments pursed for a cohort of individuals with nonalcoholic steatohepatitis(NASH) cirrhosis. METHODS We conducted a retrospective case-control study of a well-characterized cohort of patients among five liver transplant centers with NASH cirrhosis with(cases) and without HCC(controls).RESULTS Ninety-four cases and 150 controls were included. Cases were significantly more likely to be male than controls(67% vs 45%, P < 0.001) and of older age(61.9 years vs 58 years, P = 0.002). In addition, cases were more likely to have had complications of end stage liver disease(83% vs 71%, P = 0.032). On multivariate analysis, the strongest association with the presence of HCC were male gender(OR 4.3, 95%CI: 1.83-10.3, P = 0.001) and age(OR = 1.082, 95%CI: 1.03-1.13, P = 0.001). Hispanic ethnicity was associated with a decreased prevalence of HCC(OR = 0.3, 95%CI: 0.09-0.994, P = 0.048). HCC was predominantly in the form of a single lesion with regional lymph node(s) and distant metastasis in only 2.6% and 6.3%, respectively. Fifty-nine point three percent of individuals with HCC underwent locoregional therapy and 61.5% underwent liver transplantation for HCC. CONCLUSION Male gender, increased age and non-Hispanic ethnicity are associated with HCC in NASH cirrhosis. NASH cirrhosis associated HCC in this cohort was characterized by early stage disease at diagnosis and treatment with locoregional therapy and transplant. | Kathleen E Corey Samer Gawrieh Andrew S deLemos Hui Zheng Andrew E Scanga Jennifer W Haglund Jorge Sanchez Christopher J Danford Megan Comerford Krista Bossi Samina Munir Naga Chalasani Julia Wattacheril | 2017 | World Journal of Hepatology2017，9，7： | 0 |
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