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primary biliary cirrhosis degree assessment by acoustic radiation force impulse imaging and hepatic fibrosis indicators

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World Journal of Gastroenterology 2016年第22期22卷 5276-5284页

作者： Hai-Chun Zhang Rong-Fei Hu Ting Zhu Ling Tong Qiu-Qin Zhang Department of Ultrasound Huashan Hospital Baoshan Branch Affiliated to Fudan University Department of Gastroenterology Huashan Hospital Baoshan Branch Affiliated to Fudan University

AIM: To evaluate the assessment of primary biliary cirrhosis degree by acoustic radiation force impulse imaging (ARFI) and hepatic fibrosis indicators. METHODS: One hundred and twenty patients who developed liver cirrhosis secondary to primary biliary cirrhosis were selected as the observation group, with the degree of patient liver cirrhosis graded by Child-Pugh (CP) score. Sixty healthy individuals were selected as the control group. The four indicators of hepatic fibrosis were detected in all research objects, including hyaluronic acid (HA), laminin (LN), type III collagen (PC III), and type IV collagen (IV-C). The liver parenchyma hardness value (LS) was then measured by ARFI technique. LS and the four indicators of liver fibrosis (HA, LN, PC III, and IV-C) were observed in different grade CP scores. The diagnostic value of LS and the four indicators of liver fibrosis in determining liver cirrhosis degree with PBC, whether used alone or in combination, were analyzed by receiver operating characteristic (ROC) curve. RESULTS: LS and the four indicators of liver fibrosis within the three classes (A, B, and C) of CP scores in the observation group were higher than in the control group, with C class > B class > A class; the differences were statistically significant (P < 0.01). Although AUC values of LS within the three classes of CP scores were higher than in the four indicators of liver fibrosis, sensitivity and specificity were unstable. The ROC curves of LS combined with the four indicators of liver fibrosis revealed that: AUC and sensitivity in all indicators combined in the A class of CP score were higher than in LS alone, albeit with slightly decreased specificity; AUC and specificity in all indicators combined in the B class of CP score were higher than in LS alone, with unchanged sensitivity; AUC values (0.967), sensitivity (97.4%), and specificity (90%) of all indicators combined in the C class of CP score were higher than in LS alone (0.936, 92.1%, 83.3%).

关键词： Acoustic radiation force imaging technology Hepatic fibrosis index primary biliary cirrhosis Diagnostic value

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primary biliary cirrhosis in Brunei Darussalam

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Hepatobiliary & Pancreatic Diseases International 2010年第6期9卷 622-628页

作者： Vui Heng Chong Pemasiri Upali Telisinghe Anand Jalihal Gastroenterology and Hepatology Unit Department of Medicine Raja Isteri Pengiran Anak Saleha(RIPAS)Hospital Department of Pathology (Telisinghe PU) Raja Isteri Pengiran Anak Saleha(RIPAS)Hospital

BACKGROUND:primary biliary cirrhosis(PBC)is an uncommon autoimmune cholestatic disease that predominantly affects *** human leukocyte antigens(HLAs) have been reported to be associated with susceptibility for *** describe the profiles of PBC in Brunei Darussalam. METHODS:All patients with PBC(n=10)were identified from our prospective *** HLA profiles(n=9,PBC)were compared to controls(n=65)and patients with autoimmune hepatitis(n=13,AIH). RESULTS:All patients were women with a median age of 51 years(27-83)at *** prevalence rate of the disease was 25.6/million-population and the estimated incidence rate varied from 0 to 10.3/million-population per *** (41.15/million)and the indigenous(42.74/million)groups had higher prevalence rates compared to Malays(22.62/ million).The prevalence among female population was 54.6/ *** patients were referred for abnormal liver *** patients had symptoms at presentations: jaundice(20%),fatigue(20%),arthralgia(30%)and pruritus (20%).Serum anti-mitochondrial antibody was positive in 80%of the *** with AIH was seen in 30%.Liver biopsies(n=8)showed stage I(n=2),II(n=4)and III(n=2) *** were no significant differences in the HLA profiles between PBC and *** to the controls,PBC patients had significantly more HLA class I alleles specifically B7(P=0.003),Cw7(P=0.002)and Cw12(P=0.007)but not the class II *** a median follow-up of 23.5 months(2 to 108), all patients were alive without evidence of disease progression. CONCLUSIONS:PBC is also a predominant female disorder in our local setting and most had mild *** HLA profiles of our patients were different to what have been reported.

