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Acute onset of autoimmune hepatitis in children and adolescents

Hepatobiliary & Pancreatic Diseases International 2020年第1期19卷 17-21页

作者： Vratislav Smolka Oksana Tkachyk Jiri Ehrmann Eva Karaskova Martin Zapalka Jana Volejnikova Department of Pediatrics Faculty of Medicine and DentistryPalacky University and University Hospital OlomoucI.P.Pavlova 185/6Olomouc 77900Czech Republic Department of Clinical and Molecular Pathology Faculty of Medicine and DentistryPalacky University and University Hospital OlomoucI.P.Pavlova 185/6Olomouc 77900Czech Republic

Background: Autoimmune hepatitis (AIH) is a rare progressive liver disease, which manifests as acute hepatitis in 40%-50% of pediatric cases. This refers predominantly to spontaneous exacerbations of previ- ously unrecognized subclinical AIH with laboratory and histological signs of chronic hepatitis, or to acute exacerbations of known chronic disease. Only a few of these patients ful ll criteria for acute liver failure (ALF). Methods: Forty children diagnosed with AIH in our center between 2000 and 2018 were included in this study. All of them ful lled revised diagnostic criteria of the International Autoimmune Hepatitis Group (IAIHG) for probable or con rmed AIH, and other etiologies of liver diseases were excluded. Patients were divided into two groups: acute AIH (A-AIH) or chronic AIH (C-AIH). Results: Acute onset of AIH occurred in 19/40 children (48%). Six of them ful lled the criteria of ALF with coagulopathy and encephalopathy. Five of 6 children with ALF suffered from exacerbation of previ- ously undiagnosed chronic AIH, among which 4 children were histologically con rmed as micronodular cirrhosis. The remaining one patient had fulminant AIH with centrilobular necrosis, but no histological signs of previous chronic liver damage. We observed signi cantly lower levels of albumin, higher levels of aminotransferases, bilirubin, INR, IgG, higher IAIHG score and more severe histological ndings in A- AIH than in C-AIH. No differences in patient age and presence of autoantibodies were observed between A-AIH and C-AIH. All children, including those with ALF and cirrhosis, were treated with corticosteroids, and are alive and achieved AIH remission. Liver transplant was not indicated in any patient. Conclusion: Rapid and accurate diagnosis of A-AIH may be di cult. However, timely start of immunosup-pressive therapy improves prognosis and decreases number of indicated liver transplantations in children with AIH.

关键词： Acute liver failure Autoimmune hepatitis Children Onset

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Long-term follow-up of children and adolescents with primary sclerosing cholangitis and autoimmune sclerosing cholangitis

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Hepatobiliary & Pancreatic Diseases International 2016年第4期15卷 412-418页

作者： Vratislav Smolka Eva Karaskova Oksana Tkachyk Kvetoslava Aiglova Jiri Ehrmann Kamila Michalkova Michal Konecny Jana Volejnikova Department of Pediatrics Faculty of Medicine and DentistryPalacky University and University Hospital OlomoucI.P.Pavlova 6Olomouc 77900Czech Republic Department of Internal Medicine Faculty of Medicine and DentistryPalacky University and University Hospital OlomoucI.P.Pavlova 6Olomouc 77900Czech Republic Department of Clinical and Molecular Pathology Faculty of Medicine and DentistryPalacky University and University Hospital OlomoucI.P.Pavlova 6Olomouc 77900Czech Republic Department of Radiology Faculty of Medicine and DentistryPalacky University and University Hospital OlomoucI.P.Pavlova 6Olomouc 77900Czech Republic

