Normalisation of High Ca 19-9 Values in Autoimmune Hepatitis after Steroidal Treatment

2005 ◽  
Vol 18 (3) ◽  
pp. 603-607 ◽  
Author(s):  
M. Montalto ◽  
L. Santoro ◽  
M. Vastola ◽  
V. Curigliano ◽  
R. Ricci ◽  
...  
Keyword(s):  
Liver Biopsy ◽  
Autoimmune Hepatitis ◽  
Clinical Picture ◽  
Immunohistochemical Analysis ◽  
Serum Levels ◽  
Specific Marker ◽  
Malignant Diseases ◽  
Laboratory Parameters ◽  
Bile Ductule ◽  
Mib 1

Carbohydrate 19-9 antigen (CA 19–9) is considered a specific marker of pancreatobiliary adenocarcinomas, but slight increase of its levels can be found in several non malignant diseases of the liver, such as autoimmune hepatitis. We describe a case of marked CA 19-9 elevation (up to 898.0 U/ml) in a patient with autoimmune hepatitis. Laboratory and instrumental examinations excluded malignant diseases. Immunohistochemical analysis for CA 19-9 and MIB-1, performed on liver biopsy, showed reactivity in inflammatory areas, in particular in bile ductule cells and hepatocytes in ductular metaplasia, suggesting that these cells could be involved in CA 19-9 serum levels increase. After steroids, the clinical picture improved and all the laboratory parameters normalised.

scholarly journals Leveraging Social Networking Sites for an Autoimmune Hepatitis Genetic Repository: Pilot Study to Evaluate Feasibility (Preprint)

2017 ◽  
Author(s):  
Megan Comerford ◽  
Rachel Fogel ◽  
James Robert Bailey ◽  
Prianka Chilukuri ◽  
Naga Chalasani ◽  
...  
Keyword(s):  
Pilot Study ◽  
Liver Biopsy ◽  
Social Networking ◽  
Autoimmune Hepatitis ◽  
Rare Disease ◽  
Social Networking Sites ◽  
Participant Recruitment ◽  
Study Materials ◽  
Immunosuppressant Medications ◽  
Oriented Research

BACKGROUND Conventional approaches to participant recruitment are often inadequate in rare disease investigation. Social networking sites such as Facebook may provide a vehicle to circumvent common research limitations and pitfalls. We report our preliminary experience with Facebook-based methodology for participant recruitment and participation into an ongoing study of autoimmune hepatitis (AIH). OBJECTIVE The goal of our research was to conduct a pilot study to assess whether a Facebook-based methodology is capable of recruiting geographically widespread participants into AIH patient-oriented research and obtaining quality phenotypic data. METHODS We established a Facebook community, the Autoimmune Hepatitis Research Network (AHRN), in 2014 to provide a secure and reputable distillation of current literature and AIH research opportunities. Quarterly advertisements for our ongoing observational AIH study were posted on the AHRN over 2 years. Interested and self-reported AIH participants were subsequently enrolled after review of study materials and completion of an informed consent by our study coordinator. Participants returned completed study materials, including epidemiologic questionnaires and genetic material, to our facility via mail. Outside medical records were obtained and reviewed by a study physician. RESULTS We successfully obtained all study materials from 29 participants with self-reported AIH within 2 years from 20 different states. Liver biopsy results were available for 90% (26/29) of participants, of which 81% (21/29) had findings consistent with AIH, 15% (4/29) were suggestive of AIH with features of primary biliary cholangitis (PBC), and 4% (1/29) had PBC alone. A total of 83% (24/29) had at least 2 of 3 proposed criteria: positive autoimmune markers, consistent histologic findings of AIH on liver biopsy, and reported treatment with immunosuppressant medications. Self-reported and physician records were discrepant for immunosuppressant medications or for AIH/PBC diagnoses in 4 patients. CONCLUSIONS Facebook can be an effective ancillary tool for facilitating patient-oriented research in rare diseases. A social media-based approach transcends established limitations in rare disease research and can further develop research communities.

scholarly journals Autoimmune Hepatitis with Concomitant Idiopathic Thrombocytopenic Purpura Diagnosed by Transjugular Liver Biopsy

