جستجوی مقالات مرتبط با کلیدواژه "autoimmune hepatitis" در نشریات گروه "پزشکی"

 Colon inflammation may occur after solid and liquid organ transplantations at a rate higher than in the general population.

 This study aimed to investigate the incidence of de novo inflammatory bowel disease (IBD) and assess some risk factors after Orthotopic Liver Transplantation (OLT) in liver transplant patients in Shiraz, Iran.

 The sample study comprised patients (n = 1702) who had received liver transplants between 2001 and 2012 at the Shiraz liver transplant center. The data were obtained from patient records, which included information on their medical history, colonoscopy reports, family history of IBD, and other risk factors. The study evaluated the status of inflammatory bowel disease (IBD) among patients who had undergone liver transplantations due to Autoimmune Hepatitis (AIH) group, Primary Sclerosing Cholangitis (PSC), and other liver transplant patients. Additionally, case groups were formed, comprising AIH patients (n1 = 10) and other patients with IBD (n2 = 10), and were compared with transplant patients without IBD as the control group (n1 = 21, n2 = 21).

 Out of the total 1702 patients, 254 (14.92%) had AIH, 211 (12.40%) had PSC, and 1237 (72.68%) had other outcomes. The mean of leukocyte counts (P-value = 0.73), types of immunosuppressant medications, and serum levels of cyclosporine (P-value = 0.77) and tacrolimus (P-value = 0.27) did not show any significant differences between the case and control groups. However, it was observed that IBD developed earlier in patients with AIH compared to other patients (20.1 ± 2.23 vs 53.9 ± 4.42 months).

 The incidence of IBD after liver transplantation was higher compared to the general population, and it occurred earlier in patients who underwent transplantation for AIH and other groups.

The aim of the current research was to assess the clinical manifestations and diagnostic methods used in juvenile cases of Autoimmune Hepatitis (AIH).

This study employed a retrospective cross-sectional design to investigate pediatric patients diagnosed with AIH at Children’s Medical Center Hospital, which is affiliated to Tehran University of Medical Sciences (TUMS), Tehran, Iran. The study included patients who received routine examinations, treatments, and follow-ups during the period from 2018 to 2021. 

The present investigation encompassed the evaluation of 52 pediatric patients, mostly female, with a mean age of 7.76 years. The vast majority of patients have encountered the occurrence of acute AIH. Positive findings for the Anti-Smooth Muscle Antibodies (ASMA) test were seen in 50% of the patients. The mean score for fibrosis in the observed individuals was 2.56, whereas the mean value for the Hepatitis Activity Index (HAI) in a subset of 29 patients was found to be 7.34. One patient succumbed to the condition, one case underwent transplantation, and another individual was identified as a candidate for liver transplantation.

Patients with AIH saw a decrease in long-term survival. There was no observed disparity in prognosis based on gender; nevertheless, it was noted that males had a shorter lifespan, perhaps attributable to an earlier beginning of the illness. The presence of cirrhosis at the time of diagnosis constituted a significant risk factor for unfavorable prognosis, as it was associated with an elevated overall risk of mortality owing to liver dysfunction.

