Autoimmune hepatitis � primary biliary cirrhosis and overlap syndrome: A case report

Important immune-mediated liver illnesses include autoimmunity hepatitis (AIH) and primary biliary cirrhosis. Typically, they are distinguished based on histological, biochemical, serological, and clinical parameters. Diagnostic criteria for many conditions are typically met by patients with autoimmune liver disease. The diagnosis of AIH/primary sclerosing cholangitis overlap is based on a mix of biochemistry, autoantibody profile, cholangiogram, and liver histology; there are no universally accepted criteria for this. The patient can remain asymptomatic or present with pruritis and jaundice. Diagnosis is through liver biopsy showing bile duct destruction and proliferation. The treatment is by high-dose ursodeoxycholic acid.

Significance of serum FGF19, Klotho and FGFR4 protein expression in patients with primary biliary cirrhosis / 中国基层医药

Objective:To investigate the significance of serum fibroblast growth factor 19 (FGF19), Klotho and fibroblast growth factor receptor 4 (FGFR4) protein expression in patients with primary biliary cirrhosis (PBC).Methods:Sixty-three patients with PBC who received treatment in Ningbo Huamei Hospital, University of Chinese Academy of Sciences between August 2017 and July 2020 were included in the PBC group. An additional 51 healthy patients who concurrently received physical examination in the same hospital were included in the control group. Serum FGF19, Klotho and FGFR4 protein expression were determined by enzyme linked immunosorbent assay.Results:Serum FGF19 and FGFR4 protein in PBC group were (178.86 ± 21.28) ng/L and (2.96 ± 0.47) ng/L, respectively, which were significantly higher than those in the control group [(69.93 ± 12.12) ng/L, (1.21 ± 0.35) ng/L, t = 32.51, 27.98, both P < 0.05]. Klotho protein expression in the PBC group was significantly lower than that in the control group [(3.25 ± 0.89) μg/L vs. (9.67 ± 1.53) μg/L, t = 22.08, P < 0.05]. Serum levels of alanine aminotransferase, aspartate aminotransferase, and alkaline phosphatase in the PBC group were (84.25 ± 13.24) U/L, (71.82 ± 10.35) U/L, (278.93 ± 32.45) U/L, respectively, which were significantly higher than those in the control group [(23.76 ± 3.42) U/L, (23.10 ± 4.53) U/L, (76.81 ± 16.36) U/L, t = 31.75, 31.26, 40.48, all P < 0.05]. The receiver operating characteristic curve analysis showed that the sensitivity and specificity of FGF19 in the diagnosis of PBC were 76.67% and 61.90%, respectively, they were 58.82% and 66.67% for Klotho protein diagnosis, 54.55% and 76.67% for FGFR4 protein. Pearson analysis revealed that there was a positive linear relationship between FGF19 and FGFR4 protein ( r = 0.78, P < 0.05), while there was a negative linear relationship between Klotho protein and FGFR4 protein ( r = -0.72, P < 0.05). Conclusion:In patients with PBC, serum FGF19 and FGFR4 protein levels are increased, while Klotho protein level is decreased. There is a positive linear relationship between FGF19 and FGFR4 protein, and there is a negative linear relationship between Klotho protein and FGFR4 protein. This study is highly innovative and scientific.

Características epidemiológicas y clínicas de pacientes con cirrosis hepática

RESUMEN Introducción: La cirrosis hepática constituye la etapa final de muchas enfermedades del hígado. Objetivo: Determinar las características epidemiológicas y clínicas de pacientes con cirrosis hepática. Métodos: Se realizó una investigación descriptiva, retrospectiva, con datos de archivo, de 57 pacientes cirróticos. Se excluyeron aquellos con datos insuficientes en la historia clínica. Las variables analizadas fueron: edad, sexo, etiología, modo de diagnóstico, comorbilidades, manifestaciones endoscópicas de la hipertensión portal, complicaciones, estadios de la enfermedad según D'Amico y clasificación de Child-Pugh. Para el análisis de los resultados se emplearon técnicas de la estadística descriptiva. Resultados: Hubo 19 pacientes (33,3 %) en el grupo de edad entre 60 y 69 años; 31 (54,4 %) mujeres y 26 (45,6 %) hombres. El virus de la hepatitis C fue encontrado en 21 pacientes (36,8 %). El 96,4 % de los enfermos se diagnosticaron mediante ecografía abdominal; 12 (21,1 %) presentaron ascitis y 38 (66,6 %) manifestaciones endoscópicas de hipertensión portal. En estadio 4 de D'Amico estaban 20 (35 %) enfermos y 26 (45,6 %) en estadio de Child-Pugh A; 24 (42,1 %) en Child-Pugh B y 7 (12,3 %) en Child-Pugh C. Conclusiones: La cirrosis hepática predomina en la séptima década de la vida, en el sexo femenino. Prevalece el ultrasonido abdominal como modo de diagnóstico. Las causas más frecuentes son el virus de hepatitis C y el alcoholismo. La ascitis es la complicación que más se presenta. La mayoría de los pacientes muestran signos de hipertensión portal. Predominan las formas no compensadas de la enfermedad.

