scholarly journals Autoimmmune hepatitis

2021 ◽  
Author(s):  
Benedetta Terziroli Beretta-Piccoli ◽  
Giorgina Mieli-Vergani ◽  
Diego Vergani
Keyword(s):  
Mhc Class Ii ◽  
Late Onset ◽  
Immunosuppressive Treatment ◽  
Elevated Serum ◽  
Clinical Aspects ◽  
Comprehensive Overview ◽  
Insufficient Response ◽  
Circulating Autoantibodies

AbstractAutoimmune hepatitis (AIH) is a T-cell mediated, inflammatory liver disease affecting all ages and characterized by female preponderance, elevated serum transaminase and immunoglobulin G levels, positive circulating autoantibodies, and presence of interface hepatitis at liver histology. AIH type 1, affecting both adults and children, is defined by positive anti-nuclear and/or anti-smooth muscle antibodies, while type 2 AIH, affecting mostly children, is defined by positive anti-liver-kidney microsomal type 1 and/or anti-liver cytosol type 1 antibody. While the autoantigens of type 2 AIH are well defined, being the cytochrome P4502D6 (CYP2D6) and the formiminotransferase cyclodeaminase (FTCD), in type 1 AIH they remain to be identified. AIH-1 predisposition is conferred by possession of the MHC class II HLA DRB1*03 at all ages, while DRB1*04 predisposes to late onset disease; AIH-2 is associated with possession of DRB1*07 and DRB1*03. The majority of patients responds well to standard immunosuppressive treatment, based on steroid and azathioprine; second- and third-line drugs should be considered in case of intolerance or insufficient response. This review offers a comprehensive overview of pathophysiological and clinical aspects of AIH.

scholarly journals Autoimmune Hepatitis: Serum Autoantibodies in Clinical Practice

2021 ◽  
Author(s):  
Benedetta Terziroli Beretta-Piccoli ◽  
Giorgina Mieli-Vergani ◽  
Diego Vergani
Keyword(s):  
Autoimmune Hepatitis ◽  
Current Knowledge ◽  
Reference Method ◽  
Smooth Muscle Antibody ◽  
Autoimmune Sclerosing Cholangitis ◽  
Specific Autoantibody ◽  
Circulating Autoantibodies ◽  
International Autoimmune Hepatitis Group

AbstractCirculating autoantibodies are a key diagnostic tool in autoimmune hepatitis (AIH), being positive in 95% of the cases if tested according to dedicated guidelines issued by the International Autoimmune Hepatitis Group. They also allow the distinction between type 1 AIH, characterized by positive anti-nuclear and/or anti-smooth muscle antibody, and type 2 AIH, characterized by positive anti-liver kidney microsomal type 1 and/or anti-liver cytosol type 1 antibody. Anti-soluble liver antigen is the only AIH-specific autoantibody, and is found in 20–30% of both type 1 and type 2 AIH. Anti-neutrophil cytoplasmic antibody is frequently positive in type 1 AIH, being associated also with inflammatory bowel disease and with primary/autoimmune sclerosing cholangitis. The reference method for autoantibody testing remains indirect immunofluorescence on triple tissue (rodent liver, kidney and stomach), allowing both the detection of the majority of liver-relevant reactivities, including those autoantibodies whose molecular target antigens are unknown. Of note, the current knowledge of the clinical significance of autoantibodies relies on studies based on this technique. However, immunofluorescence requires trained laboratory personnel, is observer-dependent, and lacks standardization, leading to ongoing attempts at replacing this method with automated assays, the sensitivity, and specificity of which, however, require further studies before they can be used as a reliable alternative to immunofluorescence; currently, they may be used as complementary to immunofluorescence.

scholarly journals Elevated serum alanine transaminase in patients with type 1 or type 2 diabetes mellitus

QJM ◽  
2006 ◽  
Vol 99 (12) ◽  
pp. 871-876 ◽  
Author(s):  
J. West ◽  
J. Brousil ◽  
A. Gazis ◽  
L. Jackson ◽  
P. Mansell ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Type 2 Diabetes ◽  
Type 2 Diabetes Mellitus ◽  
Elevated Serum ◽  
Alanine Transaminase

