Comparison of emergent versus elective laparoscopic common bile duct exploration for patients with or without nonsevere acute cholangitis complicated with common bile duct stones

Background

Laparoscopic common bile duct exploration (LCBDE) has already been established for the treatment of patients with common bile duct stones (CBDS) in elective situations. However, the effect of emergent LCBDE on those patients with nonsevere acute cholangitis has not been assessed. The aim of this study was to evaluate the effect of emergent LCBDE on patients with nonsevere acute cholangitis complicated with CBDS.

Methods

Seventy-two patients with CBDS admitted from January 2009 to December 2012 were included for this retrospective study. LCBDE of transductal approach for CBDS was performed to all patients. Thirty-seven patients underwent emergent LCBDE for nonsevere acute cholangitis and 35 patients underwent elective LCBDE. Duration of the procedure, complications, retained stone of bile duct, hospital stay, and total charges were compared between the two groups. In addition, the characteristics of patients underwent emergent LCBDE were also compared before and after surgery.

Results

There was no significant difference with regard to the diameter of common bile duct and number of CBDS from imaging and/or operative findings between the two groups. There was no conversion to open common bile duct exploration, no major bile duct injuries, and no mortality in both the group of patients. There was no significant difference in patients with or without acute or chronic cholecystitis, duration of surgery, overall hospital stay (16.41 ± 1.03 versus 14.54 ± 0.94, P > 0.05), and total charges (18,603 ± 1774.64 versus 14,951 ± 1257.09 Yuan in renminbi, P > 0.05) between the two groups. Four cases with retained stones were found in patients with emergent LCBDE and two in elective LCBDE patients. There were four cases of biliary leak in patients with emergent LCBDE and three cases in elective LCBDE group, respectively. However, there was no statistical difference between the two groups. The biliary leak was cured postoperatively after drainage. Control of septic symptoms was achieved in all patients after emergent LCBDE.

Conclusions

Our data indicated that emergent LCBDE is as safe and effective as elective LCBDE for the treatment of patients with nonsevere acute cholangitis complicated with CBDS.     

自身免疫性肝炎合并自身免疫性疾病的特征研究

目的 比较自身免疫性肝炎（AIH）合并原发性胆汁性胆管炎（PBC）或部分肝外自身免疫性疾病（autoimmune disease）患者与单纯AIH患者临床特点及并发症,为改善AIH患者的诊治提供参考。方法 收集1999年8月~2019年8月我院收治的AIH患者149例,根据合并症分为无合并病的AIH组（68例）、AIH合并PBC组（AIH-PBC 组,41例）及AIH合并肝外自身免疫性疾病组（AIH-肝外组,40例）,比较三组临床特点、并发症、肝纤维化/肝硬化进展情况。结果 ①AIH-PBC 组及AIH-肝外组年龄低于AIH组,差异有统计学意义（P＜0.05）;三组性别比较,差异无统计学意义（P＞0.05）。②三组共有的临床症状为瘙痒、黄疸、乏力、食欲不振及腹部不适,其中AIH-PBC组瘙痒症状患者多于AIH组、AIH-肝外组,差异有统计学意义（P＜0.05）;三组黄疸、乏力、食欲不振及腹部不适比较,差异无统计学意义（P＞0.05）。③AIH-PBC 组ALT低于AIH组及AIH-肝外组,ALP、GGT高于AIH组及AIH-肝外组,差异有统计学意义（P＜0.05）;AIH-肝外组的AST、DBIL高于AIH组及AIH-PBC 组,差异有统计学意义（P＜0.05）;AIH-肝外组IgG水平高于AIH组及AIH-PBC 组,差异有统计学意义（P＜0.05）。④三组ANA、ASMA、SLA、LKM-1阳性率比较,差异无统计学意义（P＞0.05）;AIH-PBC组AMA、AMA-M2 阳性率高于AIH组、AIH-肝外组,差异有统计学意义（P＜0.05）。⑤三组均以界面性肝炎和淋巴细胞浸润表现居多,其中AIH-PBC组胆管病变、胆汁淤积高于AIH组,差异有统计学意义（P＜0.05）。⑥三组并发症主要包括食管胃底静脉曲张/破裂出血、腹水、肝性脑病、肝癌、肝移植,组间比较,差异无统计学意义（P＞0.05）。⑦三组肝纤维化/肝硬化发生率比较,差异无统计学意义（P＞0.05）;但AIH-PBC组2~5年肝纤维化/肝硬化进展率高于AIH组及AIH-肝外组,差异有统计学意义（P＜0.05）。结论 合并PBC的AIH患者比单纯AIH患者诊断年龄早,肝脏炎症反应轻、胆管病变重;比AIH及合并肝外自身免疫性疾病更易出现瘙痒、胆汁淤积更重、胆管损伤更严重,且肝纤维化/肝硬化速度更快。AMA、AMA-M2可作为AIH合并PBC的鉴别指标。另外,AIH合并肝外自身免疫性疾病常存在肝功能损害,IgG对其具有提示意义。     