ABSTRACT Introduction: Liver cirrhosis is the end stage of many liver diseases. Objective: To determine the epidemiological and clinical characteristics of patients with liver cirrhosis. Results: There were 19 patients (33.3%) in the age group between 60 and 69 years; 31 (54,4 %) women and 26 (45,6 %) men. Hepatitis C virus was found in 21 patients (36,8 %). 96,4 % of the patients were diagnosed by abdominal ultrasound; 12 (21,1 %) presented ascites and 38 (66,6 %) endoscopic manifestations of portal hypertension. In D'Amico stage 4 there were 20 (35 %) patients and 26 (45,6 %) in Child-Pugh A stage; 24 (42,1 %) in Child-Pugh B and 7 (12,3 %) in Child-Pugh C. Conclusions: Liver cirrhosis predominates in the seventh decade of life, in females. Abdominal ultrasound prevails as a diagnostic mode. The most common causes are hepatitis C virus and alcoholism. Ascites is the most common complication. Most patients present with signs of portal hypertension. Uncompensated forms of the disease predominate.

Sterile, recurrent, and bilateral corneal perforation related to primary biliary cirrhosis complicated by secondary Sjögren syndrome and vitamin A deficiency / Perfuração estéril, recorrente e bilateral da córnea relacionada à cirrose biliar primária agravada com síndrome de Sjögren secundária e deficiência de vitamina A

ABSTRACT Primary biliary cirrhosis is a rare progressive autoimmune liver disease that causes chronic cholestasis. Of patients with primary biliary cirrhosis, 75% develop secondary Sjogren syndrome and could develop vitamin A deficiency. Here, we report the case of a patient with primary biliary cirrhosis who developed a secondary Sjogren syndrome and vitamin A deficiency, which led to severe and unusual eye involvement with multiple and recurrent spontaneous corneal perforations. Corneal perforations in patients with primary biliary cirrhosis and secondary Sjogren syndrome are rare but devastating complications, in contrast to other eye clinical manifestations of the disease.

RESUMO A cirrose biliar primária é uma doença hepática autoimune progressiva rara que causa colestase crônica. 75% dos pacientes com Cirrose Biliar Primária desenvolvem Síndrome de Sjögren Secundária, e podem também desenvolver deficiência de vitamina A. Aqui, relatamos um paciente com Cirrose Biliar Primária que desenvolveu Síndrome de Sjögren Secundária e deficiência de vitamina A, levando a envolvimento ocular grave e incomum com perfurações espontâneas múltiplas e recorrentes da córnea. Perfurações da córnea em pacientes com Cirrose Biliar Primária e Síndrome de Sjögren Secundária são complicações raras, mas devastadoras, em contraste com outras manifestações clínicas oculares da doença.

Esclerodermia y Cirrosis Biliar Primaria (Síndrome de Reynolds): Descripción de un Caso / Scleroderma and Primary Biliary Cirrhosis (Reynolds syndrome): Case Description

El síndrome de Reynolds, se define como cirrosis biliar primaria en pacientes con esclerodermia; este síndrome debe ser sospechado en aquellos pacientes que desarrollen un patrón colestásico. Se reporta una paciente con antecedente de esclerodermia que se presenta con ictericia, a quien se le confirma con estudios inmunológicos y biopsia hepática, el diagnóstico de cirrosis biliar prima­ ria (ahora se denomina colangitis biliar primaria). Se ordena ácido ursodesoxicólico 15mg/día.

