Sclerosing polycystic adenosis of parotid gland: A unique report of two cases occurring in two sisters

Sclerosing polycystic adenosis (SPA) of salivary glands is a tumorous lesion of salivary glands, with clinical presentation of a slow-growing mass characterized by a combination of histological features, some of which are reminiscent of mammary fibrocystic disease. SPA is mostly unifocal, but rarely may be multifocal and/or bilateral. Recurrences have been reported in up to 19% of cases. Although originally considered pseudoneoplastic, the occurrence of “dysplasia” and carcinoma in situ of ductal epithelium, and recent evidence of clonality suggest a possible neoplastic nature. Herein we describe, for the first time, two cases of SPA in two sisters (7 and 33 years old). The younger patient experienced multiple recurrences. This is the first report of familial occurrence of SPA, suggesting a possible genetic background.     

超声引导下注射新型泡沫硬化剂治疗大隐静脉曲张

目的 观察超声引导下注射新型泡沫硬化剂治疗大隐静脉曲张的短期疗效.方法 对无深静脉及交通支静脉功能不全的重度大隐静脉曲张40条患肢(38例患者,其中2例为双下肢大隐静脉曲张),在超声引导下注射新型泡沫硬化剂Fibro-Vein对大隐静脉主干进行硬化治疗.结果 40条患肢均在超声引导下注射治疗成功,7条(7/40)大隐静脉于治疗后1个月复诊时实施了第2次注射治疗.随访1～12个月,全部患者(包括经2次治疗者)下肢活动后酸胀、乏力消失;下肢明显曲张畸形的静脉消失,其中8条(8/40)患肢远端存在局限性轻度曲张的静脉;无严重并发症发生.术后3个月复查彩色多普勒超声显示,曲张的大隐静脉较治疗前变细,22条(22/40)治疗大隐静脉的部分血管段实变,18条(18/40)静脉壁回声增强,并可见不规则、不均质中等实性回声紧密黏附于血管壁,挤压远端肢体时管腔内可见流沙样缓慢流动的血流信号,其中8条静脉(8/18)有少量反流.结论 超声引导下注射新型泡沫硬化剂治疗大隐静脉曲张方便、有效、痛苦小、无瘢痕形成、无严重并发症,可重复进行,近期疗效佳,是一种治疗与美容兼备的微创治疗方法.     

鼻,咽,喉部血管瘤42例治疗分析

鼻，咽，喉部毛细血管瘤１３例，采用Ｎｄ－ＹＡＧ激光治疗１～２次，瘤体灼除。随访１年以上，无并发症及复发。２９例海绵状血管瘤，瘤体均在１ｃｍ直径范围以上，分别采用Ｎｄ－ＹＡＧ激光治疗９例，硬化剂鱼肝油酸钠治疗８例，注射硬化剂鱼肝油酸钠同时加用Ｎｄ－ＹＡＧ激光治疗１２例。其疗效对比发现，硬化剂同时加用Ｎｄ－ＹＡＧ激光治疗组疗效好，疗效短，无并发症，随访年以上无复发。     

IgG4-related sclerosing disease

Based on histological and immunohistochemical exami- nation of various organs of patients with autoimmune pancreatitis （AIP）, a novel clinicopathological entity of IgG4-related sclerosing disease has been proposed. This is a systemic disease that is characterized by extensive IgG4-positive plasma cells and T-lymphocyte infiltration of various organs. Clinical manifestations are apparent in the pancreas, bile duct, gallbladder, salivary gland, retroperitoneum, kidney, lung, and prosrate, in which tissue fibrosis with obliterative phlebitis is pathologically induced. AlP is not simply pancreatitis but, in fact, is a pancreatic disease indicative of IgG4- related sclerosing diseases. This disease includes AlP, sclerosing cholangitis, cholecystitis, sialadenitis, retro-peritoneal fibrosis, tubulointerstitial nephritis, interstitial pneumonia, prostatitis, inflammatory pseudotumor and lymphadenopathy, all IgG4-related. Most IgG4-related sclerosing diseases have been found to be associated with AlP, but also those without pancreatic involvement have been reported. In some cases, only one or two organs are clinically involved, while in others, three or four organs are affected. The disease occurs predominantly in older men and responds well to steroid therapy. Serum IgG4 levels and immunos-taining with anti-IgG4 antibody are useful in making the diagnosis. Since malignant tumors are frequently suspected on initial presentation, IgG4-related sclerosing disease should be considered in the differential diagnosis to avoid unnecessary surgery.     