Diabetes-induced Proteome Changes Throughout Development

2019 ◽  
Vol 19 (6) ◽  
pp. 732-743 ◽  
Author(s):  
Dina Johar ◽  
Sara M. Ahmed ◽  
Samer El. Hayek ◽  
Nader Al-Dewik ◽  
Eshak I. Bahbah ◽  
...  
Keyword(s):  
Insulin Resistance ◽  
Insulin Secretion ◽  
Late Onset ◽  
Hormonal Changes ◽  
Insulin Deficiency ◽  
Β Cell ◽  
Peripheral Tissues ◽  
Proteome Changes

Background: Diabetes Mellitus (DM) is a multisystemic disease involving the homeostasis of insulin secretion by the pancreatic islet beta cells (β-cells). It is associated with hypertension, renal disease, and arterial and arteriolar vascular diseases. Discussion: The classification of diabetes is identified as type 1 (gene linked β-cell destruction in childhood) and type 2 (late onset associated with β-cell overload and insulin resistance in peripheral tissues. Type 1 diabetes is characterized by insulin deficiency, type 2 diabetes by both insulin deficiency and insulin resistance. The former is a genetically programmed loss of insulin secretion whereas the latter constitutes a disruption of the homeostatic relationship between the opposing activity of β- cell insulin and alpha cell (α-cell) glucagon of the Islets of Langerhans. The condition could also occur in pregnancy, as a prenatal occurring event, possibly triggered by the hormonal changes of pregnancy combined with β-cell overload. This review discusses the molecular basis of the biomolecular changes that occur with respect to glucose homeostasis and related diseases in DM. The underlying link between pancreatic, renal, and microvascular diseases in DM is based on oxidative stress and the Unfolded Protein Response (UPR). Conclusion: Studying proteome changes in diabetes can deepen our understanding of the biomolecular basis of disease and help us acquire more efficient therapies.

Diabetes mellitus in patients with rheumatoid arthritis in the Republic of Karelia

2020 ◽  
Vol 14 (2) ◽  
pp. 57-61
Author(s):  
N. V. Koryakova ◽  
I. I. Polskaya ◽  
I. M. Marusenko ◽  
T. I. Kulagina
Keyword(s):  
Rheumatoid Arthritis ◽  
Diabetes Mellitus ◽  
General Population ◽  
Late Onset ◽  
Autoimmune Diabetes ◽  
Type 2 Dm ◽  
The Republic ◽  
Type 1 Dm

Objective: to estimate the incidence of diabetes mellitus (DM) in patients with rheumatoid arthritis (RA) in the Republic of Karelia and to analyze the types of carbohydrate metabolism disorders.Patients and methods. The investigation enrolled 889 patients aged 18 years and older with RA treated in the Rheumatology Department, V.A. Baranov Republican Hospital (Petrozavodsk), in 2000 to 2019. Among them, there were 709 (79.8%) women and 180 (20.2%) men; the median age was 59 [52; 66] years; the median duration of RA was 6.5 [1.75; 13] years. Most patients were found to have positive rheumatoid factor (68.3%), the second and more advanced radiological stages (90.6%), and moderate and high RA disease activity (84.8%). Methotrexate as a mainstay disease-modifying anti-rheumatic drug was taken by 68.2%; biological agents and glucocorticoids (GCs) were used in 4.8 and 3.3%, respectively. The incidence of DM and its types and risk factors (RFs) were analyzed.Results and discussion. DM was recorded in 67 (7.5%) RA patients (55 women and 12 men; the median age was 62 [56; 66] years). Among these patients, 9 (1.0%) patients with RA were observed to have type 1 DM, 50 (5.6%) and 8 (0.9%) patients had steroid-induced DM (SIDM), and type 2 DM, respectively. In 55.5% of cases, type 1 DM was represented by latent autoimmune diabetes in adults (LADA), which is of late onset. SIDM was detected in 75% of patients over 60 years of age who had RFs for type 2 DM. In this study, the incidence of type 2 DM in RA patients exceeded official figures in the general population of the Russian Federation, but was close to the predicted prevalence rates of type 2 DM. Patients with RA and type 2 DM had major RFs, such as age over 45 years, hypertension, overweight or obesity; moreover, 60% of patients were found to have a combination of these factors.Conclusion. RA patients showed a higher incidence rate of carbohydrate disorders of different types; the number of cases of type 1 DM and type 2 DM among the examined patients with RA exceeded that in the regional DM registries in the general population in the Republic of Karelia. It seems advisable to screen for carbohydrate disorders in patients with RA, especially in the presence of RFs for type 2 DM and during systemic therapy with GCs.