Reynolds syndrome is defined as primary biliary cirrhosis in patients with scleroderma; this syndro­me should be suspected in those patients who develop a cholesteric pattern. We report a patient with scleroderma who presented with jaundice. After immunological and liver biopsy, a diagnosis of Primary Biliary Cholangiopathy (new name) was confirmed. Ursodeoxycholic acid 15mg / day was prescribed

Esclerosis sistémica (esclerodermia) asociada con cirrosis biliar primaria / Systemic sclerosis (scleroderma) associated with primary biliary cirrhosis

Resumen La esclerosis sistèmica (esclerodermia) es la enfermedad reumática autoinmunitaria asociada más comúnmente con cirrosis biliar primaria. La relación entre la forma cutánea limitada de esclerosis sistémica y cirrosis biliar primaria se describió en el decenio de 1970. Se comunica el caso de una paciente de 65 años de edad con esclerosis sistémica cutánea limitada de larga evolución, positiva a anticuerpos contra centròmero y mitocondria. La biopsia hepática confirmó cirrosis biliar primaria en fase portal (estadio 1).

Abstract Systemic sclerosis (scleroderma) is an autoimmune rheumatic disease most commonly associated with primary biliary cirrhosis. The relationship between limited cutaneous systemic sclerosis and primary biliary cirrhosis was first described in the early 1970's decade. The case of 65 year-old woman with limited cutaneous systemic sclerosis and primary biliary cirrhosis seropositive for anticentromere and anti-mitochondrial antibodies is reported. Liver biopsy confirmed primary biliary cirrhosis in stage 1.

Change of PD-L1 in Peripheral Blood Mononuclear Cells of Patients with Primary Biliary Cirrhosis and Its Significance / 现代检验医学杂志

Objective To explore the expression of PD-L1 in peripheral blood mononuclear cells (PBMC) from patients with primary biliary cirrhosis (PBC) and its significance.Methods Peripheral blood of 23 healthy individuals and 23 patients with PBC diagnosed in Changhai Hospital was collected during February 2013 to December 2015.PBMC was separated by density gradient centrifugation,then cultured.The mRNA expression of PD-L1 in PBMC was examined by real-time fluorescent quantitative PCR (qRT-PCR),the expression of cytokines such as TNF-α,TGF-β,IL-23,IL-17 in medium was examined by enzyme-linked immunosorbent assay (ELISA).Two independent samples' t test was used to compare variables in two groups.Pearson correlative coefficient was used to reveal the relationship between two variables.Results mRNA expression of PD-L1 in experiment and control groups was 0.23±0.08 vs 1.27±0.40 (t=12.23,P＜0.000 1) and there was statistical significance.Pearson correlation analysis revealed that PD-L1 was negatively correlated with IL-23(r=-0.531,P =0.009) with difference statistically.Conclusion PD-L1 might involve in the development of PBC,and could be the potential biomarker for predicting and treating this disease.

Study on immune function and liver function injury in patients with primary biliary cirrhosis / 国际检验医学杂志

Objective To study the changes of immune function and liver function in patients with primary biliary cirrhosis.Methods From March 2013 to March 2016 in our hospital patients with primary biliary cirrhosis 30 cases as the observation group.There were 17 patients with mild liver injury and 13 with severe hepatic dysfunction.And at the same time for the admission of 30 patients with other liver diseases in patients with liver disease as a group,30 cases underwent physical examination as healthy group were detected by flow cytometry in three groups of subjects of immunity comparison of three groups of cells,dendritic cell subsets in peripheral blood and liver function index.Results The CD4+ cells and CD8+ cells in the observation group were significantly higher than those in the healthy group(P＜0.05),while the CD11c+ cells,CD123+ cells and NK cells were significantly lower than those in the healthy group(P＜0.05).In the observation group,CD4+ cells,CD8+ cells,CD11c+ cells and CD123+ cells were significantly higher than those of other liver diseases but the NK cells were significantly lower than those of the other groups (P＜0.05).In healthy group,CD4+ cells,CD8+ cells,CD11c+ cells,CD123+ cells and NK cells were significantly higher than those of other liver disease group(P＜0.05) The CD4+,CD8+ and CD123+ cells in the liver injury group were significantly higher than those in the liver function damage group,while the CD11c+ and NK cells were significantly lower than those in the liver function group,and the difference was statistically significant(P＜0.05).The CD4+ and CD8+ cells in the AMA-M2 negative group were significantly lower than that in the AMA-M2 positive group,while those in CD123+,CD1 1c+ and NK cells were significantly higher than those in the AMA-M2 positive group,and the difference was statistically significant (P＜ 0.05).Conclusion The immune function of patients with primary biliary cirrhosis and liver function injury are associated with production of serum AMA-M2 antibodies,including CD11c+ and CD123+ or as the main factors influence the development of the disease and the AMA-M2 antibody.

