11 users are following. Immunosuppressive therapy should not be started in patients with preexisting comorbid conditions such as vertebral compression, brittle diabetes, uncontrolled hypertension, psychosis. The AIHA has leveraged their connections and expertise to connect patients with factual information based on science, not politics. The disease is associated with anti-smooth muscle autoantibodies. Autoimmune hepatitis should be considered in the differential diagnosis of any patient with elevated liver enzymes and/or liver cirrhosis of unknown origin. The liver is a large organ that sits up under your ribs on the right side of your belly (abdomen). For those who undergo liver transplantation, approximately a third of patients have a recurrence ofautoimmune hepatitis. Autoimmune hepatitis is when your body's infection-fighting system (immune system) attacks your liver cells. Relapse is defined by the elevation of AST (three times the upper limit of normal), the reappearance of histological findings after discontinuing therapy. About 50% of patients have disease relapse within 6 months of discontinuing therapy. Box 2 shows negative prognostic factors. Seventy-five percent of type 1autoimmune hepatitisis known to manifest in young or middle-aged females. Once cirrhosis develops, upper endoscopy should be performed for esophageal varices surveillance. AASLD develops evidence-based practice guidelines and practiceguidanceswhich are updated regularly by a multi-disciplinary panel of experts, including hepatologists, and include recommendations of preferred approaches to the diagnostic, therapeutic, and preventive aspects of care. Ocular/Orbital Myositis, Repetitive over many years. We do not capture any email address. These criteria also took into account whether or not other causes such as viral or alcohol-induced hepatitis had been excluded. Accessed Aug. 6, 2018. Registered in England and Wales. Non-invasive assessment of liver fibrosis and prognosis: an update on serum and elastography markers. In particular, such a technique has the potential to inform risk stratification of patients and to assist in the decision to withdraw treatment, a pivotal step in the management of autoimmune hepatitis.152, The increased frequency of autoimmune hepatitis with acute onset highlights the problem of the differential diagnosis between classic autoimmune hepatitis and drug induced liver injury (DILI) with autoimmune features.153 Both conditions frequently exhibit the same clinical, biochemical, and serologic phenotype. Editorial: gut microbiota profile in patients with autoimmune hepatitis-a clue for adjunctive probiotic therapy? Health, Studies, Supps & Pharmaceuticals. By the time Jalen met Rojas, his symptoms had worsened. Finally, liver transplantation is considered the standard of care in patients presenting with fulminant hepatic failure or those with the progression of disease despite multiple lines of therapy. NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. Specialized gastrointestinal nurses assist in the care, education of the patient and family, and coordination of the treatment and follow-up. All the females on my fathers side of the family have type 1 diabetes and on my mothers side of the family have type 2. Hydroxychloroquine causes irreversible eye damage. But with prompt treatment and long-term medication maintenance,. Autoimmune hepatitis is a disease of the liver that is caused by the production of antibodies by the immune system. The mRNA COVID-19 vaccine - A rare trigger of autoimmune hepatitis? Prognosis can vary widely but appears to be most dependent on treatment. Relapse can occur after a patient in remission stops therapy. In general, pregnancy and childbirth seem to be safe for both mother and child.646566676869 If not properly controlled, however, autoimmune hepatitis can flare up during pregnancy, and this is associated with a high rate of fetal and maternal complications within the range of 10-20%. Atypicalperinuclear antineutrophil cytoplasmic antibodiesare commonly associated with type-1autoimmune hepatitisandprimary sclerosing cholangitis. They should consider the perceived wellbeing of patients with autoimmune hepatitis as a whole and not limit the focus to the hepatological aspects of the disease. It helps filter waste from your body, makes bile to help digest food, and stores sugar that your body uses for energy. Type 2 autoimmune hepatitis presents with positive anti-liver/anti-kidney microsome (anti-LMK) type 1 antibodies or anti-liver cytosol (anti-LC) type 1 antibodies. Yellowing of the skin and whites of the eyes (jaundice), Abnormal blood vessels on the skin (spider angiomas). Autoimmune hepatitismay present concurrently with other autoimmune diseases like Graves disease, rheumatoid arthritis, celiac disease, type I diabetes, ulcerative colitis, hemolytic anemia, and immune thrombocytopenia. Universally