This is not surprising to detect iron overload in chronic liver diseases and end-stage liver diseases since Kupffer cells scavenge necrotic hepatocytes during the course of liver damage, leading to an increased serum iron level and transferrin saturation compatible with iron overload even in the absence of a genetic mutation suggestive of hereditary hemochromatosis. Therewith, a relative association has been found between some sorts of chronic liver diseases like non-alcoholic steatohepatitis and hepatitis C with human homeostatic iron regulator protein (HFE: High Fe2 + ) gene mutations. Moreover, impairment of ceruloplasmin ferroxidase activity in the course of Wilson’s disease (WD), leading to the accumulation of ferrous ions just like what is expected in aceruloplasminemia, is another known reason for iron overload accompanied by chronic liver disease. Of chronic liver diseases, autoimmune hepatitis (AIH), and cholestatic liver diseases are less related to iron overload. Accordingly, the coexistence of WD, AIH, and hereditary hemochromatosis when there exist clinical features, laboratory tests, genetic confirmation, and histological evaluations indicative of the three mentioned diseases is exceedingly rare. Here, we present a 55-year-old man referred with progressive generalized icterus accompanied by loss of appetite and significant weight loss. The presented case was not an appropriate candidate for liver biopsy due to recent coronary angioplasty and the urgent need for dual antiplatelet therapy. However, medical follow-ups were highly suggestive of concomitant WD, hereditary hemochromatosis, and AIH. The attempts failed for the treatment of hereditary hemochromatosis and WD with chelating agents until the completion of the course of treatment with immunosuppressants targeting components of the AIH-related immune system.

The presence of autoantibodies is a prerequisite for the diagnosis of autoimmune hepatitis (AIH). However, most autoantibodies are not disease-specific, and serological overlap between AIH and other chronic liver diseases is common. Since the prognostic parameters of AIH are limited, this study aimed to investigate the relationship between histopathological findings on liver biopsy with different types of autoantibodies associated with AIH and how autoantibodies can predict the severity and extent of disease. The present study was performed on 30 patients with a definite diagnosis of AIH according to the International Autoimmune Hepatitis Group (IAIHG) criteria. Pediatric AIH patients underwent liver tissue examinations at the time of diagnosis at accession, which confirmed characteristic histological changes. AIH-related serologic major and minor autoantibodies were measured using indirect immunofluorescence assays and ELISA kit (EUROIMMUN, Germany), respectively, and were compared within all patients, and the results were recorded. Finally, the obtained data were analyzed using SPSS V25 software. Out of 30 patients, 17 (56.66%) were female, and the age range of patients was 17-11 years (8.46 ± 6.95). Anti-nuclear antibody (ANA) (73.3%), smooth muscle antibody (SMA)-anti-smooth muscle actin antibody (ASMA) (70%), perinuclear anti- neutrophilic cytoplasmic antibodies (p-ANCA) (63%), and liver kidney microsomal (LKM) (43.3%) were the most common autoantibodies found in children with AIH. There was a significant relation between the severity of histological findings and the presence of LKM antibodies (P < 0.05). The highest sensitivity for predicting severe AIH based on histopathological findings was ANA autoantibody positivity and the presence of at least two primary autoantibodies (LKM and SMA-ASMA). On the other hand, positive LKM antibodies had the highest specificity and positive predictive value (PPV) in AIH severity prediction. The results of the present study suggested that there might be a significant correlation between the presence of primary LKM autoantibodies and biopsy results, so it can possibly act as an accurate autoantibody for predicting the severity of AIH, while other AIH-related autoantibodies did not seem to have a significant correlation with biochemical and histological findings.

 It is uncommon for autoimmune hepatitis (AIH) to occur in combination with hereditary spherocytosis (HS). The present study examined the genetic and clinical features of a seven-year-old girl with yellow sclerae and abnormalities in liver function test results.

 Blood samples were taken from this girl, her parents, and a parental grandmother to be analyzed using laboratory tests and Sanger and next-generation sequencing (NGS).

 Spectrin alpha, erythrocytic 1 (SPTA1) gene compound heterozygous mutations, were detected from this proband. Moreover, the proband inherited mutations c.6544G>C (p.D2182H) and Thec.134G>A (p.R45K) from her father and mother respectively. Moreover, both her father and grandmother shared an identical mutation. The mutations were not depicted in the Human Gene Mutation Database.

 HS shares some clinical features close to AIH hence, in the co-existence of AIH, its diagnosis can be challenging. The concurrent disorder may exist if a single autoimmune hepatopathy cannot explain laboratory findings. Pedigree investigations and genetic analyses might be required for the final diagnosis.

