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Autoimmune hepatitis in children

E Ylinen, L Salmela, J Peräsaari, T Jaatinen, A Tenca, O Vapalahti, M Färkkilä, H Jalanko, K-L Kolho
AIM: The human leukocyte antigen (HLA) allele and haplotype frequencies of the Finnish population are unique because of the restricted and homogenous gene population. There are no published data on HLA genotype associations in paediatric autoimmune liver diseases in Scandinavia. This study characterised the HLA genotypes of children with autoimmune liver or biliary disease in Finland. METHODS: The study cohort comprised 19 paediatric patients (13 female) aged 3-15 years treated for autoimmune liver or biliary disease at the Children's Hospital, Helsinki University Hospital, between 2000 and 2011 and followed up for 4...
October 19, 2016: Acta Paediatrica
Jacqueline Jossen, Rachel Annunziato, Hee-Sung Kim, Jaime Chu, Ronen Arnon
OBJECTIVES: Autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) are progressive immune-mediated inflammatory diseases that may require liver transplant (LT). Outcomes in children undergoing LT for these diseases are poorly studied in the Pediatric End Stage Liver Disease (PELD) era. We aimed to characterize the outcome of LT in children with AIH and PSC. METHODS: Children ≤18 years with PSC or AIH who had a first, isolated LT from 2002-2012 were identified from the UNOS database...
October 13, 2016: Journal of Pediatric Gastroenterology and Nutrition
Mirinae Jang, Min Su Oh, Se Cheol Oh, Ki Soo Kang
Although liver function test abnormality is frequently noted in children, there is no report about the distribution of the etiology and natural recovery time of the abnormal liver function. From March 2005 to February 2014, clinical information was retrospectively collected from 559 children who had abnormal liver function and were hospitalized or visited the outpatient clinic at the Jeju National University Hospital. The etiology of abnormal liver function was classified into groups and the natural recovery time of abnormal liver function was analyzed...
November 2016: Journal of Korean Medical Science
Gamal Hasan, Asaad Assiri, Naglaa Marzuuk, Enas Daef, Sayed Abdelwahab, Ahmed Ahmed, Ismail Mohamad, Ayman Al-Eyadhy, Ali Alhaboob, Mohamad-Hani Temsah
OBJECTIVE: To describe the characteristics of hepatitis E virus (HEV) infection in a cohort of children from Upper Egypt using data from a large multicentre prospective study of acute viral hepatitis (AVH). METHODS: Data from subjects aged 2-18 years with AVH or close contacts of those with AVH found to have asymptomatic AVH were included in the analysis. Information concerning medical history, clinical examination, liver function tests and screening for hepatotropic viruses was recorded and analysed...
September 29, 2016: Journal of International Medical Research
Airi Tiirats, Triin Viltrop, Margit Nõukas, Ene Reimann, Andres Salumets, Sulev Kõks
BACKGROUND: Despite extensive research the genetic component of extremely low birth weight (ELBW) in newborns has remained obscure. RESULTS: The aim of the case study was to identify candidate gene(s) causing ELBW in newborns and hypotrophy in infants. A family of four was studied: mother, father and two ELBW-phenotype children. Studies were made of the medical conditions of the second child at birth and post-partum - peculiar phenotype, micro-anomalies, recurrent infections, suspicion of autoimmune hepatitis, multifactorial encephalopathy and suspected metabolic and chromosomal abnormalities...
2016: BMC Genetics
Haider Al Taii, Mohamad A Hanouneh, Ibrahim Hanouneh, Rocio Lopez, Nizar Zein, Naim Alkhouri
No abstract text is available yet for this article.
September 15, 2016: Scandinavian Journal of Gastroenterology
Aileen Raizner, Nick Shillingford, Paul D Mitchell, Sarah Harney, Roshan Raza, Jessica Serino, Maureen M Jonas, Christine K Lee
OBJECTIVES: Transient elastography (TE) measures liver stiffness to assess fibrosis. Studies in adults have shown that inflammation increases stiffness, leading to an overestimation of fibrosis. We investigated the contribution of inflammation to liver stiffness measurements (LSM) in children/ young adults. METHODS: This was a cohort analysis of children/ young adults who underwent TE within 1 year of liver biopsy. ALT was obtained within 30d of the biopsy and LSM...