scholarly journals Association and biomarker potential of elevated serum adiponectin with nephropathy among type 1 and type 2 diabetics: A meta-analysis

PLoS ONE ◽  
2018 ◽  
Vol 13 (12) ◽  
pp. e0208905 ◽  
Author(s):  
Noel Pabalan ◽  
Raphael Enrique Tiongco ◽  
Jefferyl Kae Pandac ◽  
Noemi Anne Paragas ◽  
Shamar Lo Lasta ◽  
...  
Keyword(s):  
Meta Analysis ◽  
Elevated Serum ◽  
Serum Adiponectin ◽  
Type 2 Diabetics

scholarly journals Paragangliomas/Pheochromocytomas: Clinically Oriented Genetic Testing

2014 ◽  
Vol 2014 ◽  
pp. 1-14 ◽  
Author(s):  
Rute Martins ◽  
Maria João Bugalho
Keyword(s):  
Genetic Testing ◽  
Somatic Mutations ◽  
Parasympathetic Nervous System ◽  
Multiple Endocrine Neoplasia Type ◽  
Neurofibromatosis Type ◽  
Clinical Aspects ◽  
Von Hippel Lindau

Paragangliomas are rare neuroendocrine tumors that arise in the sympathetic or parasympathetic nervous system. Sympathetic paragangliomas are mainly found in the adrenal medulla (designated pheochromocytomas) but may also have a thoracic, abdominal, or pelvic localization. Parasympathetic paragangliomas are generally located at the head or neck. Knowledge concerning the familial forms of paragangliomas has greatly improved in recent years. Additionally to the genes involved in the classical syndromic forms:VHLgene (von Hippel-Lindau),RETgene (Multiple Endocrine Neoplasia type 2), andNF1gene (Neurofibromatosis type 1), 10 novel genes have so far been implicated in the occurrence of paragangliomas/pheochromocytomas:SDHA, SDHB, SDHC, SDHD, SDHAF2, TMEM127, MAX, EGLN1, HIF2A,andKIF1B. It is currently accepted that about 35% of the paragangliomas cases are due to germline mutations in one of these genes. Furthermore, somatic mutations ofRET, VHL, NF1, MAX, HIF2A,andH-RAScan also be detected. The identification of the mutation responsible for the paraganglioma/pheochromocytoma phenotype in a patient may be crucial in determining the treatment and allowing specific follow-up guidelines, ultimately leading to a better prognosis. Herein, we summarize the most relevant aspects regarding the genetics and clinical aspects of the syndromic and nonsyndromic forms of pheochromocytoma/paraganglioma aiming to provide an algorithm for genetic testing.

Postmenopausal Patients With Endometrial Cancer of Type 1 Have Elevated Serum Estradiol Levels in the Ovarian Vein

2014 ◽  
Vol 24 (8) ◽  
pp. 1455-1460 ◽  
Author(s):  
Keisuke Ashihara ◽  
Tomohito Tanaka ◽  
Risa Maruoka ◽  
Yoshihiro J. Ono ◽  
Akiko Tanabe ◽  
...  
Keyword(s):  
Endometrial Cancer ◽  
Elevated Serum ◽  
Sex Steroid ◽  
Ovarian Vein ◽  
Bilateral Oophorectomy ◽  
Sex Steroid Hormone ◽  
Ovarian Stromal Hyperplasia ◽  
A Prospective Study