Simultaneous occurrence of immune-based gastrointestinal diseases and autoimmune hepatitis although is not common but is of clinical importance. Some clinical and laboratory findings such as severe pruritus and an elevation in alkaline phosphatase raise suspicion of a biliary disease which overlaps autoimmune hepatitis. A strong clinical suspicion of overlap syndrome in a patient with autoimmune hepatitis prompts more diagnostic evaluations like MRCP, liver biopsy and secondary laboratory tests. Patients who fall into the category of overlap syndrome would be proceeded with timely monitoring of known complications including colorectal carcinomas, cholangiocarcinomas and gallbladder cancers. So, it is highly recommended to search for all simultaneous immune-based involvements prior to labelling a patient as having pure autoimmune hepatitis. In this study, attempts have been made to express all challenges about a case with overlap syndrome referred to gastroenterology ward of Taleghani hospital and review the latest articles and related guidelines about the diagnosis, treatment, complications, and surveillance of the mentioned patient with autoimmune hepatitis (AIH), primary sclerosing cholangitis (PSC), and inflammatory bowel disease (IBD).

Abnormal growth in children with autoimmune hepatitis (AIH) is anticipated, either due to hepatic affection or the growth inhibitory effects of corticosteroids. We aimed to describe children's anthropometry with AIH, and study the factors affecting height.

The present observational study investigates the anthropometric measures of 28 children with AIH followed at a university hospital for 9.5±3 years. We calculated the initial AIH score, the Child-Pugh score, and the pediatric end-stage liver disease score (PELD), follow-up anthropometry, and corticosteroid history. We defined abnormal growth as under nutrition (underweight, wasting, stunting), short stature, overweight, and obesity.

At AIH diagnosis, children had a mean age of 7.4±3.1 years, ranging from 2 to 13.8; among whom ~20% had ascites, ~79% had jaundice, and ~82% had type 1 AIH, ~70% had a definite diagnosis of AIH, ~64% were Child-Pugh Score B, ~64% showed severe fibrosis/cirrhosis, and the median PELD score was 8.1 (0.1-12.1). At follow-up, their mean age was 15.9±1.6 years, with mean corticosteroid duration of 7.1±3.1 years, and remission occurred in 50%. We observed a significant improvement in the initial rates of underweight (46.4% vs. 17.8%), mainly stunted, and increased rates of overweight/obesity (14.3% vs. 32.2%). The final rates of height affection without weight affection were comparable to the initials (28.6% vs. 32.1%). Cases with abnormally low final height had significantly more frequent Child-Pugh Score B, higher PELD score, and severe hepatic fibrosis at presentation, with no difference regarding the continuation/ total duration of steroids.

the final height in children with AIH is significantly affected by the disease severity at presentation and not the continuation or the duration of corticosteroids use.

The specialists should identify the features of Wilson disease and autoimmune hepatitis when both affect a patient to adopt appropriate treatment. 

This study was conducted to determine features of the patient, disease, diagnostic studies, and therapeutic measures in cases of simultaneity of Wilson disease and autoimmune hepatitis.

To find evidence related to the study objectives, we searched databases such as Barakat knowledge network system, SID, Magiran, Google Scholar, Web of Science, ProQuest, Springer, ScienceDirect, Medline via PubMed, and Scopus with specified Persian and English keywords, including “Wilson’s Disease”, “Autoimmune”, and “Hepatitis”. The inclusion criteria for the studies were 1) the study was observational and 2) the study was published in Persian or English. The exclusion criteria included low-quality studies based on the score obtained from the checklist. The obtained studies were screened in terms of titles, abstracts, and full text, and finally, the qualified studies entered the review process. The relevant data were extracted according to a designed checklist.