关键词： primary biliary cirrhosis cholestasis chronic liver disease human leukocyte antigen Southeast Asian

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primary biliary cirrhosis: Pathophysiology, clinical presentation and therapy

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World Journal of Hepatology 2015年第7期7卷 926-941页

作者： Treta Purohit Mitchell S Cappell Division of Gastroenterology and Hepatology William Beaumont Hospital Oakland University William Beaumont School of Medicine

primary biliary cirrhosis(PBC) is an autoimmune, slowly progressive, cholestatic, liver disease characterized by a triad of chronic cholestasis, circulating anti-mitochondrial antibodies(AMA), and characteristic liver biopsy findings of nonsuppurative destructive cholangitis and interlobular bile duct destruction. About 10% of PBC patients, however, lack AMA. A variant, called PBC-autoimmune hepatitis(AIH) overlap, is characterized by the above findings of PBC together with findings of elevated serum alanine aminotransferase, elevated serum immunoglobulin G, and circulating anti-smooth muscle antibodies, with liver biopsy demonstrating periportal or periseptal, lymphocytic, piecemeal necrosis. PBC is hypothesized to be related to environmental exposure in genetically vulnerable individuals. It typically occurs in middle-aged females. Prominent clinical features include fatigue, pruritis, jaundice, xanthomas, osteoporosis, and dyslipidemia. The Mayo Risk score is the most widely used and best prognostic system. Ursodeoxycholic acid is the primary therapy. It works partly by reducing the concentration and injury from relatively toxic bile acids. PBC-AIH overlap syndrome is treated with ursodeoxycholic acid and corticosteroids, especially budesonide. Obeticholic acid and fibrate are promising new, but incompletely tested, therapies. Liver transplantation is the definitive therapy for advanced disease, with about 70% 10-year survival after transplantation. Management of pruritis includes local skin care, dermatologist referral, avoiding potential pruritogens, cholestyramine, and possibly opioid antagonists, sertraline, or rifaximin. Management of osteoporosis includes life-style modifications, administration of calcium and vitamin D, and alendronate. Statins are relatively safe to treat the osteopenia associated with PBC. Associated Sjogren's syndrome is treated by artificial tears, cyclosporine ophthalmic emulsion to stimulate tear production; and saliva substitutes, c

关键词： primary biliary cirrhosis Ursodeoxycholicacid cirrhosis Liver transplantation Cholestatic liverdisease

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primary biliary cirrhosis and primary Sclerosing Cholangitis:a Review Featuring a Women's Health Perspective

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Journal of Clinical and Translational Hepatology 2014年第4期2卷 266-284页

作者： Renée M. Marchioni Beery Haleh Vaziri Faripour Forouhar Division of Internal Medicine Department of Gastroenterology and Hepatology University of Connecticut Health Center Farmington CT USA Department of Pathology and Lab Medicine University of Connecticut Health Center Farmington CT USA

primary biliary cirrhosis (PBC) and primary sclerosing cho-langitis (PSC) are two major types of chronic cholestatic liver disease. Each disorder has distinguishing features and variable progression, but both may ultimately result in cirrhosis and hepatic failure. The following offers a review of PBC and PSC, beginning with a general overview of disease etiology, pathogenesis, diagnosis, clinical features, natural course, and treatment. In addition to commonly associated manifestations of fatigue, pruritus, and fat-soluble vitamin deficiency, select disease-related topics pertaining to women's health are discussed including metabolic bone disease, hyperlipidemia and cardiovascular risk, and preg-nancy-related issues influencing maternal disease course and birth outcomes. This comprehensive review of PBC and PSC highlights some unique clinical considerations in the care of female patients with cholestatic liver disease.

关键词： primary biliary cirrhosis primary sclerosing cholangitis Cholestasis Women's health Metabolic bone disease Hyperlipidemia Pregnancy Fatigue Pruritus Fat-soluble vitamin deficiency

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primary biliary cirrhosis in India

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Hepatobiliary & Pancreatic Diseases International 2006年第1期5卷 105-109页

作者： Shiv Kumar Sarin Rajnish Monga Bimaljeet Singh Sandhu Barjesh Chander Sharma Puja Sakhuja Veena Malhotra Department of Gastroenterology Department of Pathology G. B. Pant Hospital