164例自身免疫性肝病临床分析

目的 分析比较自身免疫性肝炎（autoimmune hepatitis，AIH）、原发性胆汁性肝硬化（primary biliary cirrhosis，PBC）、原发性硬化性胆管炎（primary sclerosing cholangltis）及其重叠综合征的临床特点、生化特征和治疗反应，提高对自身免疫性肝病的认识。方法对77例AIH患者、46例PBC患者、11例PSC患者和30例PBC-AIH重叠综合征患者的临床及实验室检查资料进行回顾性分析。结果除PSC外，大多数自身免疫性肝病多发于中年女性，从出现症状到明确诊断平均需要2．5年。AIH、PBC-AIH重叠患者具有较高的转氨酶，PBC、PSC具有较明显的GGT、ALP升高。临床表现上AIH、PBC、PSC、AIH-PBC黄疸发生率分别为84％、78％、90％和67％，皮肤瘙痒的发生率分别为43％、56％、81％和60％。PSC和AIH-PBC具有较高的AIH评分，27％的PSC患者和33％AIH-PBC的评分达到可能的AIH。合理应用UDCA和免疫抑制剂可使90％的PBC和AIH患者症状在六个月内得到缓解、肝功能恢复明显改善。结论 AIH、PBC-AIH的肝功能异常以转氨酶升高为主，PBC、PSC以胆汁淤积为主。应用AIH评分系统诊断可能的AIH时应注意鉴别PSC及其它自身免疫性肝病。UDCA和免疫抑制剂可改善绝大多数患者的症状和肝功能异常。     

Duct-to-Duct Biliary Anastomosis Yields Similar Outcomes to Roux-en-Y Hepaticojejunostomy in Liver Transplantation for Primary Sclerosing Cholangitis

Background:

While Roux-en-Y hepaticojejunostomy (RYH) is the common anastomotic technique for liver transplantation (LT) in patients with primary sclerosing cholangitis (PSC), duct-to-duct (DD) reconstruction may be used if the recipient common bile duct is normal. There are conflicting observational data on the rate of success of DD reconstruction versus RYH, in PSC.

Objectives:

The aim of this study was to assess the safety and efficacy of DD anastomosis, compared to RYH reconstruction, among adults transplanted for PSC.

Patients and Methods:

All adult patients, who underwent primary LT for PSC between 1990 and 2012, were evaluated, according to type of biliary reconstruction. Recipient and graft survival, postoperative medical and surgical complications, and postoperative resource utilization rates were compared between the two groups.

Results:

Totally, 73 patients fulfilled the inclusion criteria. Of them, 58 had RYH and 15 had DD reconstruction. A total of 53 subjects (73%) were male, with the mean age ± standard deviation at LT of 43.3 ± 14.4 years. Rates of recipient mortality, graft failure, biliary complications, acute cellular rejection, and reoperation were similar in both groups. Postoperative cholangiography was used more frequently in patients with DD reconstruction (33.3% vs. 8.6%, P = 0.026).

Conclusions:

In selected recipients with PSC, DD reconstruction is a safe and efficacious technique, with long-term clinical outcomes comparable to RYH.     

以硬化性胆管炎为首发表现的儿童朗格罕细胞组织细胞增生症1例并文献复习

目的 探讨以硬化性胆管炎为首发表现的儿童朗格罕细胞组织细胞增生症(LCH)的临床特点,治疗及预后.方法 对我院收治的1例以硬化性胆管炎为首发表现的LCH患儿的病历资料进行回顾性总结,并复习相关文献.结果 患儿18个月,表现为多系统受累、高危组的LCH.临床以黄疸、皮疹为首发症状,经LCHⅢ方案化疗后未见好转,最终放弃治疗.结论 以硬化性胆管炎为首发表现的LCH比较罕见,临床可以黄疸为首发症状,预后差,熊去氧胆酸可改善生化指标,合并严重肝损害时可选择肝移植.     

Waitlist mortality in patients with autoimmune liver diseases

Introduction and ObjectivesAutoimmune liver diseases such as autoimmune hepatitis, primary biliary cholangitis, and primary sclerosing cholangitis are the primary indication for ～24% of total liver transplants. The liver transplant allocation system is currently based upon the Model for End-Stage Liver Disease and it often underestimates the severity of autoimmune liver diseases. We aim to compare the rate of adverse waitlist removal among patients with all autoimmune liver diseases and other indications for liver transplant in the Model for End-Stage Liver -Na era.Materials and MethodsUsing the United Network for Organ Sharing database, we identified all patients listed for liver transplant from 2016 to 2019. The outcome of interest was waitlist survival defined as the composite outcome of death or removal for clinical deterioration. Competing risk analysis was used to evaluate the waitlist survival.ResultsPatients with autoimmune hepatitis had a higher risk of being removed from the waitlist for death or clinical deterioration (SHR 1.37, 95% CI 1.08–1.72; P<0.007), followed by primary biliary cholangitis (SHR 1.34, 95% CI 1.07–1.68; P<0.011).ConclusionsHigh waitlist death or removal for clinical deterioration was observed in patients with PBC and AIH when compared to other etiologies. It may be useful to reassess the process of awarding MELD exception points to mitigate such disparity.     

Macrophages contribute to the pathogenesis of sclerosing cholangitis in mice

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