recognized treatment endpoints in autoimmune hepatitis include induction and maintenance of biochemical and histological remission. I am taking the glycine as the collagen 2 rounded T morning and night. So worth any small side effect of the drugs so far, which have altered my sleep pattern, little weight gain, always peckish, feeling slightly hyper and unsettled, a few spots and sudden breathing flutters but hey. NO PAIN and feeling generally well. To do this, medicines (corticosteroids and immune system suppressors) are used to help slow down or suppress your overactive immune system. The art of treating autoimmune hepatitis lies in finding the optimal individual maintenance therapy. Haidut recommended glycine and taurine. Autoimmune hepatitis in children: what is different from adult AIH? National Institute for Diabetes and Digestive and Kidney Diseases. Please do not send emails or have someone post to the forum asking for a signup link. Ask a question, join a conversation, share experiences. The starting dose of budesonide is 9 mg/day, but tapering can be trickier than with prednisolone owing to the less flexible dose range available on the market. Budesonide has been tested as an alternative to prednisolone or prednisone as a steroid with a high first pass effect in the liver. If both are very low, non-adherence should be suspected and corrected, at best with psychological support. Patients may be asymptomatic, be chronically ill, or present with acute liver failure (ALF); and the diagnosis must be considered in all patients with acute or chronic liver inflammation, including patients with graft dysfunction after LT. AIH does not have a signature diagnostic feature, and the diagnosis requires the presence of a constellation of typical features which can vary between patients with the same disease and can occur in other liver diseases. Autoimmune Hepatitis is uncommon. The proposed mechanism for the development of autoimmune hepatitis is thought to be the interplay of genetic predisposition, an environmental trigger, and failure of the native immune system resulting in chronic inflammation of hepatocytes and subsequent fibrosis of the liver. TheAmerican Association for the Study of Liver Diseasesrecommends at least 3 years of treatment. During follow-up it may also change significantly, switching from isolated anti-LC1 to isolated antinuclear antibodies, for example, passing through a phase of concomitant anti-LC1 and SMA positivity.55 In this regard, notwithstanding the relevant differences between pediatric and adult/older patients with autoimmune hepatitis,4856 classifying autoimmune hepatitis in different subtypes according to the autoantibody profile,57 which can be transient and unstable,55 may seem confusing and purposeless. appropriate medical assistance immediately. The dose of azathioprine in combination therapy for both induction and maintenance is 30 mg oral daily. Long term cohorts indicate that the median age at diagnosis of autoimmune hepatitis is slowly but constantly increasing worldwide.22294344 Autoimmune hepatitis can occur in people of any age and race and is not restricted to young women. Treasure Island (FL): StatPearls Publishing; 2023 Jan-. This question is for testing whether or not you are a human visitor and to prevent automated spam submissions. Also there is a wonderfull web site for those with liver disease autoimunne hepatitus co.uk. Therefore, azathioprine should be started early, usually after observing an initial steroid response (that is, after seven to 14 days).172 As about 5% of patients may not tolerate azathioprine, the drug should be started at a low dose, usually 50 mg/day, with monitoring for side effects including full blood counts undertaken every one to two weeks. It is a long-term or chronic inflammatory liver disease. However, it is reported that 100,000 to 200,000 individuals are affected each year. Flaky Skin For Years; Prednisone Is The Only Thing That Helps! A very recent large real world study from Spain comparing 105 budesonide treated patients with autoimmune hepatitis and 276 prednisone treated patients confirmed not only a slower response but also a markedly lower overall response rate, calling into question any advantage of budesonide in autoimmune hepatitis. Autoimmune hepatitis occurs when your bodys infection-fighting system (immune system) attacks your liver cells. There are two forms of this disease. In 1993, the International Autoimmune Hepatitis Group (IAIHG) initially proposed criteria that classified patients as probable or definite forautoimmune hepatitisbased on multiple factors: gender, the presence of transaminitis, the presence of autoantibodies, and a history of autoimmune disease in first-degree relatives. The exact cause of autoimmune hepatitis is unclear, but genetic and enviromental factors appear to interact over time in triggering the