BACKGROUND: primary biliary cirrhosis (PBC) is a rare cause of chronic liver disease in India. We analyzed the clinical, biochemical, serological and histological features of patients with PBC for over a 10-year period. METHODS: PBC was diagnosed by the presence of raised level of serum alkaline phosphatase (ALP), anti-mitochondrial antibody (AMA) positivity (1:40 dilution), and/or diagnostic liver histology. RESULTS:Fifteen female patients with mean age of 46.5±11 years were studied. Pruritis (80%) followed by jaundice (67%), skin changes (pigmentation, coarsening, xanthelesma and vitiligo) (67%) and fatigue (60%) were common symptoms. The mean duration of the symptoms was 3.5± 5.4 years (3 months to 20 years). Dryness of eyes was observed in only 2 patients. Hepatomegaly was noted in 87% of the patients and ascites at presentation in 40%. Mean levels of bilirubin and albumin at the time of diagnosis were 3.4±3.3 mg/dl and 3.5±0.8 g/dl, respectively. The level of serum ALP ranged from 54 to 2400 IU/L, with a median being 552 IU/L (2×ULN). In all the 15 patients with AMA positive, 8(53%) were also positive for either anti-nuclear or anti-smooth muscle antibodies. Two patients presented with persistently elevated SAP after an acute hepatitic illness. Liver biopsy was available in 13 patients, diagnostic of PBC Ⅱ & Ⅲ(8) and with evidence of cirrhosis (5). Associated autoimmune disorders were observed in 5 patients (33%). The mean time for follow-up was 26±21 months (1 to 87 months). In 4 deaths, 3 were due to liver related causes. CONCLUSION: PBC is a rare cause of chronic liver disease in India. PBC in India, unlike in the West, presents late, often with features of cirrhosis and decompensation.

关键词： primary biliary cirrhosis chronic cholestatic liver disease India

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CD4^(+)CD25^(+)but not CD4^(+)Foxp3^(+) T cells as a regulatory subset in primary biliary cirrhosis

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Cellular & Molecular Immunology 2010年第6期7卷 485-490页

作者： Dandan Wang Huayong Zhang Jun Liang Zhifeng Gu Qiang Zhou Xiangshan Fan Yayi Hou Lingyun Sun Department of Immunology and Rheumatology The Affiliated Drum Tower Hospital of Nanjing University Medical SchoolNanjingChina Department of Pathology The Affiliated Drum Tower Hospital of Nanjing University Medical SchoolNanjingChina Immunology Laboratory Nanjing University Medical SchoolNanjingJiangsuChina

Increasing evidence indicates a role for regulatory T cells(Tregs)in the immune response and in autoimmune diseases,but the role of Tregs and cytokines in autoimmune hepatic diseases remains largely unclear and controversial,especially in patients with primary biliary cirrhosis(PBC).This study was undertaken to investigate Tregs and different cytokines in the liver and peripheral blood of PBC *** found that these patients demonstrated a reduction of CD4^(+)CD25^(+) T cells but elevated CD4^(+)Foxp3^(+) T cells in peripheral blood mononuclear cells(PBMCs)and CD41 T *** percentage of CD4^(+)CD25^(+) T cells in PBMCs was negatively correlated with elevated plasma interferon(IFN)-c levels.A liver-specific analysis showed that the frequency of Foxp31 Tregs,transforming growth factor(TGF)-b1 and IFN-c were increased in PBC *** findings suggest that an imbalance between CD4^(+)CD25^(+) Tregs and cytotoxic cytokines plays a crucial role in the pathogenesis of PBC while the role of Foxp3 needs further investigation.

关键词： cytokines forkhead box P3 primary biliary cirrhosis regulatory T cells

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primary biliary cirrhosis-specific autoantibodies in first degree relatives of Greek primary biliary cirrhosis patients

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World Journal of Gastroenterology 2012年第34期18卷 4721-4728页

作者： Theodoros A Zografos Nikolaos Gatselis Kalliopi Zachou Christos Liaskos Stella Gabeta George K Koukoulis George N Dalekos Depart ment of Medicine and Research Laboratory of Internal Medicine Medical SchoolUniversity of Thessaly41110 LarissaGreece Department of Pathology Medical SchoolUniversity of Thessaly41110 LarissaGreece

