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Nakano M.,Shonan Fujisawa Tokushukai Hospital | Fukuda Y.,Seikeikai Chiba Medical Center | Oda S.,Chiba University
Hepatology Research | Year: 2015

Aim: The outcome of acute liver failure (ALF) is influenced by its etiology, making etiological consideration of ALF important. However, specific etiology could not be identified in 30-40% of adult patients in a Japanese nationwide survey. We examined our patients with severe (SH) and fulminant hepatitis (FH) of indeterminate etiology for the better understanding of ALF. Methods: We investigated 106 adult patients with SH or FH including 24 of indeterminate etiology between 2000 and 2013, retrospectively. Results: Of 24 patients, 12 were men. Seventeen were SH and seven FH (three FH acute type and four FH subacute type). Eighty-three percent of patients were positive for antinuclear antibody. Seventeen recovered without liver transplantation (LT), two received LT and five died without LT. Histology of 15 patients showed a pattern of acute hepatitis (massive necrosis in four, submassive necrosis in one, severe acute hepatitis in two and acute hepatitis in eight). The involvement of immune-mediated liver injury was histologically suggested in some patients. Conclusion: There was no large cluster of etiology in our patients with indeterminate cause. The causes of ALF of indeterminate etiology were the mixture of various minor or rare ones, if precise diagnosis of acute AIH was done. Outcome of our patients with indeterminate cause was not poor if they were treated as early as possible after the diagnosis of severe disease. Careful examination of unknown viral infection, drugs, toxins, undefined metabolic disorders and histology may help detect some of these etiologies. © 2015 John Wiley & Sons, Ltd. Source

Fujiwara K.,Chiba University | Yasui S.,Chiba University | Yonemitsu Y.,Chiba University | Arai M.,Chiba University | And 5 more authors.
Journal of Hepato-Biliary-Pancreatic Sciences | Year: 2016

Background Autoimmune hepatitis (AIH) is one of major etiologies of acute liver failure (ALF), and the survival rate without liver transplantation (LT) of patients with fulminant AIH is especially poor worldwide. We investigated the clinicopathological features of infectious complications in autoimmune ALF retrospectively and tried to determine when to continue corticosteroid (CS) treatment or abandon it for LT. Methods Twenty patients with autoimmune ALF, comprising five severe hepatitis, 13 fulminant hepatitis and two late onset hepatic failure, were analyzed. Results Corticosteroids were administered to 19 patients. Seventeen infectious complications were observed in 12 patients. The median (range) duration between the introduction of CS and onset of infection was 15 (10-41) days. There were no significant differences in clinicobiochemical features between patients with and without infection. Of 20 patients, eight (40%) recovered without LT, four (20%) received LT and eight (40%) died without LT. Dead or transplanted patients had more advanced liver failure on admission than recovered ones (P < 0.01). Conclusions Two-week after the introduction of CS is a critical point for avoiding infectious complications. Therefore, we should have evaluated efficacy of CS and performed LT by then at the latest in case of failure to improve. Highlight Fujiwara and colleagues reveal that the critical point for switching to liver transplantation without infectious complications in autoimmune acute liver failure is two weeks after the start of corticosteroid treatment. It is crucial to evaluate corticosteroid efficacy and, if no improvement is seen, to perform liver transplantation by that time. © 2016 Japanese Society of Hepato-Biliary-Pancreatic Surgery. Source

Kurata Y.,Seikeikai Chiba Medical Center | Kurata Y.,Chiba University | Takaishi S.,Seikeikai Chiba Medical Center | Sakuma Y.,Seikeikai Chiba Medical Center | And 6 more authors.
Japanese Journal of Cancer and Chemotherapy | Year: 2014

A 65-year-old woman with a gastrointestinal stromal tumor (GIST) underwent a total gastrectomy in 1999. In 2004, she was diagnosed with an intra-abdominal recurrence and was treated with 300 mg/day of imatinib. Because of the side effects of imatinib, we interrupted the treatment and were forced to reduce the dose from 300 mg/day to 100 mg/day. However, at present, the tumor remains controlled. In conclusion, this case suggested that, even if given irregularly or at a low-dose, continuous treatment with imatinib might contribute to long-term survival in patients with GIST. Source

Fujiwara K.,Chiba University | Seza K.,Seikeikai Chiba Medical Center | Fukuda Y.,Seikeikai Chiba Medical Center | Nakano M.,Ofuna Chuo Hospital | Yokosuka O.,Chiba University
Clinical Journal of Gastroenterology | Year: 2013

Autoimmune hepatitis (AIH) is generally regarded as a clinically and histologically "chronic" hepatitis. It often shows acute presentation like acute hepatitis without typical clinicopathological features of AIH, especially in a case of overlap with primary biliary cirrhosis (PBC). A 52-year-old man showed mild liver dysfunction for the first time at an annual medical check. Two months later, he showed jaundice, and laboratory tests revealed elevation of liver enzymes, hyperbilirubinemia and prolonged prothrombin time activity like acute liver failure. Anti-mitochondrial antibody was positive and other viral and autoimmune markers were negative. His liver function tests improved upon treatment with ursodeoxycholic acid and maximum intravenous glycyrrhizin (IVGL), but liver dysfunction was again exacerbated after the gradual reduction of IVGL. He showed transient elevation of immunoglobulin G (IgG) and anti-nuclear antibody (ANA) at only one point, and liver histology was compatible with PBC and AIH overlap syndrome. Corticosteroid was administered and his liver function tests returned to normal. It is important for the diagnosis of acute onset AIH to monitor IgG level and ANA titer, especially in patients without IgG and ANA elevations at first appearance. © Springer 2013. Source

Fujiwara K.,Chiba University | Fukuda Y.,Seikeikai Chiba Medical Center | Seza K.,Seikeikai Chiba Medical Center | Saito M.,Seikeikai Chiba Medical Center | And 3 more authors.
Journal of Hepato-Biliary-Pancreatic Sciences | Year: 2016

Background There is, as yet, no gold standard for making the diagnosis of acute onset autoimmune hepatitis (A-AIH). Novel histological characteristics have been reported, but etiologies other than AIH could show similar histological pattern. We attempted to determine what clinical characteristics we should consider as A-AIH different from other etiologies, and to whom histological characteristics should be applied for the diagnosis. Methods Clinical, biochemical, immunological and pathological features of 46 patients (35 women, mean age 55.9 ± 14.2 years) with non-severe A-AIH admitted to a community hospital between 2001 and 2015 were analyzed. Results Immunoglobulin G level was normal in 28%, and anti-nuclear antibody titer was < × 80 in 28%. Liver histology of 49% showed acute form and 51% chronic one. Centrilobular necrosis/collapse and/or plasma cell accumulation, rosette formation were characteristic for A-AIH. High levels of alanine aminotranferase persisted in 21 patients who could be observed for equal to or more than 4 weeks before the start of treatment. Conclusions Long persistence of high levels of alanine aminotransferase would be one of clinical features for considering A-AIH along with conventional features. Histological diagnostic features should be applied for such patients. Guidelines for diagnosing A-AIH should be urgently drawn up. Highlight There is no gold standard for the diagnosis of acute onset autoimmune hepatitis. On the basis of their experience with treatment-naïve patients referred without diagnosis after long observation, Fujiwara and colleagues show that persistence of high alanine aminotransferase levels is one of the clinical features suggesting acute onset autoimmune hepatitis. © 2016 Japanese Society of Hepato-Biliary-Pancreatic Surgery. Source

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