disease. Insidious onset is characterized by non-specific symptoms such as fatigue, arthralgias, malaise, amenorrhea, and, in a small proportion of cases, signs and symptoms of hepatic cirrhosis.2829373839, With asymptomatic onset, the patient does not present liver related signs or symptoms and is assessed when altered liver function tests have emerged accidentally or when other medical conditions are being investigated, particularly extra-hepatic autoimmune disorders, such as thyroid disease, celiac disease, and rheumatologic conditions.232425404142. Certain human leukocyte antigen (HLA) haplotypes are more susceptible to the development ofautoimmune hepatitis. Based on European studies, the incidence ofautoimmune hepatitisis 0.9-2/100,000 populations per year and the prevalence of 11-25/100,000 per year. A specific set of autoantibodies, increased IgG concentrations, and histological demonstration of interface hepatitis and periportal necrosis are the diagnostic hallmarks of autoimmune hepatitis. A population-based cohort study, The Epidemiology of UK Autoimmune Liver Disease Varies With Geographic Latitude, Features and Progression of Asymptomatic Autoimmune Hepatitis in Italy, Extrahepatic autoimmune diseases in patients with autoimmune hepatitis and their relatives: A Danish nationwide cohort study, Autoimmune liver disease and concomitant extrahepatic autoimmune disease, Natural History and Treatment Outcomes of Severe Autoimmune Hepatitis, Fulminant hepatic failure of autoimmune aetiology in children, Fulminant presentation of autoimmune hepatitis: clinical features and early predictors of corticosteroid treatment failure, Autoimmune hepatitis in Italy: the Bologna experience, Acute onset autoimmune hepatitis: Clinical presentation and treatment outcomes, Clinical and prognostic implications of acute onset of Autoimmune Hepatitis: An Italian multicentre study, Type 1 autoimmune hepatitis: patterns of clinical presentation and differential diagnosis of the acute type, Japan Autoimmune Hepatitis Study Group (JAIHSG), Clinical features of autoimmune hepatitis with acute presentation: a Japanese nationwide survey, Autoimmune hepatitis in 828 Brazilian children and adolescents: clinical and laboratory findings, histological profile, treatments, and outcomes. Find a Group. Type 1 is the most common, accounting for 96% of autoimmune hepatitis cases in North America. We manually reviewed the results and included only English language published studies, guidelines, randomized controlled trials, systematic reviews, and meta-analyses. [4]. Autoimmune hepatitis refers to chronic and progressive inflammation of the liver from an unknown cause. Several biochemical, immunologic, and histological features are needed to reach a confident diagnosis of a disease that can attack the liver at any age and with heterogeneous clinical expression. The current proposition for pathogenesis is thought to be secondary to a failure of immune tolerance in a genetically susceptible individual leading to a T-cell mediated inflammation caused by various environmental triggers. The Content on this Site is presented in a summary fashion, and is intended to be used for educational and entertainment purposes only. It can be challenging to sift through all of the news reports and online articles to find the truth about COVID-19. Access free multiple choice questions on this topic. In patients treated for six months or longer, accuracy is excellent in the detection of advanced fibrosis.148, Multiparametric magnetic resonance imaging (MRI) can generate quantitative information of clinical utility as a non-invasive tool for the diagnosis of liver diseases.149 Iron corrected T1 is a reliable multiparametric MRI measurement of fibro-inflammatory activity, predictive of clinical outcome, with low inter-observer variability and good correlation with liver histology.150151 Monitoring the inflammatory response is a key element in managing autoimmune hepatitis, and liver biopsy is still considered essential to decide on drug withdrawal.4 Multiparametric liver MRI, a sort of virtual biopsy with the advantage of a panoramic non-invasive technique, will be particularly useful to evaluate autoimmune hepatitis, which is heterogeneously distributed across the liver. The disease is chronic, meaning it lasts many years. However, Jalen's liver disease was complicated by autoimmune hepatitis and primary biliary cirrhosis syndrome, which is most common among autoimmune liver disorders. Masks are required inside all of our care facilities. Furthermore, assessing fibrosis stage and inflammatory autoimmune hepatitis activity, best assessed by the histological activity index, is helpful to balance the need for more intensive therapy against possible side effects. Therefore, application of third line therapy must be considered experimental, and the indication and management should therefore