Study on immune function and liver function injury in patients with primary biliary cirrhosis / 国际检验医学杂志

Objective To study the changes of immune function and liver function in patients with primary biliary cirrhosis.Methods From March 2013 to March 2016 in our hospital patients with primary biliary cirrhosis 30 cases as the observation group.There were 17 patients with mild liver injury and 13 with severe hepatic dysfunction.And at the same time for the admission of 30 patients with other liver diseases in patients with liver disease as a group,30 cases underwent physical examination as healthy group were detected by flow cytometry in three groups of subjects of immunity comparison of three groups of cells,dendritic cell subsets in peripheral blood and liver function index.Results The CD4+ cells and CD8+ cells in the observation group were significantly higher than those in the healthy group(P＜0.05),while the CD11c+ cells,CD123+ cells and NK cells were significantly lower than those in the healthy group(P＜0.05).In the observation group,CD4+ cells,CD8+ cells,CD11c+ cells and CD123+ cells were significantly higher than those of other liver diseases but the NK cells were significantly lower than those of the other groups (P＜0.05).In healthy group,CD4+ cells,CD8+ cells,CD11c+ cells,CD123+ cells and NK cells were significantly higher than those of other liver disease group(P＜0.05) The CD4+,CD8+ and CD123+ cells in the liver injury group were significantly higher than those in the liver function damage group,while the CD11c+ and NK cells were significantly lower than those in the liver function group,and the difference was statistically significant(P＜0.05).The CD4+ and CD8+ cells in the AMA-M2 negative group were significantly lower than that in the AMA-M2 positive group,while those in CD123+,CD1 1c+ and NK cells were significantly higher than those in the AMA-M2 positive group,and the difference was statistically significant (P＜ 0.05).Conclusion The immune function of patients with primary biliary cirrhosis and liver function injury are associated with production of serum AMA-M2 antibodies,including CD11c+ and CD123+ or as the main factors influence the development of the disease and the AMA-M2 antibody.

Correlation analysis of NF-κB signaling pathway activated by IL-18 in CD4+ T cells and the pathogenesis of PBC / 重庆医学

Objective To explore the correlation between NF-κB signaling pathways activated by IL-18 in CD4+ T cells and the pathogenesis of PBC.Methods We detected the expression of IL-18 mRNA in PBMCs,IL-18 level in plasma,receptor IL-18R on surface of CD4+ T cell,proliferation rate of CD4+T cell and its NF-κB signaling pathway protein IκBα and NF-κB p65 by qRT-PCR,ELISA,flow cytometry,MACS and Western blot on 32 cases of patients with PBC (PBC group) and 32 healthy people (control group) in Guizhou provincial people′s hospital.Results The level of IL-18 in PBC group was significantly higher than that in control group (P<0.05).The relative expression of IL-18 mRNA in PBC group was significantly higher than that in control group (P<0.05).The percentage of CD4+T cells expressing IL-18Rα in PBC group was higher than that in control group (P<0.05).The proliferation rate of CD4+T cells stimulated by IL-18 in PBC group was significantly higher than that in healthy control group (P<0.01).The relative expression levels of NF-κB p65 protein were up-regulated in IL-18,and the expression of IκBα protein in each group was significantly increased,especially in PBC group (P<0.01).Conclusion IL-18 can activate NF-κB signal pathway in CD4+ T cells and participate in the pathogenesis of primary biliary cirrhosis.