The clinical spectrum of IgG4-related disease

IgG4-related disease (IgG4-RD) is an emerging immune-mediated disease with the capability of involving essentially any organ. The epidemiology of this disease has not been explored in detail. A majority of patients reported in the literature to date are from Japan, but the condition has been described all across the world and there is no strong evidence to suggest a predilection for Asian populations. The mean age at diagnosis is approximately 60 years and there is a decided male predominance for many clinical features, with an overall male:female ratio of 8:3. A cardinal feature of IgG4-RD is single or multiple organ swelling that often raises concern for malignancy. IgG4-RD should be suspected in patients presenting with unexplained enlargement or swelling of one or more organs. Presenting features vary substantially according to the specialty to which patients present first; in addition, the disease can be diagnosed unexpectedly in pathological specimens or identified incidentally on radiology studies. Involvement of major organs is common and IgG4-RD may lead to organ failure, particularly in the pancreas, liver and biliary tree, kidneys, thyroid gland, lungs, and aorta. The diagnosis of IgG4-RD relies on the coexistence of various clinical, laboratory and histopathological findings, although none is pathognomonic by itself.     

Romiplostim treatment allows for platelet transfusion‐free liver transplantation in pediatric thrombocytopenic patient with primary sclerosing cholangitis

Thrombocytopenia is a major risk factor for cirrhotic liver disease. Patients with thrombocytopenia may have esophageal or gastric varices secondary to portal hypertension, leading to variceal bleeding which exposes the liver to further damage. Here, we present a female pediatric patient with PSC and CD, whose progressive thrombocytopenia was successfully controlled by romiplostim, a TPO receptor agonist. The patient developed bloody diarrhea at four yr of age, and was subsequently diagnosed with PSC and CD when seven yr old. While CD was well‐controlled by immunomodulators, the patient's thrombocytopenia gradually progressed resulting in petechiae (platelet count of 11 × 10⁹/L) when she was 10 yr and four months old. She responded poorly to immunoglobulin and corticosteroids. Weekly subcutaneous injection of romiplostim was therefore initiated, and platelet counts were maintained over at 50 × 10⁹/L. She was able to undergo successful LDLT without platelet transfusion seven months after the initiation of romiplostim. Romiplostim was not required after LDLT with improved platelet counts. This case report suggests that romiplostim may be effective in the treatment of thrombocytopenic children with liver cirrhosis and portal hypertension, and in eliminating the need for platelet transfusion during the peri‐transplant period.     

Role of liver biopsy in autoimmune liver disease

This article will review histological aspects of three chronic liver diseases – autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) – in which autoimmune mechanisms are thought to be involved. The changing role of liver biopsy in the diagnosis and management of patients with autoimmune liver disease will also be discussed. In the case of autoimmune hepatitis, histological assessments remain important in establishing a diagnosis, identifying prognostic features and monitoring therapeutic responses. By contrast, for many patients with PBC and PSC a diagnosis can now be made on the basis of biochemical, serological and/or radiological findings alone and histological confirmation may not be required. Liver biopsy can still be used to assess disease severity in such cases and remains important in establishing a diagnosis in patients with atypical features (e.g. AMA-negative PBC or the small-duct variant of PSC). Liver biopsy is also increasingly used in the assessment of patients suspected to have “overlap syndromes” involving AIH and PBC or PSC.     