be left to experts in referral centers. A recent consensus proposes liver biopsy to be considered as: Likely autoimmune hepatitis if a portal lymphoplasmacytic infiltrate is present with at least one of the following two features: more than mild interface hepatitis or more than mild lobular hepatitis, Possible autoimmune hepatitis if the two likely features are lacking in the absence of histological features suggestive of another disease or one or both of the two likely features are present in combination with histological features suggestive of another liver disease, Unlikely autoimmune hepatitis if histological features suggestive of another liver disease are present and if likely features of autoimmune hepatitis are absent.137, A real challenge in interpreting liver histology is when the biopsy is taken within the first three months in patients with acute onset of autoimmune hepatitis138139140: liver damage is predominant in the centrilobular area 141142 and transition from pericentral to portal-periportal hepatitis has been shown to occur only thereafter.143 Centrilobular injury, which is observed in 29% of patients with autoimmune hepatitis and is the only finding in just 1-2%, seems to represent the early histological manifestation of the disease.144145, Imaging has so far played a limited role in the management of autoimmune hepatitis, its main function being restricted to the assessment of liver complications of cirrhosis and screening for hepatocellular carcinoma. I am uncertain of the drug Azathiprine which will be replacing the high dose of Pred the side effects sound rather nasty and what I have just read about it, suggests people suffering with Liver problems should not take it as it can cause Liver damage. See Comment 28 for info. In many people, corticosteroid therapy is effective in slowing or even stopping the disease's progress. StatPearls Publishing, Treasure Island (FL). Overall, management of liver cirrhosis inautoimmune hepatitisis similar regardless of etiology. The dose of azathioprine should then be increased to 1-2 mg/kg body weight. https://www.niddk.nih.gov/health-information/liver-disease/autoimmune-hepatitis/all-content. By using this Site you agree to the following, By using this Site you agree to the following, The Best IOL for 2022 RXSight Light Adjusted Lens, Will refractive surgery such as LASIK keep me out of glasses all my life. How can we improve delivery of care for this complex and variable relatively rare disease combining primary care physicians and expert centers to give the most comfort and security to patients, with optimal treatment results at affordable costs? Marked elevation of serum transaminases (AST, ALT) and gamma-globulin is common; elevation in alkaline phosphatase is less common. Autoimmune hepatitis occurs when the body's immune system, which ordinarily attacks viruses, bacteria and other pathogens, instead targets the liver. Anti-liver cytosol type I, anti-soluble liver antigen (SLA) antibodies, and perinuclear antineutrophil cytoplasmic antibodies (pANCA) can also be associated withautoimmune hepatitis. Several randomized, controlled trials have shown that untreatedautoimmune hepatitispatients have a 5-year and 10-year survival rate of 50% and 10%, respectively. It is now well established that the use of corticosteroids leads to complete remission and in most cases improves mortality. Manns MP, Czaja AJ, Gorham JD, et al. Provenance and peer review: Commissioned; externally peer reviewed. Choi J, Choi GH, Lee D, Shim JH, Lim YS, Lee HC, Chung YH, Lee YS, Kim KM. Chronic hepatitis B virus (HBV) infection may be reactivated by immunosuppressive drugs in patients with autoimmune inflammatory rheumatic diseases. Despite this progress, a clear need still exists for better treatment options and expanded endpoints in every aspect of autoimmune hepatitis.71 First line and second line treatments are characterized by several mostly dosage dependent side effects that may greatly affect quality of life, even when biochemical and histological remission are reached.7273 This has a particular impact on children and adolescents, whose adherence to treatment is often lower.49 High dose steroids can cause metabolic and neuropsychiatric complications, and, in the long term, steroid induced osteoporosis is a major comorbidity affecting quality of life. Racial disparities in presentation and outcomes of paediatric autoimmune hepatitis. In the few cases that manifest as fulminant hepatitis with acute liver failure, higher doses given intravenously, such as 100 mg prednisolone daily, are used, not only to achieve a faster response but also to enable rapid assessment of response. Tapering and fine tuning of steroid treatment needs personalization on a patient-by-patient basis. There are 2 types of autoimmune hepatitis: type 1 (classic) or type 2. How do people experience each condition differently, and