Mesenchymal stem cell alleviate primary biliary cirrhosis mice by down regulating autophagy of intrahepatic biliary epithelial cells via signal transducer and activator of transcription 3 / 中华风湿病学杂志

Objective To investigate the abnormality of intrahepatic biliary epithelial cells autophagy in primary biliary cirrhosis (PBC) mice,and explore the mechanism of UC-Mesenchymal stem cell (MSCs) in treating PBC.Methods After establishing the PBC model,we divided them into the PBC model group;the UC-MSCs treatment group and the Stattic group [Signal transducer and activator of transcription 3 (STAT3) inhibitor group].Six mice were used as the control group.Liver pathology and the serum pyruvate dehydrogenase complex E2 subunit (PDC-E2) antibody titers were detected.Autophagosome of the intrahepatic biliary epithelial cell was observed by electronic microscope.Protein levels of STAT3/pSTAT3,Beclin-1 were detected by western blot.We cultured human intrahepatic biliary epithelial cells in vitro,and down regulated STAT3.After stimulated by GCDC,we co-cultured them with UC-MSCs,and collected the cells in order to detect LC3 Ⅱ.The measurement data were compared with t test or single factor analysis of variance.Results Compared with the control group,periportal inflammatory cell infiltration and granuloma formation were observed in the PBC group.MSCs treatment decreased the infiltration of inflammatory cells.The level of antiPDC-E2 of the PBC group (107±18) ng/ml was higher than that of the control group (42±6) ng/ml (q=6.326,P＜0.01),MSCs treatment down regulated anti-PDC-E2 level (43±4) ng/ml (q=5.801,P＜0.01).More autophagosomes in the PBC group (5.00±1.29) than the control group (1.75±0.25) were observed (q=4.061,P＞0.05).Western blot showed that the level of Beclin-1 was higher in PBC group (1.80±0.36) than the control group (0.40±0.20) (q =6.757,P＜0.01),MSCs reduced the expression of Beclin-1 (0.86±0.06)(q=4.536,P＜0.05) as well as Stattic (0.72±0.03) (q=5.226,P＜0.05).PBC group had a higher expression level of STAT3 (1.80±0.42) (q=5.730,P＜0.05) and pSTAT3 (2.04±0.29)(q=6.492,P＜0.01) than the control group (0.50±0.05)(0.91±0.14).MSCs treatment decreased the expression of STAT3 (0.51±0.13)(q =5.703,P＜0.01) and pSTAT3 (0.76±0.07) (q =7.388,P＜0.01) in intrahepatic biliary epithelial cells.After down regulated STAT3 of HiBECs,MSCs reduced the expression of LC3 Ⅱ of HiBECs.Conclusion The intrahepatic biliary epithelial cells autophage of PBC mice is abnormal,MSCs can alleviate PBC by down regulating the autophage of intrahepatic biliary epithelial cells via STAT3.

Clinical value of related indexes in patients with primary biliary cirrhosis with esophageal varices / 中华传染病杂志

Objective To investigate the clinical indicators which can predict esophageal varices in patients with primary biliary cirrhosis (PBC).Methods A total of 351 patients with PBC from the First Affiliated Hospital of Xinjiang Medical University from January 2013 to December 2016 were retrospectively analyzed, including 173 patients with esophageal varices and 178 patients without varicose veins.The alanine aminotransferase (ALT), aspartate transaminase (AST), alkaline phosphatase (ALP), γ-glutamyl transpeptadase (γ-GT), total bilirubin (TBil), albumin (Alb), prothrombin time (PT), platelet (PLT), AST to ALT ratio (AAR), fibrosis index based on the 4 fator (FIB-4), AST to PLT ratio index (APRI) and Mayo scores were compared between two groups.Group t test or rank sum test was used to compare the two groups.Relation between the indicators mentioned above and esophageal varices were tested by univariate analysis.Multivariate unconditional Logistic regression was used to screen these indicators to independently predict esophageal varices in PBC patients.Results Age, PT, TBil, AAR, FIB-4, APRI and Mayo scores of PBC patients with esophageal varices were all higher than those of patients without esophageal varices ([60.3±10.6] years old vs [51.9±10.9] years old, [13.31±3.12] s vs [11.17±2.42] s, 28.06 [18.05, 60.06] mmol/L vs 15.39 [10.64, 33.63] μmol/L, 1.69±0.91 vs 1.23±0.95, 6.18 [4.05,9.16] vs 1.80 [1.10,2.74], 1.95 [1.12,3.08] vs 0.69 [0.38,1.57], 6.45±1.52 vs4.62±1.53, respectively).Whereas ALT, γ-GT, Alb and PLT levels were all lower than those without varicose veins (36.60 [19.88, 74.28] U/L vs 59.32 [23.58, 132.70] U/L, 71.00 [38.36, 165.38] U/L vs 125.00 [37.50, 336.21] U/L, [29.78±6.33] g/L vs [39.51±25.16] g/L, [103.43±52.84]×109/L vs [234.44±90.40]×109/L, respectively).The differences were all statistically significant (t=-7.25, t=-7.18, Z=-5.823, t=-4.60, Z=-8.427, Z=-12.661, t=-11.25, Z=-3.218, Z=2.987, t=4.94, t=16.63, respectively;all P11.95 s (OR=0.705, 95%CI: 0.569-0.874), TBil>17.19 μmol/L (OR=0.99, 95%CI: 0.982-0.999), FIB-4>3.02 (OR=0.868, 95% CI: 0.807-0.932) and Mayo score>4.88 (OR=6.053, 95%CI: 2.388-15.342) were independent clinical indicators for the prediction of esophageal varicose veins.Conclusions PLT, PT, TBil, FIB-4, and Mayo scores can be used as predictors of esophageal varices in patients with PBC.