2018 ◽  
Vol 2018 ◽  
pp. 1-6
Author(s):  
Hiromi Fukuda ◽  
Kazuhide Takata ◽  
Takanori Kitaguchi ◽  
Ryo Yamauchi ◽  
Hideo Kunimoto ◽  
...  
Keyword(s):  
Platelet Count ◽  
Liver Biopsy ◽  
Autoimmune Hepatitis ◽  
Histological Examination ◽  
Idiopathic Thrombocytopenic Purpura ◽  
Liver Dysfunction ◽  
Severe Thrombocytopenia ◽  
Percutaneous Liver Biopsy ◽  
Thrombocytopenic Purpura ◽  
Transjugular Liver Biopsy

Patients with autoimmune hepatitis (AIH) may sometimes have concomitant idiopathic thrombocytopenic purpura (ITP). Severe thrombocytopenia in ITP interferes with percutaneous liver biopsy for pathological diagnosis of AIH. Here, we report a case of AIH with ITP in a 63-year-old woman. The patient presented to our hospital with liver dysfunction and thrombocytopenia. For histological examination, transjugular liver biopsy (TJLB) was performed, leading to a diagnosis of AIH. Corticosteroids treatment led to an improvement in her liver enzyme levels and platelet count. In conclusion, patients with AIH may sometimes have concomitant ITP. TJLB was effective for making the diagnosis of AIH with severe thrombocytopenia due to ITP.

Neutrophil Extracellular Traps May Contribute to the Pathogenesis in Adult-onset Still Disease

2019 ◽  
Vol 46 (12) ◽  
pp. 1560-1569 ◽  
Author(s):  
Mi-Hyun Ahn ◽  
Jae Ho Han ◽  
Young-Jun Chwae ◽  
Ju-Yang Jung ◽  
Chang-Hee Suh ◽  
...  
Keyword(s):  
Immunohistochemical Analysis ◽  
Serum Levels ◽  
Neutrophil Extracellular Traps ◽  
Polymorphonuclear Neutrophils ◽  
Adult Onset ◽  
Cell Free Dna ◽  
Extracellular Traps ◽  
Free Dna ◽  
Still Disease

Objective.Release of neutrophil extracellular traps (NET) has been described as an effector mechanism of polymorphonuclear neutrophils in several inflammatory diseases. Thus, this study was performed to evaluate the role of NET in the pathogenesis of adult-onset Still disease (AOSD).Methods.We determined the serum levels of NET molecules and investigated their associations with clinical disease activities in patients with AOSD. Further, we analyzed the differences in the NETosis response in AOSD patients compared to healthy controls (HC). To explore the in vivo involvement of NET in AOSD, we performed immunohistochemical analysis of skin and lymph node (LN) biopsies for proteins related to NET in patients with active AOSD.Results.Serum levels of cell-free DNA, myeloperoxidase (MPO)-DNA complex, and α-defensin were significantly increased in patients with AOSD compared to HC. Serum levels of the NET molecules, cell-free DNA, MPO-DNA, and α-defensin were correlated with several disease activity markers for AOSD. In followup of patients with AOSD after treatment with corticosteroid, the levels of cell-free DNA and α-defensin decreased significantly. On immunohistochemistry, neutrophil elastase–positive and MPO-positive inflammatory cells were detected in skin and LN of patients with AOSD, and were expressed in fiber form in the lesions. The serum from patients with active AOSD induced NETosis in neutrophils from HC. NET molecules induced interleukin 1β production in monocytes, representing a novel mechanism in the pathogenesis of AOSD.Conclusion.The findings presented here suggest that NET may contribute to the inflammatory response and pathogenesis in AOSD.

Immunoglobulin G4 related systemic sclerosing disease involving the temporal bone

2010 ◽  
Vol 124 (10) ◽  
pp. 1106-1110 ◽  
Author(s):  
L Masterson ◽  
M Martinez Del Pero ◽  
N Donnelly ◽  
D A Moffat ◽  
E Rytina
Keyword(s):  
Temporal Bone ◽  
Plasma Cells ◽  
Rare Condition ◽  
Immunohistochemical Analysis ◽  
Surgical Excision ◽  
Serum Levels ◽  
Inflammatory Myofibroblastic Tumour ◽  
Inflammatory Pseudotumour ◽  
Immunoglobulin G4 ◽  
Anaplastic Lymphoma