AIM:To determine the prevalence and significance of primary biliary cirrhosis (PBC)-specific autoantibodies in firstdegree relatives (FDRs) of Greek PBC patients. METHODS:The presence of antimitochondrial antibodies (AMA) and PBCspecific antinuclear antibodies (ANA) were determined using indirect immunofluores-cence assays, dot-blot assays, and molecularly based enzyme-linked immunosorbent assays in 101 asymp-tomatic for liver-related symptoms FDRs of 44 PBCpatients. In order to specify our results, the same investigation was performed in 40 healthy controls and in a disease control group consisting of 40 asymptomatic for liver-related symptoms FDRs of patients with other autoimmune liver diseases namely, autoimmune hepati-tis-1 or primary sclerosing cholangitis (AIH-1/PSC). RESULTS: AMA positivity was observed in 19 (only 4 with abnormal liver function tests) FDRs of PBC patients and none of the healthy controls. The preva-lence of AMA was significantly higher in FDRs of PBC patients than in AIH-1/PSC FDRs and healthy controls [18.8%, 95% confidence interval (CI):12%-28.1% vs 2.5%, 95% CI:0.1%-14.7%, P = 0.01; 18.8%, 95% CI:12%-28.1% vs 0%, 95% CI: 0%-10.9%, P = 0.003, respectively]. PBC-specific ANA positivity was observed in only one FDR from a PSC patient. Multivariate analysis showed that having a proband with PBC independently associated with AMA positivity (odds ratio: 11.24, 95% CI:1.27-25.34, P = 0.03) whereas among the investigated comorbidities and risk factors, a positive past history for urinary tract infections (UTI) was also independently associated with AMA detection in FDRs of PBC patients (odds ratio:3.92, 95% CI:1.25-12.35,P = 0.02). CONCLUSION:In FDRs of Greek PBC patients, AMA prevalence is significantly increased and independently associated with past UTI. PBC-specific ANA were not detected in anyone of PBC FDRs.

关键词： primary biliary cirrhosis Antimitochondrial antibodies Anti-gp210 Anti-sp100 Liver autoimmunity

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Increased ΤGF-β3 in primary biliary cirrhosis: An abnormality related to pathogenesis?

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World Journal of Gastroenterology 2010年第40期16卷 5057-5064页

作者： Argyro Voumvouraki Mairi Koulentaki Maria Tzardi Ourania Sfakianaki Penelope Manousou George Notas Elias Kouroumalis University Hospital Department of Gastroenterology University of Crete Faculty of Medicine Liver Research Laboratory University of Crete Faculty of Medicine University Hospital Department of Pathology University of Crete Faculty of Medicine Laboratory of Experimental Endocrinology University of Crete Faculty of Medicine

AIM: To investigate the transforming growth factor-β (TGF-β) isoforms in the peripheral and hepatic venous blood of primary biliary cirrhosis (PBC) patients. METHODS: We examined TGF-β1, TGF-β2 and TGF-β3 (enzyme-linked immunosorbent assay), in 27 stage Ⅳ PBC patients (27 peripheral and 15 hepatic vein sera), 35 early (Ⅰ-Ⅱ) PBC and 60 healthy controls. As disease controls 28 hepatitis C virus (HCV) cirrhosis (28 peripheral and 17 hepatic vein serum), 44 chronic HCV hepatitis and 38 HCV-related hepatocellular carcinomas were included. We also tested liver tissue by immunohistochemistry to identify localization of TGF isoforms. RESULTS: TGF-β1 was significantly decreased in all cirrhotics (PBC Ⅲ-Ⅳ: median 13.4 ng/mL; range, 7.4-26.2, HCV cirrhosis: 11.6 ng/mL; range, 5.0-33.8), compared to controls (30.9 ng/mL; range, 20.9-37.8). TGF-β2 was increased in viral cirrhosis but not in PBC and chronic hepatitis. TGF-β3 (47.2 pg/mL; range, 27.0-79.7 in healthy controls) was increased in early and late PBC (Ⅰ-Ⅱ: 94.3 pg/mL; range, 41.5-358.6; Ⅲ-Ⅳ: 152.8 pg/mL; range, 60.4-361.2; P cirrhosis (37.4 pg/mL; range, 13.3-84.0; P < 0.05). Hepatic vein TGF-β levels were analogous to those in peripheral blood. Immunohistochemistry identified all isoforms in portal tract lymphocytes, sinusoidal cells and cholangiocytes. TGF-β3 was additionally overexpressed in hepatocytes in PBC patients. CONCLUSION: The serum profile of TGF-β isoforms is different in cirrhotics. Increased TGF-β3 is characteristic of PBC. These findings may be related to the immunological abnormalities of PBC.