Research advances of relationship between non-alcoholic fatty liver disease and biliary tract diseases / 中华肝脏病杂志

Recent studies have found that non-alcoholic fatty liver disease(NAFLD) has great impact on the development of biliary tract diseases. Here in this review, we summarized the relationship between NAFLD and the occurrence and development, risk factors and severity of cholestasis, gallstones, intrahepatic cholangiocarcinoma, primary biliary cirrhosis and bile microbiota, so as to further illuminate the pathogenesis of NAFLD and biliary tract diseases, obtain better diagnostic and therapeutic outcomes on NAFLD and biliary tract diseases.

Increasing Expression of IL-3 7 in Peripheral Blood Mononuclear Cell of Patients with Primary Biliary Cirrhosis and Its Clinical Significance / 现代检验医学杂志

Objective To test the expression level of IL-37 in peripheral blood mononuclear cells (PBMCs)of patients with primary biliary cirrhosis (PBC)and further explore its clinical significance in the pathological process of PBC.Methods Pe-ripheral blood samples were collected from 42 patients diagnosed as PBC and 38 health individuals examined at the same time during June 2013 to August 2015 in Changhai Hospital.PBMCs were separated by sucrose density gradient centrifugation, qualified Real Time-Polymerase Chain Reaction (qRT-PCR)was used to measure IL-37 mRNA expression level in PBMCs. Enzyme-Linked Immuno Sorbent Assay (ELISA)was to measure the protein level of IL-37,IL-6,IL-17,TNF-α,TGF-β,IL-18 and IL-23 in plasma.Meanwhile,the pathological stages of PBC cases were recorded.Pearson correlation analysis was performed on IL-37 and IL-6,TNF-α,IL-17,TGF-β,IL-18 and IL-23.Spearman rank correlation analysis was on IL-37 and pathological stages of PBC.Results The mRNA and protein level of IL-37 in experimental and controlled group were 2.81 ±0.94 vs 1.09±0.56,356.14±169.36 pg/ml vs 86.68±48.23 pg/ml separately(t=9.811,9.462,P<0.000 1),with sta-tistical differences.The correlation analysis showed that IL-37 was positively related with IL-17,TNF-α,IL-6 and TGF-β(r=0.561 2,0.661 9,0.672 1,0.765 3,P<0.001),and disease stages (Ⅰ~Ⅳ)(rs=0.348 9,P<0.05).Conclusion IL-37 might involve in the pathological process of PBC,and it is significant for disease prediction and diagnosis.

Patología del trasplante hepático, aspectos más relevantes del período postrasplante tardío / Most Relevant Pathology Issues in the Late Post Liver Transplant Period