AbstractObjective:To report a rare condition affecting the temporal bone. Immunoglobulin G4 related systemic sclerosing disease is a recently described autoimmune condition with manifestations typically involving the pancreas, biliary system, salivary glands, lungs, kidneys and prostate. Histologically, it is characterised by T-cell infiltration, fibrosis and numerous immunoglobulin G4-positive plasma cells. This condition previously fell under the umbrella diagnosis of inflammatory pseudotumour and inflammatory myofibroblastic tumour.Case report:We present the case of a 58-year-old woman with multiple inflammatory masses involving the pharynx, gall bladder, lungs, pelvis, omentum, eyes and left temporal bone, over a seven-year period. We describe this patient's unusual clinical course and pathological features, which resulted in a change of diagnosis from metastatic inflammatory myofibroblastic tumour to immunoglobulin G4 related systemic sclerosing disease. We also review the literature regarding the management of inflammatory pseudotumours of the temporal bone, and how this differs from the management of immunoglobulin G4 related systemic sclerosing disease.Conclusion:We would recommend a full review of all histological specimens in patients with a diagnosis of temporal bone inflammatory pseudotumour or inflammatory myofibroblastic tumour. Consideration should be given to immunohistochemical analysis for anaplastic lymphoma kinase and immunoglobulin G4, with measurement of serum levels of the latter. Management of the condition is medical, with corticosteroids and immunosuppression, rather than surgical excision.

scholarly journals The Imbalance between Foxp3+Tregs and Th1/Th17/Th22 Cells in Patients with Newly Diagnosed Autoimmune Hepatitis

2018 ◽  
Vol 2018 ◽  
pp. 1-12 ◽  
Author(s):  
Ma Liang ◽  
Zhang Liwen ◽  
Zhuang Yun ◽  
Ding Yanbo ◽  
Chen Jianping
Keyword(s):  
Autoimmune Hepatitis ◽  
Murine Model ◽  
Potential Role ◽  
Serum Levels ◽  
Newly Diagnosed ◽  
Foxp3 Mrna ◽  
Th22 Cells ◽  
Pathogenic Process ◽  
Experimental Autoimmune

This study is aimed at examining the potential role of regulatory T- (Treg-) Th1-Th17-Th22 cells in the pathogenic process of autoimmune hepatitis (AIH). The numbers of Foxp3+Tregs and Th1, Th17, and Th22 cells were measured in 32 AIH patients using flow cytometry. Moreover, a murine model of experimental autoimmune hepatitis (EAH) was also established and used to investigate the function of Treg-Th1-Th17-Th22 cells in disease progression. AIH patients undergoing an active state had significantly decreased numbers of CD3+CD4+CD25+Foxp3+Tregs and increased numbers of CD3+CD4+CD25−Foxp3+T, CD3+CD4+IFN-γ+Th1, CD3+CD4+IL-17+Th17, and CD3+CD4+IL-2+Th22 cells as well as higher levels of Th1/Th17/Th22-type cytokines compared to AIH patients in remission and HC. Additionally, the numbers of CD3+CD4+CD25+Foxp3+Tregs were negatively correlated with the numbers of Th1-Th17-Th22 cells. Also, the serum levels of IL-17A and IL-22 were correlated positively with liver injury (ALT/AST), whereas the serum levels of IL-10 were correlated negatively with hypergammaglobulinaemia (IgG, IgM) in AIH patients. Interestingly, the percentages of spleen Tregs, expression of Foxp3 mRNA, and liver IL-10 levels decreased, whereas the percentages of spleen Th1-Th17-Th22 cells, expression of T-bet/AHR/RORγt mRNA, and liver IFN-γ, IL-17, and IL-22 levels increased in the murine model of EAH. Our findings demonstrated that an imbalance between Tregs and Th1-Th17-Th22 cells might contribute to the pathogenic process of AIH.

Patients With Typical Laboratory Features of Autoimmune Hepatitis Rarely Need a Liver Biopsy for Diagnosis

2011 ◽  
Vol 9 (1) ◽  
pp. 57-63 ◽  
Author(s):  
Einar Björnsson ◽  
Jayant Talwalkar ◽  
Sombat Treeprasertsuk ◽  
Matthias Neuhauser ◽  
Keith Lindor
Keyword(s):  
Liver Biopsy ◽  
Autoimmune Hepatitis ◽  
Laboratory Features

Use of Erythropoietin (EPO) in Children with Malignancies.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 5533-5533
Author(s):  
Gabor T. Kovacs ◽  
Judit Muller ◽  
Monika Csoka ◽  
Eszter Vonnak ◽  
Hajna Erlaky ◽  
...  
Keyword(s):  
Pediatric Population ◽  
Lymphoblastic Leukemia ◽  
Elevated Serum ◽  
Serum Levels ◽  
Control Group ◽  
Recombinant Erythropoietin ◽  
Malignant Diseases ◽  
Human Erythropoietin ◽  
Unsuccessful Treatment ◽  
The Mean