Finally, 10 studies were included in the review process. Information about 14 patients was reported. The Mean±SD age of the participants in the studies was 19±11 years. The direction of diagnosis was from autoimmune hepatitis to Wilson disease in 8 cases and from Wilson disease to autoimmune hepatitis in 3 cases. The simultaneity of autoimmune hepatitis and Wilson disease was considered in 3 patients with no primary and secondary diagnosis.

The comorbidity of Wilson disease and autoimmune hepatitis is uncommon but is important. In the presence of relevant symptoms in these patients, the comorbidity of these two diseases should be considered. Accordingly, additional assessments such as serum ceruloplasmin, urinary 24-h copper, molecular genetic testing, MRI, serological tests, anti-nuclear antibody, anti-mitochondrial antibody, anti-smooth muscle antibody, complement level, gamma globulin, IgG, albumin, Kayser-Fleischer ring eye examination, and liver biopsy should be considered for correct diagnosis. If appropriate treatment was started for the disease with a diagnosis of Wilson disease or autoimmune hepatitis, but the response to treatment was insufficient, it is better to consider the simultaneous occurrence of two diseases or the initial misdiagnosis.

Atypical presentations in hepatitis A virus (HAV) infection are uncommonly encountered; nevertheless, they may lead to serious clinical complications. The present study reported the frequency of atypical presentations among children with acute HAV infection in south-east of Iran.

This prospective (cohort) study was conducted in the gastroenterology clinic of Amir-Al-Momenin Hospital (Zabol, Sistan and Baluchestan province, Iran). A total of 294 children with positive anti-HAV IgM test were enrolled during 2015-2018. They were prospectively monitored for the incidence of any atypical presentation.

Out of 294 children, 152 (51.7%) were males, and the mean age was 7.3±3.5. Nausea and vomiting (41.8%) constituted the most frequent clinical presentations. Overall, atypical presentations were observed in 38 (12.8%). The atypical presentations included autoimmune hepatitis (AIH) (15, 5.1%), pancytopenia (11, 3.7%), non-immune hemolytic anemia (5, 1.7%), Wilson disease (3, 1%), prolonged cholestasis (3, 1%), and gallbladder hydrops (1, 0.3%). The mean level of alanine aminotransferase at diagnosis was significantly lower in patients with atypical presentations compared with those without such complications (801.28±986.61 vs. 1119.09±1109.98 IU/l, P=0.01). Patients with atypical manifestations also had significantly lower levels of total bilirubin (3.77±2.88 vs. 5.57±5.28 mg/dl, P=0.03) and direct bilirubin (2.03±2.06 vs. 2.91±3.20 mg/dl, P=0.04).

Atypical manifestations are relatively common among children with acute HAV infection and should be routinely screened. With timely and appropriate interventions, clinical outcomes may not be significantly different from patients with typical presentation.

Pyodermatitis-pyostomatitis vegetans (PD-PSV) is a very rare inflammatory disease characterized by exudative pustular lesions of the skin and mucous membranes. The pathogenesis is unknown, but it may be related to immune or infectious processes. It is usually associated with inflammatory bowel disease (IBD). We describe a 32-year-old male with PD-PSV associated with manifestations suggestive of autoimmune hepatitis (AIH). To the best of our knowledge, this association has not been reported previously.

T cells are major players in chronic inflammatory diseases such as autoimmune hepatitis (AIH). However, it is not clear which subset of T cells participates in the pathophysiology of the disease. The aim of this study was to assess the expression profile of signature transcription factor and cytokines of T helper 17 (Th17) cells in patients with AIH. A total of 24 patients with AIH and 24 normal subjects were recruited in the study. Comparison of gene expression patterns between the patients and normal subjects was done by quantitative real-time reverse transcriptase polymerase chain reaction (qRT-PCR). The results showed that retinoic acid receptor-related orphan receptors gamma (RORɣt), interleukin-17A (IL-17A), and interleukin-22 (IL-22) mRNA expression were increased greatly in the patients group compared with the normal controls group (p < 0.05). Deregulated production of Th17-related molecules may be associated with the pathogenesis of AIH.