August 18, 2016: Journal of Pediatric Gastroenterology and Nutrition
Vratislav Smolka, Eva Karaskova, Oksana Tkachyk, Kvetoslava Aiglova, Jiri Ehrmann, Kamila Michalkova, Michal Konecny, Jana Volejnikova
BACKGROUND: Sclerosing cholangitis (SC) is a chronic cholestatic hepatobiliary disease with uncertain long-term prognosis in pediatric patients. This study aimed to evaluate long-term 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)...
August 2016: Hepatobiliary & Pancreatic Diseases International: HBPD INT
Júlio Rocha Pimenta, Alexandre Rodrigues Ferreira, Eleonora Druve Tavares Fagundes, Thaís Costa Nascentes Queiroz, Regiane Aparecida Nascimento Baptista, Eduardo Guimarães de Araújo Moreira, Camilo Brandão de Resende, Paulo Fernando Souto Bittencourt, Simone Diniz Carvalho, José Andrade Franco Neto, Francisco José Penna
OBJECTIVE: Bleeding of esophageal varices is the main cause of morbidity and mortality in children with portal hypertension. It is important to understand the factors related with a bleeding episode to evaluate more effective primary prophylaxis. This study aims to describe the endoscopic and laboratory findings associated with upper gastrointestinal bleeding (UGIB) secondary to esophageal varices. METHOD: A cross-sectional study with 103 children and adolescents with cirrhosis, divided into a group that had experienced an episode of upper UGIB (35 patients) and a group without a history of UGIB (68 patients), was carried out...
August 4, 2016: Journal of Pediatric Gastroenterology and Nutrition
Michael R Narkewicz, Simon Horslen, Steven H Belle, David A Rudnick, Vicky L Ng, Philip Rosenthal, Rene Romero, Kathleen M Loomes, Song Zhang, Regina M Hardison, Robert H Squires
OBJECTIVES: The purpose of this study is to estimate autoantibody (autoAB) frequency, clinical characteristics and 21 day outcome of participants in the Pediatric Acute Liver Failure Study Group (PALFSG) by ANA, SMA and LKM antibody status. METHODS: AutoAB were determined at local and/or central laboratories. Subjects were assigned to autoimmune hepatitis (AIH), indeterminate and other diagnoses groups. RESULTS: Between 1999 and 2010, 986 subjects were enrolled in the PALFSG...
August 4, 2016: Journal of Pediatric Gastroenterology and Nutrition
Behairy E Behairy, Hanaa A El-Araby, Hasan H Abd El Kader, Nermin A Ehsan, Menan E Salem, Haidy M Zakaria, Mohammed A Khedr
UNLABELLED:  Background. T-cell populations regulate the balance of immune responses. The CD (Cluster of differentiation) 4+CD25+ regulatory T cells (Tregs) are crucial for maintaining negative control of various immune responses. There are different T-cell subpopulations with regulatory functions, as natural killer T cells, CD8+ and CD28. The forkhead box P3 (FOXP3) regulates Treg development and is required for its suppressive function. AIM: To evaluate the hepatic expression of the intrahepatic Tregs, Ig (immunoglobulin) G and IgM plasma cells in autoimmune hepatitis (AIH) and other chronic liver diseases (CLDs)...
September 2016: Annals of Hepatology
Line K Rasmussen, Elisabeth V Stenbøg, Gitte B Kerndrup, Henrik Hasle
An association between hepatitis and aplastic anemia (AA) is known as hepatitis-associated AA, and is characterized by an acute attack of hepatitis followed by the development of AA. We report 2 clinical cases of acute seronegative hepatitis in which pancytopenia with mild dysplasia developed after 3 months; however, neither of our cases fulfilled the histological criteria of AA, but rather myelodysplastic syndrome. This novel association bears considerable resemblance to hepatitis-associated AA, and raises the question of whether hepatitis-associated dysmyelopoiesis should be included in the spectrum of hypocellular myelodysplastic syndrome...
November 2016: Journal of Pediatric Hematology/oncology
S Varma, J Ambroise, M Komuta, D Latinne, P Baldin, R Reding, F Smets, X Stephenne, E M Sokal
AIM: To determine predisposing factors of idiopathic allograft fibrosis among pediatric liver transplant recipients. BACKGROUND: Protocol biopsies (PB) from stable liver transplant (LT) recipient children frequently exhibit idiopathic fibrosis. The relation between allograft inflammation, humoral immune response and fibrosis is uncertain. Also the role of HLA-DRB1 genotype has not been evaluated, though it's associated with fibrosis in autoimmune hepatitis. PATIENTS AND METHODS: This observational study, included 89 stable LT recipient transplanted between 2004-2012 with mean follow-up of 4...