Abstract Recombinant erythropoietin is widely used for the treatment of anemia in malignant diseases in adults. There are only limited data of its use in pediatric population. In this study we analysed the effectiveness and tolerability of recombinant human erythropoietin (NeoRecormon) in children with malignant diseases. 80 children with malignant diseases were analysed. 40 patients (15 girls, 25 boys) received EPO in a mean dosage of 144.5±14.1 IU/kg three times a week. The mean age of the EPO-treated patients was 8.8 (2.5–16) years. 26 children had acute lymphoblastic leukemia and 14 patients had solid tumor. Match-paired, retrospective control patients (n=40) with similar diagnosis were used for the data analysis as control group (C). The mean duration of EPO treatment was 5.8 months (3–8 mo). In 6 patients the therapy was ceased due to elevated serum hemoglobin (Hb) (>130 g/L), in 6 patients the dose was increased up to 200 IU/kg three times a week, and 5 patients discontinued the therapy (2 died, 3 unsuccessful treatment). The mean amount of erythrocyte transfusion in the first 3 months of chemotherapy (CT) was 4.1±3.1 U/patient in the EPO group, and 8.0±4.2 in C, and during 6 months of CT 4.5±3.4 with EPO, and 11.6±7.1 in C (p<0.05). Soluble transferrine receptor (STFR) levels in serum increased in the EPO group after 2 weeks of therapy from 3.2±2.0 up to 4.8±2.9 (p<0.05). In general in 26/40 patients a significant elevation of the Hb levels and decrease of the need of erythrocyte transfusions could be detected. In 22 patients the STFR levels increased more than 50 % after 2 weeks of therapy. In this subgroup 18/22 children responded to EPO therapy. All patients tolerated the therapy well, no severe side effects were detected. In summary, EPO treatment is effective in about 2/3 of pediatric oncology patients. The therapy is well-tolerated. Increase in the STFR serum levels might be a useful marker for the effectiveness of EPO in children.

scholarly journals Validation of Hepascore as a Predictor of Liver Fibrosis in Patients with Chronic Hepatitis C Infection

2011 ◽  
Vol 2011 ◽  
pp. 1-6 ◽  
Author(s):  
Hamid Kalantari ◽  
Hannan Hoseini ◽  
Anahita Babak ◽  
Majid Yaran
Keyword(s):  
Chronic Hepatitis ◽  
Hepatitis C ◽  
Liver Biopsy ◽  
Chronic Hepatitis C ◽  
Total Bilirubin ◽  
Serum Levels ◽  
Hepatitis C Infection ◽  
Glutamyl Transferase ◽  
Sensitivity Specificity ◽  
Severe Fibrosis

Introduction. Liver biopsy is an invasive determinator for hepatic fibrosis. Serum biomarkers can probably be used as an alternative to liver biopsy in assessment of the degree of fibrosis in patients with chronic Hepatitis C. Method. Eighty patients with chronic Hepatitis C were included in the study using simple nonrandom sampeling metod. After fulfillment of liver biopsy, the patients were categorized according to the METAVIR Scoring system. The Hepascore algorithm is computed based on age, sex, and the serum levels of total bilirubin, -glutamyl transferase, 2-Macroglobulin, and hyaluronic acid. The spearman and ROC tests were used. Results. According to the liver biopsy results, 12, 25, 20, 7 and 16 patients had F0, F1, F2, F3, and F4, respectively. With regard to the 0.34 cut-off point Hepascore had 67%, 56%, 64%, and 56% sensitivity, specificity, respectively, positive prediction value (PPV), and negative prediction value (NPV), respectively, for diagnosis of significant fibrosis. For a Hepascore cut-off point 0.61, sensitivity, specificity, respectively, PPV and NPB 82%, 86%, 70%, and 92% in diagnosis of severe fibrosis. For a Hepascore cut-off point 0.84, sensitivity, specificity, PPV and NPB were respectively 100%, 97%, 89%, and 100% for diagnosis of cirrhosis. Conclusion. Hepascore has a high value in diagnosis of the level of fibrosis, particularly cirrhosis. Therefore, it can be used for primary screening of patients to determine the need for liver biopsy.

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