Wilson's disease (WD) is a genetic disorder with various clinical presentations due to excessive accumulation of copper in the liver and other organs. It can present as acute/chronic hepatitis, liver failure, extrahepatic and neuromuscular manifestations. Autoimmune hepatitis (AIH) is a necroinflammatory disease of the liver, which affects a lot of people particularly the children population. AIH has a broad clinical presentation that is similar to WD. Coexistence of WD with elevated creatinine phosphokinase (CPK) and AIH, may be a diagnostic dilemma.
Case Report: We presented a 6 years old boy with dysarthria, aggressive behavior, weak attention, concentration and weight loss with abnormal physical examination. Laboratory, histochemical, genomic studies, muscle/liver biopsy and atomic absorption test confirmed the diagnosis of both WD and AIH in the boy.
Although CPK and liver enzyme elevation is a rare presentation of chronic hepatitis with dominant feature of WD and AIH; however, simultaneous therapy with immunosuppressive drugs and Penicillamine may have superior benefit with a significant response.

Autoimmune hepatitis (AIH) is an inflammatory liver disorder that commonly affects women. The T cells and one of their major products, IFN-γ, are critically involved in the pathogenesis of AIH. Therefore, targeting of IFN-γ can probably be therapeutically useful while avoiding the long-term side effects of conventional immunosuppressive therapy. RNA interference, provides an ideal way to achieve this purpose. Thus, the aim of this study was to investigate the effect of IFN-γ-siRNA on IFN-γ expression in human peripheral blood mononuclear cells of patients with AIH.
In order to evaluate the anti-cytokine therapy with IFN-γ-siRNA, the PBMCs of AIH patients were cultured and transfected with IFN-γ-siRNA. After validation of transfection efficiency, the effects of gene silencing were tested with quantitative real-time polymerase chain reaction and intracellular flow cytometry.
The efficiently transfected cells, with the targeted IFN-γ-siRNA, without affecting cell viability showed a strong gene knockdown. The IFN-γ gene expression was significantly decreased in transfected cells (P Collectively, the results of the present study suggested that modifying the cytokine profile without inducing apoptosis using siRNA-based technology could be a promising tool for therapeutic intervention in T cells-dependent inflammatory diseases like AIH.

BACKGROUNDPrevious studies have indicated an elevated level of serum Interleukin (IL)-22 in patients with autoimmune hepatitis (AIH). However, there are no experimental data on the master transcription factor (aryl hydrocarbon receptor) that plays an important role in the development of T helper type 22 (Th22) cells as major producers of IL-22. The aim of the present study was to examine the expression of aryl hydrocarbon receptor in patients with AIH and in normal controls.
METHODSLevels of mRNA transcripts were measured in the peripheral blood mononuclear cells of 18 patients with AIH and compared with 18 normal controls by a quantitative real-time polymerase chain reaction.
RESULTSmRNA expression of aryl hydrocarbon receptor was significantly higher in patients with AIH compared with the healthy control group (P=0.006).
CONCLUSIONTh22 cells may play an important role in the pathogenesis of AIH.

Autoimmune hepatitis (AIH) is a relatively rare cause of hepatic dysfunction, which can lead to acute liver failure (ALV) and cirrhosis if not treated. The performance of transient elastography (TE) compared to liver biopsy has been evaluated in many liver diseases. The aim of the present study was to evaluate the performance of TE and other non-invasive markers for liver fiibrosis in patients with biopsy-proven AIH..
Fifty-three patients who were treated at the department of gastroenterology and hepatology of the University Clinic Essen from 2008 to 2013 included in this retrospective study. Laboratory parameters were used to calculate non-invasive markers for liver fiibrosis. Every patient underwent a liver biopsy within 6 months of the liver stiffness measurement..
Transient elastography score, non-alcoholic fatty liver disease (NAFLD) fiibrosis score, Fiibrosis 4 score (FIB-4), and FibroQ were associated with the stage of fiibrosis, whereas other non-invasive markers of liver fiibrosis (aspartate transaminase (AST) to alanine transaminase (ALT) ratio, and AST to platelet ratio index (APRI)) did not demonstrate a significant correlation. NAFLD fiibrosis score and FibroQ performed slightly better in ROC curve analysis than TE in differentiating mild to moderate from severe fiibrosis (AUC 0.895 and 0.773 vs. 0.739; P Transient elastography, NAFLD fiibrosis score, and FibroQ are valuable non-invasive markers for the evaluation of liver fiibrosis in autoimmune hepatitis but they cannot replace liver biopsy, especially in differentiating mild from more advanced stages of fiibrosis..