BACKGROUND： Sclerosing cholangitis（SC） is a chronic cholestatic hepatobiliary disease with uncertain long-term prognosis in pediatric patients. This study aimed to evaluate longterm results in children with SC according to the types of SC.METHODS： We retrospectively followed up 25 children with SC over a period of 4-17 years（median 12）. The diagnosis of SC was based on biochemical, histological and cholangiographic findings. Patients fulfilling diagnostic criteria for probable or definite autoimmune hepatitis at the time of diagnosis were defined as having autoimmune sclerosing cholangitis（ASC）; other patients were included in a group of primary sclerosing cholangitis（PSC）. The incidence of the following complications was studied： obstructive cholangitis, portal hypertension, advanced liver disease and death associated with the primary disease.RESULTS： Fourteen（56%） patients had PSC and 11（44%） had ASC. Patients with ASC were significantly younger at the time of diagnosis（12.3 vs 15.4 years, P=0.032） and had higher Ig G levels（22.7 vs 17.2 g/L, P=0.003）. The mentioned complications occurred in 4（16%） patients with SC, exclusively in the PSC group： one patient died from colorectal cancer, one patient underwent liver transplantation and two patients, in whom severe bile duct stenosis was present at diagnosis, were endoscopically treated for acute cholangitis. Furthermore, twoother children with ASC and 2 children with PSC had elevated aminotransferase levels. The 10-year overall survival was 95.8% in all patients, 100% in patients without complicated liver disease, and 75.0% in patients with complications.CONCLUSION： In children, ASC is a frequent type of SC, whose prognosis may be better than that in patients with PSC.

关键词： autoimmune sclerosing cholangitis childhood inflammatory bowel disease primary sclerosing cholangitis prognosis

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Different clinical presentations of metachronous pulmonary metastases after resection of pancreatic ductal adenocarcinoma: Retrospective study and review of the literature

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World Journal of Gastroenterology 2017年第35期23卷 6420-6428页

作者： Martin Lovecek Pavel Skalicky Josef Chudacek Marek Szkorupa Hana Svebisova Radmila Lemstrova Jiri Ehrmann Bohuslav Melichar Tharani Yogeswara Dusan Klos Radek Vrba Roman Havlik Beatrice Mohelnikova-Duchonova Department of Surgery I University Hospital Olomouc and Faculty of Medicine and DentistryPalacky University Department of Oncology University Hospital Olomouc and Faculty of Medicine and DentistryPalacky University Department of Molecular Pathology Faculty of Medicine and DentistryPalacky University OlomoucUniversity Hospital Olomouc Institute of Molecular and Translational Medicine Faculty of Medicine and DentistryPalacky University Department of Oncology Faculty of Medicine and DentistryPalacky University OlomoucUniversity Hospital Olomouc Department of Surgery I Faculty of Medicine and DentistryPalacky University

AIM To analyze pancreatic cancer patients who developed metachronous pulmonary metastases(MPM) as a first site of recurrence after the curative-intent surgery.METHODS One-hundred-fifty-nine consecutive pancreatic ductal adenocarcinoma(PDAC) patients who underwent radical pancreatic surgery between 2006 and 2013 were included in this retrospective analysis. The clinical data including age, sex, grade, primary tumor location, p TNM stage, lymph node infiltration, microangioinvasion, perineural invasion, lymphovascular invasion, the therapy administered, and follow-up were all obtained from medical records. Further analysis covered only patients with metachronous metastases. Clinical and histopathological data(age, sex, grade, primary tumor location, p TNM stage, lymph node infiltration, microangioinvasion, perineural invasion, lymphovascular invasion, the therapy administered and follow-up) of patients with metachronous non-pulmonary metastases and patients with metachronous pulmonary metastases were statistically assessed. Disease-free survival(DFS) from pancreas resection until metastases onset and overall survival(OS) were calculated. Wilcoxon test, χ~2 test and survival functions computed by the KaplanMeier method were used. Statistical significance was evaluated by the log-rank test using SPSS. A P-value of less than 0.05 was considered statistically significant.RESULTS Metachronous pulmonary metastases were observed in 20(16.9%) and were operable in 3(2.5%) of PDAC patients after a prior curative-intent surgery. Patients with isolated pulmonary metastases(oligometastases and multiple metastases) had estimated prior DFS and OS of 35.4 and 81.4 mo, respectively, and those with metachronous pulmonary metastases accompanied by other metastases had prior DFS and OS of 17.3 and 23.4 mo, respectively. Patients with non-pulmonary metastases had prior DFS and OS of 9.4 and 15.8 mo, respectively. Different clinical scenarios according to the presentation of MPM were observed and patients could be divided to three subgr

关键词： Pancreatic cancer Pulmonary metastases Metachronous metastases Surgical resection Overall survival Chemotherapy

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