La sobrevida de los pacientes postrasplante hepático supera el 90% al año y el 75% a los 5 años. Entender las causas de pérdida del injerto, o inclusive la muerte del paciente, es esencial para mejorar aún más los resultados a largo plazo. La evaluación de las biopsias hepáticas tiene un papel importante en la explicación y manejo de la disfunción del injerto de hígado, que ocurre después del primer año del trasplante. La interpretación de estas biopsias puede ser muy difícil, en especial por la alta incidencia de enfermedades recurrentes que pueden mostrar un cuadro clínico y unas características histopatológicas que semejan varias condiciones, especialmente cuando el rechazo agudo o crónico pueden sobreponerse a una patología ya existente o presentarse de manera simultánea y contribuir a la disfunción tardía del injerto, por lo que el análisis de la biopsia puede ayudar a determinar el componente principal de la lesión. Es indispensable la correlación clínico patológica, teniendo en cuenta la enfermedad original, el tipo de inmunosupresión, las pruebas de función hepática, las serologías virales, los autoanticuerpos y los hallazgos radiológicos. En este artículo comentaré las patologías más frecuentes y las que causan más problemas en su diagnóstico durante el período postrasplante tardío

One year survival rates of liver transplant patients exceed 90% while five year survival rates exceed 75%. Understanding the causes of graft losses and patient deaths is essential for further improvement of long-term results. Evaluation of liver biopsies has an important role in explaining liver graft dysfunction that occurs more than one year after transplantation, and thus is key for post-transplant patient management. The interpretation of these biopsies can be very difficult especially because of the high incidence of recurrent diseases that sometimes have clinical and histopathological features that resemble various other conditions. This is especially true for acute and chronic rejection which can overwhelm an existing condition and which can develop simultaneously with other conditions that contribute to late graft dysfunction. Analysis of the biopsy can help determine the main component of a lesion. Clinical findings must be correlated to pathological findings, and the correlation must take into account the original disease, the type of immunosuppression, liver function tests, viral serology, autoantibodies and radiological findings. In this article I will discuss the most common diseases and those that cause the most problems for diagnosis during the late post-transplant period

Síndrome de Reynolds en Hospital Carlos Andrade Marín, serie de casos y revisión / Reynolds Syndrome in Carlos Andrade Marin Hospital: a series of cases and review

Introducción: El Síndrome de Reynolds es la asociación de esclerosis sistémica (SSC) con cirrosis biliar primaria (CBP). Descrito en 1934 por Milbradt y Reynolds en 1976 describió seis casos. Caso: Presentamos tres casos de mujeres atendidas en el Servicio de Reumatologìa del Hospital Carlos Andrade Marín, con patología hepática autoinmune y signos de esclerosis sistémica. El diagnóstico fue confirmado mediante biopsia hepática en dos de ellas y la prueba de fibromax en la restante. Discusión: Reconocer el Síndrome de Reynolds permite el diagnóstico temprano de cirrosis biliar primaria en pacientes con esclerosis sistémica y sospechar esta entidad en quienes padecen enfermedad hepática colestásica autoinmune que, muchas veces, coexisten en forma silenciosa. El diagnóstico oportuno permite intervenciones terapéuticas precoces que podrían mejorar el pronóstico de esta asociación.

Introduction: The Reynolds' syndrome is the combination of systemic sclerosis (SSC) and primary biliary cirrhosis (PBC). It was first described in 1934 by Milbradt. Reynolds in 1976 presented six cases in two of them and fibromax-test in another. Case report: We present three cases of three women treated in the Rheumatology department at Carlos Andrade Marin hosppital who had autoinmune liver disease confirmed by biopsy. Discusion: Recognizing Reynolds' Syndrome allow us to make earlier diagnosis. This autoimmune cholestatic liver disease often remains silent, so that their identification is a valuable diagnostic tool leading to therapeutic interventions.

Significance of serum 25 (OH)-vitamin D in its diagnostic value of primary biliary cirrhosis / 中国免疫学杂志

Objective:To investigate the significance of 25 ( OH )-vitamin D in its diagnostic value of primary biliary cirrhosis. Methods:44 cases with PBC,41 cases with Hepatitis B cirrhosis and 110 normal people as control group were all analyzed bi-ochemical results and significance of serum 25 ( OH )-vitamin D. We Used receiver operator characteristic curve ( ROC ) to analyze serum 25(OH)-vitamin D in its diagnostic value of primary biliary cirrhosis. Results:Biochemical results of TBIL,DBIL,IBIL,GLB, AST,ALT,ALP,γ-GT were higher in PBC than that in normal people and Hepatitis B cirrhosis. While the serum 25(OH)-vitamin D of PBC was much more lower than that in normal and people and Hepatitis B cirrhosis. The difference between them were statistical difference(P<0. 05). The area under the ROC curve of PBC was 0. 926,it showed serum 25(OH)-vitamin D diagnosis for PBC had quite value. When the cut-off level of serum 25(OH)-vitamin D was 17. 275 ng/ml,the sensitivity for the diagnosis was 92. 8%,the re-spectively was 84. 1%. Conclusion:Serum 25(OH)-vitamin D diagnosis for PBC has quite value,the cut-off level of serum 25(OH)-vitamin D is 17. 275 ng/ml.

