原发性胆汁性肝硬化的治疗

原发性胆汁性肝硬化(PBC)是一种慢性肝内胆汁淤积性疾病.熊去氧胆酸(UDCA)是各种指南推荐且唯一经FDA批准的治疗PBC安全有效的药物.UDCA治疗对PBC患者的肝硬化进展、远期预后和生存率密切相关.对UDCA治疗无效患者应如何处理,目前尚无统一的方案.对乏力和瘙痒等症状的改善有助于提高PBC患者的生活质量.间充质干细胞移植可能对自身免疫性肝病有效,肝移植仍是治疗终末期PBC的唯一有效的方法.     

原发性胆汁性胆管炎患者中枢神经系统改变相关研究进展

原发性胆汁性胆管炎(PBC)是一种以胆汁淤积为主要表现的自身免疫性肝病,好发于中老年女性。大约50%的患者伴有乏力和瘙痒,20%的患者伴有抑郁或者情绪的改变。近年有研究证明,PBC患者非特异性症状如乏力、瘙痒以及认知改变等症状与中枢神经系统结构和功能改变存在联系。通过大脑影像学改变及早期发现临床前期的PBC患者,可能成为该病早期诊断的检查手段。     

老年瘙痒性皮肤病患者生活质量调查

目的研究瘙痒性皮肤病对老年患者生活质量的影响。方法采用皮肤病生活质量指数量表,对428例老年瘙痒性皮肤病患者和50例健康对照人群进行调查问卷。结果瘙痒程度越重,生活质量评分越高(5.01±4.44～19.86±4.72,P<0.000 1),患者实际生活质量越差,瘙痒控制后患者生活质量明显提高(16.24±5.67,7.85±2.19,P<0.000 1);不同疾病对老年患者生活质量影响不同,其中以药疹、接触性皮炎、慢性湿疹、慢性荨麻疹最为严重。结论瘙痒性皮肤病明显降低老年患者生活质量,瘙痒程度越重患者生活质量越低。     

原发性胆汁性胆管炎预后的影响因素

原发性胆汁性胆管炎(PBC)属于慢性胆汁淤积性自身免疫性肝病,若不予及时有效治疗可进展至肝纤维化、肝硬化,甚至终末期肝病。PBC治疗主要目的是预防终末期肝病的发生,并改善相关临床症状,提升患者生活质量。及时准确地风险分层可使难治性PBC患者受益。准确评估PBC患者的预后对于临床治疗至关重要。从宿主因素、实验室检查、药物治疗等方面综述近十年PBC预后因素的研究进展。     

浅谈胆汁淤积性肝病瘙痒的治疗

正胆汁淤积性肝病导致的瘙痒症状对患者来说常常难以忍受,临床上也缺乏有效的治疗手段。尽管大部分胆汁淤积性肝病都会引起瘙痒,包括原发性胆汁性胆管炎(PBC)、原发性硬化性胆管炎(PSC)、妊娠肝内胆汁淤积症(ICP)、遗传性或药物性胆汁淤积,在研究瘙痒症时我们仍把重点放在PBC。据统计,70%PBC患者存在瘙痒症状,而其中只有极少部分症状严重,这些人通常对药物治疗无应答[1]。现有的临床推荐治疗药     

原发性胆汁性胆管炎合并骨质疏松症的诊断及处理

原发性胆汁性胆管炎(PBC)是一种慢性进行性胆汁淤积性肝病,多发于中老年女性,最终可导致肝硬化和肝衰竭。骨质疏松症是PBC患者的常见并发症,表现为骨量减少,骨折易感性增加。骨质疏松及其所致骨折严重影响PBC患者的生活质量,而随着PBC治疗策略的进步和人们预期寿命的增加,对PBC患者合并骨质疏松症的早期诊断及防治则显得尤为重要。现对PBC患者合并骨质疏松症的流行病学、发病机制及诊疗现状进行简要总结,同时提出目前面临的挑战及未来的研究方向。     

补气活血汤治疗原发性胆汁性肝硬化28例

正原发性胆汁性肝硬化(PBC)是一种较为常见的慢性胆汁淤积性肝病,熊去氧胆酸(UDCA)是目前治疗PBC的主要药物~([1]),但有报道称UDCA并不能延长PBC患者的生存期,也不能改善其乏力、瘙痒等临床症状~([2])。为此,我们采用UDCA联合补气活血汤治疗PBC获得良好治疗效果,现报道如下。1资料与方法1.1一般资料51例患者为2014年6月至2015年7月我院     