This prospective study was designed to examine the role of fibrosis staging on selection and success of treatment options for autoimmune hepatitis (AIH).
The project was conducted on 110 selected AIH patients who, based on the results of liver biopsy, had been assigned into one of the three groups (mild, stages 1 and 2, moderate, stages 3 and 4, and severe, stages 5 and 6 fibrosis). The patients received prednisolone alone or in combination with azathioprine and the response to the treatment were assessed.
The number of patients who were identified to have mild, moderate and severe fibrosis were 34 (31%), 35 (32%), and 41 (37%), respectively. Of 110 patients, 56 patients (51%) received prednisolone alone and 54 patients (49%) received combined drugs protocol. In total, 77 patients (70%) showed response to the treatment. The response rate for both modalities was much lower in the third group (POur results clearly showed that response to treatment in AIH patients is decreased as hepatic fibrosis becomes more severe. Our findings indicate that pathological staging could navigate the selection of appropriate therapy, i.e. prednisolone alone is used for mild and moderate fibrosis while combination therapy is reserved for severe cases.

An 11-year-old female patient with autoimmune hepatitis (AIH) was referred to our rheumatology clinic due to her current musculoskeletal manifestations. The patient had been diagnosed with AIH 3 months previously, based on jaundice and impaired liver function tests, and she had been treated with low-dose prednisolone and azathioprine. She presented with malaise, arthritis, a malar rash on the face, and oral ulcers. Laboratory tests revealed a positive ANA/anti-dsDNA test. Liver biopsy showed chronic hepatitis with severe inflammatory activity, in favor of a diagnosis of definite AIH. She fulfilled the international criteria for both SLE and AIH. The clinical symptoms and laboratory findings of SLE improved with ongoing treatment with corticosteroids and azathioprine, accompanied with hydroxychloroquine sulfate. The present case indicates that AIH can be the first manifestation of SLE in children.

Aim: We hypothesized that AIH outcomes might be different in our patient population that consists of a large number of Latinos.
Literature has suggested that the presentation and outcome of autoimmune hepatitis can be different among different ethnicity and communities.
Patients and We performed a retrospective chart review of Latino patients with AIH diagnosed between 2002-2012. Complete and partial remissions were defined as normalization of liver enzyme values, or achieving less than twice the upper limit normal (ULN), respectively.
A total of 28 patients were identified. 26 (93%) were female. 13 (46%) had an acute presentation, one with type 2 AIH and 3 with ANA seronegative disease. The average pathologic stage (Ishak score) was 3.44±1.67 (range: 0-6). Complete and partial remission was achieved in 20 (71%) and 5 (18%) patients respectively. Ten patients (38%) required maintenance prednisone either alone (2), or in combination with Azathioprine (6) or Mycophenolate Mofetil (2). Remission in the majority of patients, including 14 (50%) who were cirrhotic. Six of 14 (43%) cirrhotic patients were asymptomatic at the time of diagnosis.
In an urban Latino population, cirrhosis was the initial presentation of AIH in a significant percentage of patients raising concerns regarding insufficient screening for AIH in this patient population. A large number of patients required continuous prednisone to avoid relapse.