Expression of Th9 Cells from Peripheral Blood of Primary Biliary Cirrhosis Patients and Its Clinical Significance / 现代检验医学杂志

Objective To investigate the role of Th9 cells in primary biliary cirrhosis and its clinical significance.Methods The percentages of Th9 cells in patients with PBC (n=38)and healthy controls (n=38)were measured by flow cytometry. Patients clinical data were collected and Mayo risk scores were calculated.Determined the levels of IL-9,PU-1 and TGF-βmRNA expressions and the protein levels of IL-9 in plasma.Further analyzed the correlation between Th9 cells and clinical parameters.Results Compared to healthy controls,Th9 cells percentage was higher in PBC patients and increased with dis-ease stages (t=27.29;P<0.01).IL-9,PU-1 and TGF-βmRNA expressions were increased compared with healthy controls (t=14.69,23.92,10.48;all P<0.01)and the protein level of IL-9 was also up-regulated (t=11.66;P<0.01).The clinical parameters (ALP,AST,ALT,GGT and TBIL)were higher than healthy controls (t=10.94,12.95,10.56,14.92,27.70;all P<0.01).Moreover,the percentage of Th9 cells were positively correlated to Mayo risk,ALT,AST,GGT and TBIL (P<0.05).Conclusion Th9 cells may be involved in the pathogenesis of PBC and related to the disease stage,which provides new clues for future immunotherapy.

Expression of STING in Peripheral Blood in Patients with Primary Biliary Cirrhosis and Its Clinical Significance / 现代检验医学杂志

Objective To explore the clinical significance of STING in peripheral blood in patients with primary biliary cirrho-sis.Methods Admitted 28 PBC patients hospitalized in Shanghai Changhai Hospital and Shanghai Changzheng Hospital from January 2014 to October 2015.Also enrolled 28 healthy controls.Baseline data and laboratory indicators were extrac-ted,and STING mRNA expression was determined using q-PCR.The correlation between STING and clinical parameters were analyzed.The changes of STING mRNA in 5 followed-up patients with PBC were analyzed.Results Compared with healthy controls,STING mRNA was significantly increased in PBC patients and was increased in patients with severer dis-ease stages (U=0.00,P0.05).After therapy,STING mRNA were significantly re-duced in 5 PBC patients (U=0.00,P<0.05).Conclusion STING may be involved in PBC pathogenesis.

Analysis on autoantibodies spectrum of Uygur patients with autoimmune liver disease in Xinjiang / 重庆医学

Objective To analyze the positive rate of autoantibodies in Xinjiang Uygur patients with different types of auto‐immune liver disease and its diagnostic value .Methods Fifty‐one Uygur outpatients and inpatients with of autoimmune liver disease in our hospital from October 2012 to October 2014 were selected ,including 20 cases of autoimmune hepatitis (AIH ,AIH group) and 31 cases of primary biliary cirrhosis (PBC ,PBC group) .The indirect immunofluorescence and Western blotting were used to detect the serum biochemical indexes and autoantobodies ,and the comparative analysis was performed .Results The levels of various bio‐chemical indexes in the two groups were increased ,in which ALT ,γ‐GT ,ALP and IgM levels had statistical difference between the two groups(P<0 .05) .The positive rates of ANA ,SMA ,LKM‐1 antibody ,LC‐1 antibody ,SDLA/LP antibody and AMA in the AIH group were 65 .0% ,40 .0% ,10 .0% ,5 .0% ,10 .0% and 5 .0% respectively ;the positive rates of ANA ,AMA and M2 subtype (AMA‐M2) in the PBC group were 61 .3% ,100 .0% and 96 .8% respectively .Conclusion The autoantibodies spectrum in Xinjiang Uygur patients with autoimmune liver diseases has certain characteristics .The autoantibodies spectrum detection has an important significance for the diagnosis ,classification and differential diagnosis of autoimmune liver diseases .