一项关于回肠胆汁酸转运体抑制剂GSK2330672对原发性胆汁性胆管炎瘙痒症状影响的Ⅱa期随机、双盲、安慰剂对照、交叉研究

<正>【据《Lancet》2017年2月报道】题:一项关于回肠胆汁酸转运体抑制剂GSK2330672对原发性胆汁性胆管炎瘙痒症状影响的Ⅱa期随机、双盲、安慰剂对照、交叉研究(作者Vinod SH等)高达70%的原发性胆汁性胆管炎(PBC)患者在病程中会出现瘙痒症状,而对其治疗又非常困难,熊去氧胆酸(UDCA)是治疗PBC的标准一线药物,但对瘙痒无效,因此临床上迫切需要新的治疗方法。回肠胆汁酸转运体(IBAT)抑制剂可阻断胆汁酸肠肝循环,但目前尚无临床研究评估其对PBC瘙痒症的影响。     

原发性胆汁性肝硬化的诊断与治疗

原发性胆汁性肝硬化(PBC)是自身免疫介导的慢性胆汁淤积性疾病。介绍了PBC的病因、发病机制、临床特征及治疗进展。对早期诊断、密切监测疾病并指导规范治疗,改善PBC患者的生活质量及预后具有重要意义。     

胆汁淤积性肝病相关并发症的处理

正胆汁淤积性肝病(cholestatic liver disease,CLD)是指由肝脏原因引起的胆汁形成、分泌和(或)排泄障碍,使胆汁流淤积于肝胆系统而不能正常地流向肠道的疾病,临床上种类较多,如原发性胆汁性胆管炎(PBC)、原发性硬化性胆管炎(PSC)、药物性胆汁淤积等。CLD常见的并发症有瘙痒、疲劳、骨质疏松、认知障碍和脂溶性维生素缺乏等。1瘙痒1.1瘙痒的定义及其发病机制瘙痒是包括原发性胆汁性肝硬化(PBC)和PSC等胆汁淤积性肝病患者的常见症状~([1]),是指皮肤产生的一种刺激且不愉快的感觉,并容易激发搔抓     

Pruritus and fatigue in primary biliary cirrhosis

Pruritus and fatigue are the most common symptoms of patients with PBC, and both have marked negative impact on quality of life. Over the past decade, evidence has emerged supporting a role of the central nervous system in the pathogenesis of these two common manifestations of PBC. There is no evidence that the pruritus of cholestasis is mediated in the skin. Clinical and laboratory data do support a role of the opioid neurotransmitter system in the mediation of the pruritus of cholestasis; a central mechanism has been proposed. Treatment with opiate antagonist is thus a specific alternative. Studies of the behavioral consequence of the pruritus of cholestasis, scratching activity, allow for the design of clinical trials with objective end-points. The etiology of fatigue is unknown. A central component is being considered. The identification of objective alterations in fatigue and the adoption of a definition that incorporates the perception and the behavioral consequences of fatigue should facilitate the development of objective methodology. The potential role of various neurotransmitter systems, including the serotonin system and the opioid system, in the mediation of the fatigue of PBC seems to merit further investigation.     

Successful use of the Molecular Adsorbent Recirculating System (MARS) in a patient with primary biliary cirrhosis (PBC) and treatment refractory pruritus

Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease often associated with severe pruritus. Despite maximal medical management pruritus often persists and has a detrimental effect on quality of life. For patients that are refractory to all medical treatments, more invasive approaches have been tried. Recently, a new extracorporeal hemodiafiltration method, Molecular Adsorbent Recirculating System (MARS), has been described. Based on the hypothesis that hydrophobic, protein-bound metabolites play a major role in the development of liver failure, this device uses an albumin enriched dialysate to facilitate the removal of albumin bound toxins. Aim: To assess the safety and efficacy of a single MARS treatment on the pruritus score in a patient with PBC and treatment refractory pruritus. Design/Patients: A 61-year-old female patient with biopsy proven PBC, who had been accepted on our liver transplantation waiting list because of treatment refractory pruritus, was subjected to a single MARS treatment. Results: At the end of a single 8 h MARS treatment session pruritus completely disappeared. Not unexpectedly, however, this effect was only short lived. Except for a slight hypercalcemia no adverse events were observed. Conclusion: A single MARS treatment very effectively improved pruritus. Long-term repetitive treatment, however, might be necessary to sustain its effectiveness.     

Role of plasmapheresis in the treatment of severe pruritus in pregnant patients with primary biliary cirrhosis: case reports.