原发性胆汁性胆管炎易感基因多态性的研究现状

原发性胆汁性胆管炎(PBC)是一种慢性进行性肝内胆汁淤积的自身免疫性疾病,临床特点以大量抗线粒体抗体(AMA)的产生和胆管上皮细胞的损伤为特征。PBC的病因和发病机制尚未完全明确,可能与遗传和环境等相互作用所导致的异常自身免疫反应有关。PBC的家族聚集性、女性患病率明显高于男性以及同卵双胞胎患病的高度一致性,均提示了遗传易感性在PBC的发生和发展中起着重要的作用。全基因组关联分析(GWAS)以及病例对照研究已经鉴定出与PBC相关的各种人类白细胞抗原(HLA)和非HLA等位基因,并证实了PBC的种族差异性。本文综述了可能与PBC相关的易感基因多态性的研究现状。     

原发性胆汁性胆管炎患者血清核因子相关因子2、血红素加氧酶1的含量与熊去氧胆酸短期应答的相关性

目的观察核因子相关因子2(Nrf2)、血红素加氧酶1(HO-1)在原发性胆汁性胆管炎(PBC)患者治疗前后血清中的水平变化,并探讨二者与熊去氧胆酸(UDCA)短期疗效的相关性。方法纳入山西医科大学第一医院PBC患者及健康对照者各80例。收集PBC患者治疗前后的临床资料及血清样本,采用ELISA法检测样本中Nrf2及HO-1的含量,硫代巴比妥酸法、黄嘌呤氧化酶法分别检测丙二醛(MDA)及超氧化物歧化酶(SOD)的含量,并进一步分析UDCA治疗前后PBC患者血清中Nrf2、HO-1的水平变化及临床意义。结果治疗前PBC患者血清Nrf2、HO-1含量分别为(626.07±103.95)U/L、(16.62±5.06)U/L显著高于健康对照者[分别为(164.45±35.12)U/L、(11.74±2.0)U/L],差异均有统计学意义(P均<0.001);与治疗前比较,UDCA治疗后1个月PBC患者血清中Nrf2[(754.30±104.36)U/L]、HO-1[(22.60±5.51)U/L]含量显著增加(P均<0.001),肝功能各项指标也得到改善(P均<0.001)。患者治疗前血清中Nrf2水平(r=0.751,P=0.012)、HO-1水平(r=0.621,P=0.038)与治疗效果均呈正相关。以治疗前Nrf2=586.17 U/L作为阈值,预测的UDCA短期疗效的敏感度为84.6%,特异度为77.5%,曲线下面积为0.824(P<0.05);以治疗前HO-1=14.92 U/L作为阈值,预测的UDCA短期疗效的敏感度为88.5%,特异度为75.0%,曲线下面积为0.861(P<0.05)。结论Nrf2、HO-1在PBC患者疾病过程中发挥重要作用,二者在血清中的基线水平及动态变化同UDCA疗效相关,可以提示PBC患者对UDCA短期治疗的应答情况。     

Value of posttransplant protocol biopsies in 2 biliary autoimmune liver diseases: A step toward personalized immunosuppressive treatment

The value of protocol liver graft biopsies with good liver function was evaluated in patients with primary sclerosing cholangitis (PSC) or primary biliary cholangitis (PBC).A total of 250 protocol liver biopsy reports from 182 PSC and PBC patients were compared. Overall histopathological findings and those leading to changes in immunosuppression therapy were retrospectively analyzed.The mean time to first protocol biopsy after transplantation was 5.5 (±4.5) years for PSC patients and 9.3 (±6.6) years for PBC patients. More than 1 abnormal histopathological parameter was found in 43% and 62% of PSC and PBC patients, respectively. However, the histology was interpreted as normal by the pathologist in 78% of PSC and 60% of PBC patients. Immunosuppression therapy was reduced in 10% and increased in 6% patients due to protocol biopsy findings. Biopsies leading to increased immunosuppression therapy had more portal (P = .004), endothelial (P = .008), interphase (P = .021), and lobular (P = .000) inflammation.Mild histopathological findings were frequently found in the protocol biopsies despite the normal biochemistry. PBC patients had more histological abnormalities than those transplanted due to PSC; however, PBC patients had longer follow-up times. Immunosuppression therapy could be safely increased or decreased according to protocol biopsy findings after multidisciplinary meeting discussions.