ObjectiveType 1 endometrial cancer (EC) is typically sex hormone sensitive; however, most women diagnosed with EC have already gone through menopause. Several studies have reported that the postmenopausal ovary is hormonally active, and estradiol (E2) production from the ovaries persists for as much as 10 years beyond menopause. The aim of this study was to evaluate whether sex steroid production from the ovaries contributes to the pathogenesis of type 1 EC.Materials and MethodsThis was a prospective study of 53 women treated for EC (28 cases of type 1 disease and 25 cases of type 2 disease). Serum specimens were collected from the peripheral and ovarian veins of participants undergoing bilateral oophorectomy. The sex steroid hormone levels and hormonal milieu on cervical cytology were evaluated as maturation value (MV). In addition, the degree of stromal hyperplasia of the ovary was evaluated histologically.ResultsAlthough the E2 levels of the peripheral veins did not show any significant differences [8.2 (5.1–12.4) vs 7.4 (5.1–11.7) pg/mL, respectively; P < 0.05], the patients with type 1 EC had a higher E2 level in the ovarian vein than those with type 2 EC [25 (13.8–42.5) vs 15 (10.0–23.0) pg/mL, respectively; P < 0.05]. There were also no significant differences in the rate of moderate to marked hyperplasia of the ovarian stroma between the groups; however, the thickness of the ovarian cortex demonstrated a correlation with the ovarian E2 level. In addition, the MV displayed a strong correlation with the ovarian E2 level, but not the peripheral E2 level.ConclusionsThe postmenopausal ovary is hormonally active, especially in patients with type 1 EC. The degree of ovarian stromal hyperplasia may (at least in part) contribute to the progression of type 1 EC, and MV may predict the level of E2 production from the ovaries in postmenopausal women.

Autoimmune Hepatitis

2015 ◽  
Author(s):  
Jennifer Y Chen ◽  
Karin L. Andersson
Keyword(s):  
Autoimmune Hepatitis ◽  
Scoring System ◽  
Elevated Serum ◽  
Smooth Muscle Antibody ◽  
Treatment Regimens ◽  
Recommended Treatment ◽  
Serum Transaminases ◽  
Disease Subtypes

Autoimmune hepatitis (AIH) is defined by elevated serum transaminases along with the presence of one or more characteristic serum autoantibodies, including antinuclear antibody (ANA), anti–smooth muscle antibody (ASMA), and anti–liver-kidney microsomal type 1 antibody (anti-LKM-1); elevated levels of serum immunoglobulin G (IgG); interface hepatitis on histology; and responsiveness to immunosuppressive therapy. AIH has been classified into two disease subtypes based on serologic markers: type 1, which is characterized by the presence of either ANA or ASMA, and type 2, which is characterized by the presence of either anti-LKM-1 or anti–liver-cytosol antibody type 1 (anti-LC-1). This review addresses the epidemiology, natural history, pathogenesis, and management of AIH, as well as ongoing challenges. Several recent advances are highlighted, including the creation of a simplified diagnostic scoring system and the use of budesonide for AIH treatment. Figures show the pathology of AIH, treatment approach of moderate to severe AIH with combination therapy, azathioprine metabolism, and management of treatment outcomes. Tables list a comparison of type 1 and type 2 AIH, antibodies in AIH, the revised diagnostic scoring system (including a simplified version), indications for treatment in AIH, recommended treatment regimens for AIH by the American Association for the Study of Liver Diseases, and adverse effects associated with therapy for AIH. This review contains 4 highly rendered figures, 7 tables, and 146 references.

What Breathing Insulin Means for Diabetes Treatment

2006 ◽  
Vol 00 (02) ◽  
Author(s):  
Marc Rendell
Keyword(s):  
Glycosylated Hemoglobin ◽  
Glucose Levels ◽  
Blood Glucose Levels ◽  
Severe Deficiency ◽  
Patients With Diabetes ◽  
Insufficient Response ◽  
Relative Deficiency ◽  
Insulin Secretory Capacity

Type 1 diabetes is a disease of severe deficiency of endogenously secreted insulin. When introduced in the late 1920s, injected insulin treatment proved to be a lifesaving treatment for type 1 patients. The primary abnormality in type 2 diabetes is a relative deficiency of insulin secretory capacity resulting in insufficient response to tissue insulin resistance. Normalization of blood glucose levels is the goal of diabetes treatment.Yet, a large proportion of patients with diabetes fail to meet recommended glycemic goals. Two-thirds of patients (67%) in one survey conducted by the American College of Clinical Endocrinologists failed to meet the target goal of 6.5% glycosylated hemoglobin (HbA1c).1

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