Risk factors of organ failure in cholangitis with bacteriobilia

AIM: To identify the risk factors for organ failure (OF) in cholangitis with bacteriobilia.METHODS: This study included 182 patients with acute cholangitis who underwent percutaneous transhepatic biliary drainage between January 2005 and April 2013. We conducted a retrospective analysis of comprehensive clinical and laboratory data.RESULTS: There were 24 cases (13.2%) of OF and five deaths (2.7%). Bile culture was positive for microbial growth in 130 out of 138 (94.2%) patients. In multivariate analysis of 130 patients with positive bile cultures, significant predictive factors for OF were the presence of extended-spectrum beta-lactamase (ESBL) organisms in blood cultures, pre-existing renal dysfunction, and choledocholithiasis as an etiology, with odds ratios of 15.376, 6.319, and 3.573, respectively. We developed a scoring system with a regression coefficient of each significant variable. The OF score was calculated using the following equation: (2.7 × ESBL organisms in blood cultures) + (1.8 × pre-existing renal dysfunction) + (1.3 × choledocholithiasis). This scoring system for predicting OF was highly specific (99.1%) and had a positive predictive value of 86.2%.CONCLUSION: ESBL organisms in blood cultures, pre-existing renal dysfunction, and choledocholithiasis are risk factors for OF in cholangitis with bacteriobilia. The OF scoring system may aid clinicians to identify a poor prognosis group.     

First case of IgG4-related sclerosing cholangitis associated with autoimmune hemolytic anemia

To our knowledge, patients with immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) associated with autoimmune hemolytic anemia (AIHA) have not been reported previously. Many patients with IgG4-SC have autoimmune pancreatitis (AIP) and respond to steroid treatment. However, isolated cases of IgG4-SC are difficult to diagnose. We describe our experience with a patient who had IgG4-SC without AIP in whom the presence of AIHA led to diagnosis. The patient was a 73-year-old man who was being treated for dementia. Liver dysfunction was diagnosed on blood tests at another hospital. Imaging studies suggested the presence of carcinoma of the hepatic hilus and primary sclerosing cholangitis, but a rapidly progressing anemia developed simultaneously. After the diagnosis of AIHA, steroid treatment was begun, and the biliary stricture improved. IgG4-SC without AIP was thus diagnosed.     

2008年非病毒性肝病临床进展回顾

回顾2008年关于肝豆状核变性、非酒精性脂肪肝、自身免疫性肝炎、原发性胆汁性肝硬化及原发性硬化性胆管炎在诊断、治疗及检测等方面的研究进展。     

胰岛素对急性重症胆管炎脓毒症大鼠骨骼肌代谢的影响

目的 探讨胰岛素对大鼠急性重症胆管炎（ACST）脓毒症状态下泛素系统介导骨骼肌蛋白质分解的调理作用。方法 20只雄性SD大鼠按月龄、体重同窝饲养,随机分为假手术组（SO）、ACST脓毒症模型组（AS）、低剂量胰岛素ACST脓毒症模型组（LIAS,胰岛素给予量为2.4 mU·kg-1·min-1）、高剂量胰岛素ACST脓毒症模型组（HIAS,胰岛素给予量4.8 mU·kg-1·min-1）,每组5只。所有大鼠均行正常血糖钳夹模型制备,并通过调节25%葡萄糖的输注率（GIR）将血糖维持在5～6 mmol/L的稳态;只有当血糖超过11.9 mmol/L时才需加用胰岛素。采用高效液相-色谱分析法检测伸趾长肌内3-甲基组氨酸（3-MH）;RT-PCR法检测伸趾长肌内编码泛素、蛋白酶体C2亚基的mRNA表达变化。结果 AS组大鼠伸趾长肌内3-MH含量为（3.69±0.20）nmol/g,较SO组（2.07±0.13）nmol/g显著升高（P＜0.001）;且显著高于HIAS组（2.39±0.21）nmol/g（P＜0.001）和LIAS组（2.87±0.19）nmol/g（P＜0.001）。HIAS组大鼠伸趾长肌内3-MH含量较LIAS组显著降低（P＜0.001）。AS组大鼠伸趾长肌内泛素mRNA表达较SO组明显升高,其相对表达量增加了19.70倍。LIAS组泛素mRNA相对表达量为6.96（范围为4.17～11.67）、HIAS组相对表达量为1.89（范围为1.43～2.48）,与AS组比较均明显下降（P=0.028,0.009）。HIAS组泛素mRNA表达水平低于LIAS组（P=0.009）。AS组大鼠伸趾长肌内蛋白酶体C2亚基mRNA表达较SO组明显升高,其相对表达量增加了191.34倍。LIAS组蛋白酶体C2亚基mRNA相对表达量为31.12（15.74～61.39）、HIAS组蛋白酶体C2亚基mRNA相对表达量为7.67（4.08～14.50）,与AS组比较表达均明显下降（P值均为0.009）。HIAS组C2亚基mRNA表达水平低于LIAS组（P=0.009）。结论 急性重症胆管炎脓毒症状态下,胰岛素可抑制骨骼肌蛋白降解,该作用可能是在基因水平通过抑制泛素-蛋白酶体通路表达和活化而实现的。     

Treatment of PBC—A step forward

Recent years have seen a growing interest in PBC within the scientific community, healthcare providers and industries, coupled with great advances in the understanding of the molecular and genetic basis and the natural history of the disease. Several disease‐modifying agents targeting the immune‐mediated response and bile‐acid therapies are at different stages of development, some with promising results. A new drug, obeticholic acid, has been recently registered in the US and Europe as a second‐line treatment in refractory PBC. International cohort studies have highlighted a disease heterogeneity, and so the need to provide patients with a more personalised management based on their risk of disease progression. Major challenges remain the development of surrogate endpoints in clinical trials acceptable to regulatory authorities, in a disease with a relatively low rate of events; and the development of clinical tools for patient's risk stratification and selection of those with greatest potential benefit from second‐line therapies.     

Significant influence of the primary liver disease on the outcomes of hepatic retransplantation

Background  There are many indications for hepatic retransplantation. Aim  To identify factors influencing retransplantation needs and outcomes. Patients and methods  Retransplantation records from January 1993 to March 2005 were analysed. Patient and disease characteristics and survival outcomes for retransplantation were compared between various groups. Results  Totally, 286 primary and 42 hepatic retransplantations were performed. Retransplantation indications included primary sclerosing cholangitis (PSC), primary biliary cirrhosis, chronic hepatitis C (HCV), chronic active hepatitis (CAH), and alcohol-related disease. Mean follow-up post-retransplantation was 31 ± 9 months. Actuarial patient survival at 3 months, 1 year, 3 years, 5 years, and at the end of study was 71.4, 69, 59.5, 54.7, and 50%, respectively. Early and late retransplantation had 1-year survival of 73 and 68.5%, respectively. Retransplantation need was significantly higher for PSC, HCV, and CAH. Conclusions  Hepatic retransplantation remains a successful salvage option for transplant complications; however, its need is significantly influenced by the primary liver disease.