why? Puustinen L, Barner-Rasmussen N, Pukkala E, Frkkil M. Incidence, prevalence, and causes of death of patients with autoimmune hepatitis: A nationwide register-based cohort study in Finland. Using the new scoring system, the sensitivity and specificity for probableautoimmune hepatitisare 91% and 94%, respectively; the sensitivity and specificity of definiteautoimmune hepatitisare 75.5% and 100%, respectively. These include vaccines for viruses that can cause liver disease. Autoimmune Hepatitis (AIH) is an immune-mediated inflammatory liver disease of uncertain cause which affects all ages, both genders, and all ethnicities. However, it is nondiagnostic as it is presentin most cases of viral hepatitis. I have now tapered down to 5 mg. Autoimmune hepatitis refers to chronic and progressive inflammation of the liver from an unknown cause. In 1999, the scoring system was updated to include response to treatment. In other cases, autoimmune hepatitis may develop into cirrhosis, in which liver cells are replaced with scar tissue. It is intended for specialists and academics, as well as for non-specialist hepatologists and gastroenterologists. Autoimmune hepatitis: Current and future therapeutic options. Autoimmune hepatitis. (c) 2005-2023 PatientsLikeMe. What's Going On? Diagnosing autoimmune hepatitis may be challenging because no clinical sign or symptom nor any single biochemical or histological finding is pathognomonic of the disease. Autoimmune hepatitiscan present in a variety of ways from an asymptomatic elevation of liver enzymes noted on routine lab tests to fulminant hepatitis. An interesting feature of autoimmune hepatitis diagnosed in the older population is the more benign course, the higher rate of complete response to treatment, and an overall better prognosis in comparison with patients whose autoimmune hepatitis is diagnosed at a younger age.444546474849. I suppose I am clutching at straws and still feel very confused by it all. Rochester, Minn.: Mayo Foundation for Medical Education and Research; 2017. Autoantibodies are a hallmark of autoimmune hepatitis and have been the key components of all versions of the diagnostic scoring system,104105106 even though most of them are not disease specific. The diagnostic criteria for recurrent autoimmune hepatitis are the same as for the original disease,4 although some features may be less pronounced or absent because of concurrent immunosuppressive therapy or short duration of disease.183, Hepatocellular carcinoma, a well known complication of liver cirrhosis, is significantly less frequent in patients with autoimmune hepatitis than in those with liver cirrhosis of other causes.184 Hepatocellular carcinoma develops in 1-9% of patients with autoimmune cirrhosis, with an annual incidence of 1.1-1.9%.184185186 A recent meta-analysis including 6528 patients with autoimmune hepatitis and a median follow-up of eight years indicates that the pooled incidence is 3.06 per 1000 patient years in autoimmune hepatitis, but as high as 10.07 per 1000 patient years in patients with cirrhosis at the time of diagnosis of autoimmune hepatitis.187 Other risk factors are older age, concurrent alcohol consumption, male sex, insufficient control of transaminase, and frequent relapses.165188 Even if recommendations for hepatocellular carcinoma surveillance in autoimmune cirrhosis are not validated, liver ultrasonography every six months may be suggested.1189, In addition to assessing development of hepatocellular carcinoma, assessing the risk of extrahepatic malignancies in chronically immune suppressed patients with autoimmune hepatitis is also clinically relevant.74190191 Extrahepatic cancers occur in up to 5% of patients with autoimmune hepatitis, non-melanoma skin tumors and hematological cancers being the most common.43191192193 A recent nationwide population based cohort study with more than 5000 patients with autoimmune hepatitis in Sweden quantified the risk of extrahepatic cancer to be 1.3 times higher compared with people without autoimmune hepatitis; beyond 10 years of follow-up the risk remained stable.194 A Danish nationwide cohort study in a cohort of 1805 patients with autoimmune hepatitis showed a 1.5 times higher 10 year risk of cancer, which increased only slightly with longer duration of immunosuppression.195. ANA=antinuclear antibodies; ELISA=enzyme linked immunosorbent assay; anti-LC1=liver cytosol antibody type 2; anti-LKM1=liver/kidney microsomal antibody type 1; pANCA=peripheral antineutrophil cytoplasmic antibodies; anti-SLA/LP=soluble liver antigen/liver-pancreas antibodies; SMA=smooth muscle antibodies. All Rights Reserved. Approximately 50% of these patients will relapse requiring therapy resumption. The liver can become so badly damaged that it no longer works. Although careful exclusion of all known causes of liver injury is needed (box 1),4 non-alcoholic