关键词： Transforming growth factor-β primary biliary cirrhosis Liver fibrosis cirrhosis

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Genetic association of cytokines polymorphisms with autoimmune hepatitis and primary biliary cirrhosis in the Chinese

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World Journal of Gastroenterology 2005年第18期11卷 2768-2772页

作者： Lie-YingFan xiao-QingTu TU,YeZhu ThomasPfeiffer RalphFeltens WinfriedStoecker Ren-QianZhong DepartmentofMedicineLaboratory EastHospitalTongJiUniversityShanghai200120China TheCenterofClinicalImmunology ChangzhengHospitalSecondMilitaryMedicalUniversityShanghai200120China MedicalLaboratorydiagnosticGmbH LeubeckGermany

AIM: To characterize gene polymorphism of several cytokine gene in-patients with AIH and PBC and to analyze the difference of the polymorphism distribution between Chinese patients and healthy ***: The study population consisted of 62 patients with AIH, and 77 patients with PBC. The genetic profile of four cytokines was analyzed by restriction fragmentlength polymorphism after specific PCR amplification (PCR-RFLP) or sequence-specific primers PCR (SSP-PCR). The analyzed gene polymorphism included interleukin-1 (IL-1) (at position +3 953 and IL-1RN intron 2), IL-6 (atposition -174), IL-10 promoter (at position -1 082, -819, and -592). The control group consisted of 160 healthyblood ***: The majority of Chinese people including patients and healthy controls exhibited IL-1B 1,1genotype, and there was no significant difference in AIH, PBC patients and controls. There were highly statistically significant differences in the distribution of the IL-1RN gene polymorphism between the patients with PBCcompared with controls. The frequency of IL-1RN 1,1was significantly higher (90.9% vs 79.4%, P = 0.03)and the frequency of IL-1RN 1,2 was significantly lower in PBC patients (6.5% vs 17.5%, P = 0.01). No statistical difference was observed between AIH patients and controls. All of the 160 healthy controls and 62 cases of AIH patients exhibited IL-6-174GG genotype, and there were four cases, which expressed IL-6-174GC genotype in 77 cases of PBC patients. The frequency of IL-6-174GC was markedly significantly higher in PBC patients compared with controls (5.2% vs 0%, P = 0.004). No statistically significant difference was found in the distribution of IL-10 promoter genotype in AIH and PBC patients compared with controls. CONCLUSION: The polymorphisms of IL-1RN and IL-6 -174G/C appear to be associated with PBC in Chinese patients.

关键词： Autoimmune hepatitis primary biliary cirrhosis Cytokine Polymorphisms Gene susceptibility

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A serum metabolomic analysis for diagnosis and biomarker discovery of primary biliary cirrhosis and autoimmune hepatitis

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Hepatobiliary & Pancreatic Diseases International 2015年第4期14卷 413-421页

作者： Jiang-Shan Lian Wei Liu Shao-Rui Hao De-Ying Chen Yin-Yin Wang Jian-Le Yang Hong-Yu Jia Jian-Rong Huang Collaborative Innovation Center for Diagnosis and Treatment of Infectious Diseases State Key Laboratory for Diagnosis and Treatment of Infectious Disease First Affiliated Hospital Zhejiang University School of Medicine Yantai City Hospital for Infectious Diseases

Because of the diversity of the dinical and laboratory manifestations, the diagnosis of autoimmune liver disease （AILD） remains a challenge in clinical practice. The value of metabolomics has been studied in the diagnosis of many diseases. The present study aimed to determine whether the metabolic profiles, based on ultraperformance liquid chromatography-mass spectrometry （UPLC-MS）, differed between autoimmune hepatitis （AIH） and primary biliary cir- rhosis （PBC）, to identify specific metabolomic markers, and to establish a model for the diagnosis of AIH and PBC. METHODS： Serum samples were collected from 20 patients with PBC, 19 patients with AIH, and 25 healthy individuals. UPLC-MS data of the samples were analyzed using principal component analysis, partial least squares discrimination analysis and or- thogonal partial least squares discrimination analysis. RESULTS： The partial least squares discrimination analysis model （R2y=0.991, Q2=0.943） was established between the AIH and PBC groups and exhibited both sensitivity and speci- ficity of 100%. Five groups of biomarkers were identified, in- eluding bile acids, free fatty acids, phosphatidylcholines, lyso- lecithins and sphingomyelin. Bile acids significantly increased in the AIH and PBC groups compared with the healthy con- trol group. The other biomarkers decreased in the AIH andPBC groups compared with those in the healthy control group. In addition, the biomarkers were downregulated in the AIH group compared with the PBC group. CONCLUSIONS： The biomarkers identified revealed the pathophysiological changes in AILD and helped to discrimi- nate between AIH and PBC. The predictability of this method suggests its potential application in the diagnosis of AILD.

关键词： autoimmune liver disease biomarkers metabolomics autoimmune hepatitis primary biliary cirrhosis overlap syndrome

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