July 2016: EBioMedicine
(no author information available yet)
No abstract text is available yet for this article.
June 2016: Annals of Intensive Care
Rodrigo Liberal, Diego Vergani, Giorgina Mieli-Vergani
Approximately 10% of children with autoimmune hepatitis (AIH) and 30% of those with sclerosing cholangitis (SC) require liver transplantation (LT). LT is indicated in patients who present with fulminant hepatic failure (ie, with encephalopathy) and in those who develop end-stage liver disease despite treatment. After LT, recurrent AIH is reported in approximately 30% of patients and recurrent SC in up to 50%. Diagnosis of recurrence is based on biochemical abnormalities, seropositivity for autoantibodies, interface hepatitis on histology, steroid dependence, and, for SC, presence of cholangiopathy...
September 2016: Liver Transplantation
Z H Deng, L R Jiang, B Zhang, Y Z Xu, C H Shen, T Zhou, Q Xia, T A Zhang
OBJECTIVE: To explore the pathogenesis, treatment and prognosis of primary hypogammaglobulinemia complicated with liver cirrhosis in a child. METHOD: Pathogenesis, treatment and prognosis of X-linked agammaglobulinemia (XLA ) complicated with liver cirrhosis in a child were analyzed in Shanghai Children's Medical Center.Using"primary hypogammaglobulinemia"and"liver cirrhosis"as keywords, literatures were searched from Pubmed and Chinese data of Weipu and Wanfang data from January 1988 to January 2015...
May 2016: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Behairy El-Sayed Behairy, Mostafa Mohamed Sira, Khaled Refat Zalata, El-Sayed Ebrahem Salama, Mohamed Ahmed Abd-Allah
AIM: To evaluate transient elastography (TE) as a noninvasive tool in staging liver fibrosis compared with liver biopsy and morphometry in children with different chronic liver diseases. METHODS: A total of 90 children [50 with chronic hepatitis C virus (HCV), 20 with autoimmune hepatitis (AIH) and 20 with Wilson disease] were included in the study and underwent liver stiffness measurement (LSM) using TE. Liver biopsies were evaluated for fibrosis, qualitatively, by Ishak score and quantitatively by fibrosis area fraction (FAF) using digital image analysis (morphometry)...
April 28, 2016: World Journal of Gastroenterology: WJG
Anna Pniewska, Małgorzata Sobolewska-Pilarczyk, Małgorzata Pawłowska
INTRODUCTION: Autoimmune hepatitis is rarely diagnosed in children, but the course of the disease is often aggresive. Combination therapy with prednisone and azathioprine improves the prognosis of patients. AIM: To evaluate the effectiveness of combination therapy with prednisone and azathioprine of autoimmune hepatitis (AIH) in children. MATERIAL AND METHODS: There was a retrospective analysis of the medical records of 15 patients with AIH, diagnosed before18 years of age, treated in the Provincial Infectious Diseases Hospital in Bydgoszcz in the years 2002 to 2013...
2016: Przegla̜d Gastroenterologiczny
Giuseppe Maggiore, Gérard Socie, Marco Sciveres, Anne-Marie Roque-Afonso, Silvia Nastasio, Catherine Johanet, Fréderic Gottrand, Sébastien Fournier-Favre, Emmanuel Jacquemin, Olivier Bernard
BACKGROUND: A few children with acute or chronic liver disease display histological features compatible with autoimmune hepatitis, but lack specific serological markers. AIM: To describe features, management and outcome of childhood seronegative autoimmune hepatitis. METHODS: From 1988 to 2010, 38 children were included under the following criteria: negative virological studies, no serum autoantibodies, exclusion of other causes of liver diseases, and liver histology compatible with autoimmune hepatitis...
July 2016: Digestive and Liver Disease
Shigeto Nagao, Takayuki Kondo, Takashi Nakamura, Tomokazu Nakagawa, Sadayuki Matsumoto
We report a case of human immunodeficiency virus (HIV) infection that showed subacute progressive cerebellar ataxia without HIV encephalopathy or other encephalopathies, including progressive multifocal leukoencephalopathy or encephalitis of other human herpes virus (HHV) infections. A 43-year-old man exhibited unsteady gait. Neurological examination disclosed ataxia of the trunk and lower extremities. Personality change and dementia were absent. Magnetic resonance imaging did not reveal any abnormal finding, including of the cerebellum...
April 28, 2016: Rinshō Shinkeigaku, Clinical Neurology
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