BACKGROUND: Primary biliary cirrhosis (PBC) may be associated with pruritus and, when present, may be accentuated during pregnancy. Several therapeutic modalities have been used to control itching caused by cholestasis, with variable responses. Drug therapies are ill-advised, particularly in early pregnancy. Plasmapheresis has been successful in controlling pruritus in patients with cholestasis. The use of plasmapheresis to alleviate severe life-threatening pruritus during pregnancy is reported in two patients with PBC. CASE PRESENTATIONS: Two patients with PBC presented during their second trimester of pregnancy with severe pruritus that did not respond to the anion exchange resin cholestyramine. Their symptoms were disabling to the point that one patient had suicidal ideation. Given the severity of their symptoms, multiple sessions of plasmapheresis were instituted with good control of pruritus. Both patients tolerated the procedure well and delivered healthy babies. CONCLUSION: Plasmapheresis is a relatively safe and rapidly effective treatment for severe pruritus during pregnancy in patients with PBC.     

Evaluation of fatigue in U.S. patients with primary biliary cirrhosis

OBJECTIVES: Fatigue, which may have a significant impact on quality of life, is the most common reported symptom in primary biliary cirrhosis (PBC). Multiple instruments to quantify fatigue and quality of life in liver disease have been validated, but have not been broadly applied to U.S. PBC patients. This study examines the extent of fatigue and its effect on quality of life in U.S. PBC patients. METHODS: Seventy patients with PBC were administered two validated questionnaires about quality of life (the Mayo version of the NIDDK-QA) and fatigue (the Fisk Fatigue Impact Score) and a proposed physical measure of fatigue in PBC (the grip strength test) on the day of routine physician visit. Nonparametric methods were employed. RESULTS: The fatigue and quality of life domain scores (physical functioning, liver symptoms, health satisfaction, Karnofsky index) discriminated between patients with and without self-reported fatigue (p < 0.05), as opposed to the grip strength results. Fatigue and quality of life domains correlated strongly with each other (r between 0.33 and 0.74, p相似文献   

Jaundice in non-cirrhotic primary biliary cirrhosis: the premature ductopenic variant

The clinical and pathological findings of four females with primary biliary cirrhosis (PBC) with an unusual and hitherto not well recognised course are reported. Patients suffered severe pruritus and weight loss with progressive icteric cholestasis which did not respond to such treatments as ursodeoxycholic acid and immunosuppressives. In all cases liver histology revealed marked bile duct loss without however significant fibrosis or cirrhosis. Further diagnostic studies and repeat biopsies confirmed the absence of liver cirrhosis as well as other potential causes of hyperbilirubinaemia. Comparison of the fibrosis-ductopenia relationship for our cases with that for a group of 101 non-cirrhotic PBC patients indicated that in the former the severity of bile duct loss relative to the amount of fibrosis was significantly higher. The proportion of portal triads containing an interlobular bile duct was 3%, 4%, 6%, and 10% compared with 45% (median; range 8.3--100%) for controls (p<0.001). Three patients received a liver transplant 6--7 years after the first manifestation of PBC because of progressive cholestasis, refractory pruritus, and weight loss, while the fourth patient is considering this option. In one case cirrhosis had developed at the time of transplantation while the others still had non-cirrhotic disease. These cases suggest that cholestatic jaundice in non-cirrhotic PBC may be secondary to extensive "premature" or accelerated intrahepatic bile duct loss. Although the extent of fibrosis may be limited initially, progression to cirrhosis appears to be inevitable in the long run. Despite intact protein synthesis and absence of cirrhotic complications, liver transplantation in the pre-cirrhotic stage for preventing malnutrition and to improve quality of life should be considered for these patients.     

Long-term efficacy of sertraline as a treatment for cholestatic pruritus in patients with primary biliary cirrhosis

OBJECTIVES: Generalized pruritus is a common complication of cholestatic liver diseases, although its pathogenesis remains elusive. Current treatments are often inadequate and may be poorly tolerated, so the clinician is sometimes faced with a patient in misery and no good therapeutic options. Because, in our experience, several patients with primary biliary cirrhosis (PBC) claimed that sertraline had improved their pruritus, we sought to determine whether sertraline use was associated with changes in pruritus medications or self-reported severity of pruritus in a large cohort of patients with PBC. METHODS: The self-reported severity of pruritus was followed prospectively in 40 patients with PBC for a mean of 7.5 +/- 1.3 yr. These data were then retrospectively examined to determine the effect of sertraline on pruritus in all subjects who had received sertraline at some time during the study. RESULTS: For 28 of 32 patients with pruritus, itching was stable or fluctuated slightly over the follow-up period. No patient experienced rapid progression of pruritus, and four patients experienced a sustained resolution of their pruritus. Ten subjects started sertraline and continued it long enough (>6 months) to determine its lasting effect on pruritus. Three of these individuals did not have significant pruritus before or after sertraline. Of the seven patients with pruritus, six (86%) recorded a significant reduction or resolution of pruritus in their weekly diaries and also decreased or completely stopped other medications for pruritus. CONCLUSIONS: Sertraline use is associated with an improvement in cholestatic pruritus. This novel observation implies that serotonergic fibers are important in regulating the perception of itch.     