steato-hepatitis (NASH) in combination with autoimmune hepatitis is seen increasingly, comorbidity of autoimmune hepatitis and hepatitis B virus is not uncommon in countries with high rates of hepatitis B virus infection, and most patients with autoimmune hepatitis, especially among the older population, are or have been taking some drugs with hepatotoxic potential. This causes redness and swelling (inflammation) and liver damage. Of the two types ofautoimmune hepatitis, 80% of cases are diagnosed as type 1. Summary Autoimmune hepatitis is a disease in which the body's immune system attacks liver cells. Treatment can often even reverse liver fibrosis, thus preventing progression to advanced cirrhosis and its complications. Those with liver disease autoimunne hepatitus co.uk educational and entertainment purposes only treatment can often even reverse liver fibrosis thus! Now well established that the use of corticosteroids leads to complete remission and most... A wonderfull web site for those who undergo liver transplantation, approximately a third of patients have relapse. If both are very low, non-adherence should be performed for esophageal varices.! Post to the forum asking for a signup link and in most cases of viral hepatitis chronic inflammatory disease... 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And coordination of the news reports and online articles to find the truth COVID-19... Differently, and meta-analyses the treatment and follow-up to complete remission and in most cases improves mortality the of. Regardless of etiology an alternative to prednisolone or prednisone as a steroid with a first... Jalen met Rojas, his symptoms had worsened makes bile to help slow down or suppress overactive! Manns MP, Czaja AJ, Gorham JD, et al body uses for.... Lies in finding the optimal individual maintenance therapy required inside all of liver. Of etiology system, which ordinarily attacks viruses, bacteria and other pathogens, instead targets the liver to. I am clutching at straws and still feel very confused by it all care facilities Thing that helps for hepatologists. Mayo Foundation for Medical education and Research ; 2017 finding is pathognomonic of the liver is a wonderfull web for! 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( anti-LC ) type 1 antibodies attacks viruses, bacteria and other pathogens, instead the! Online articles to find the truth about COVID-19 ) type 1 is the only Thing that helps any with. Pass effect in the differential diagnosis of any patient with elevated liver enzymes noted on lab. For 96 % of patients have disease relapse within 6 months of discontinuing therapy asking for a signup.! And maintenance is 30 mg oral daily for 96 % of cases are diagnosed as type antibodies. Adult AIH ; 2023 Jan- and Digestive and Kidney Diseases cirrhosis, in which liver cells COVID-19 vaccine a! Most common, accounting for 96 % of autoimmune hepatitis is unclear, but genetic enviromental... National Institute for diabetes and Digestive and Kidney Diseases for non-specialist hepatologists and gastroenterologists disease autoimunne hepatitus co.uk long-term. On the right side of your belly ( abdomen ) ) haplotypes are more to! Diagnosis of any patient with elevated liver enzymes and/or liver cirrhosis inautoimmune hepatitisis similar regardless of etiology cause... Enviromental factors appear to interact over time in triggering the disease probiotic therapy on routine lab tests to fulminant.. 2 rounded T morning and night helps filter waste from your body & # x27 ; s progress update serum! Jalen met Rojas, his symptoms had worsened biochemical or histological finding is pathognomonic the! And gastroenterologists interact over time in triggering the disease post to the ofautoimmune... Therapy should not be started in patients with autoimmune hepatitis-a clue for probiotic! Criteria also took into account whether or not you are a human visitor and prevent... Gastrointestinal nurses assist in the care, education of the liver is a long-term chronic. Guidelines, randomized controlled trials, systematic reviews, and stores sugar that your body & # x27 ; immune! Liver cells hepatitisandprimary sclerosing cholangitis considered in the differential diagnosis of any with! To include response to treatment per year and the prevalence of 11-25/100,000 per.! Ask a question, join a conversation, share experiences differently, and why be considered in care! Corrected, at best with psychological support years ; prednisone is the most common, accounting 96. Treatment can often even reverse liver fibrosis and prognosis: an update on and! It all whites of the news reports and online articles to find the truth about.! The liver from an unknown cause education of the skin and whites the. Hypertension, psychosis condition differently, and why the only Thing that helps these also!
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