男性老年瘙痒症患者瘙痒与生活质量关系的评价研究

目的分析评价男性老年瘙痒症患者瘙痒特点以及瘙痒与生活质量的关系。方法采用现况调查的研究方法,填写调查表、瘙痒评分表及生活质量量表,评价205名男性老年瘙痒症患者瘙痒的特点以及对生活质量的影响。结果男性老年瘙痒症患者发作部位以局限性瘙痒为主,其中胫前和阴囊瘙痒明显;发作特点以单一瘙痒为主;发作时间以冬季和晚上最为明显;瘙痒程度按其瘙痒评分越高,瘙痒程度越重,生活质量评分越高,其生活质量越差。结论老年瘙痒症降低了患者的生活质量,尤其影响夜间睡眠。     

Complications,symptoms, quality of life and pregnancy in cholestatic liver disease

Cholestatic liver diseases (CLDs) encompass a variety of disorders of bile formation and/or flow which generally result in progressive hepatobiliary injury and ultimately end‐stage liver disease. Many patients with CLD are diagnosed between the ages of 20‐50 years, a particularly productive period of life professionally, biologically and in other respects; it is not surprising, thus, that CLD is often associated with impaired health‐related quality of life (HRQOL) and uncertainty regarding implications for and outcomes of pregnancy. Primary sclerosing cholangitis (PSC) and primary biliary cholangitis (PBC) are the most prominent CLDs, both having considerable morbidity and mortality and representing major indications for liver transplantation. These disorders, as a consequence of their complications (eg ascites, hepatic osteodystrophy), associated conditions (eg inflammatory bowel disease) and symptoms (eg pruritus and fatigue), can significantly impair an array of domains of HRQOL. Here we review these impactful clinical aspects of PSC and PBC as well as the topics of fertility and pregnancy.     

C ASE R EPORT: Oral antioxidant therapy for the treatment of primary biliary cirrhosis: A pilot study

BACKGROUND: The symptoms of the chronic cholestatic liver disease primary biliary cirrhosis (PBC), in particular fatigue and chronic pruritus, adversely affect quality of life and respond only poorly to treatment. Recent studies have suggested that oxidative stress may play a role in tissue damage in cholestatic liver disease and may contribute to symptoms, such as fatigue. We have, therefore, examined, in an open-label pilot study, the therapeutic effects of antioxidant medication on the biochemistry and symptomatology of PBC. METHODS: Patients were randomized to 3 months treatment with a compound antioxidant vitamin preparation (Bio-Antox), four tablets daily (n = 11, group 1), or the combination of Bio-Quinone Q10 (100 mg) with Bio-Antox (n = 13, group 2). Biochemical and symptomatic responses were assessed at 3 months. RESULTS: Significant improvement in both pruritus and fatigue was seen in the patients in group 2. Mean itch visual analogue score improved from 2.4 +/- 3.0 to 0.4 +/- 0.7 post therapy (P < 0.05) while mean night itch severity score improved from 2.6 +/- 1.9 to 1.3 +/- 0.7 (P < 0.05). Nine of 13 of these patients reported less fatigue, while 10/13 showed an improvement in at least one domain of their Fisk Fatigue Severity Score. No significant improvement in itch and only limited improvement in fatigue were seen in the patients in group 1. No change in biochemical parameters was seen in either group. CONCLUSIONS: Antioxidant therapy, as a combination of Bio-Antox and Bio-Quinone Q10, may improve the pruritus and fatigue of PBC. This combination of therapy should be investigated further in a double-blind, placebo-controlled trial.     

Primary biliary cholangitis: perception and expectation of illness

An important tool to explore personal experience of symptoms, treatment and clinical outcome is stratification of illness perception in patients affected by PBC.AimTo assess the perception of illness in a cohort of Italian patients with PBC.MethodsBetween June and December 2019, a specific questionnaire was administered to a pool of 210 patients from 7 tertiary Italian centers, in order to identify and assess the patient's past history, symptoms and their impact on the quality of life, follow-up, treatment and perceived satisfaction of patients toward the provided care.ResultsFatigue, pruritus, and abdominal discomfort and sicca syndrome were present in 50.4%, 45%, 30.4% and 28.5% of patients, fatigue having the most impacting the daily-life. After a consultation with a specialist, the diagnosis of PBC was met within 18 months for 143 patients. Patients were mostly concerned about possible health problems that occur and in 25% of cases, symptoms had a negative impact on their life. Eighty percent of patients said they were satisfied with efficacy and tolerability of treatment, while 26% requested an improvement in the relationship with the specialist.ConclusionsThe results highlight the importance of both promoting timely referral to the specialist and facilitating communication between healthcare professionals and patients.