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Autoimmune hepatitis in children

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https://www.readbyqxmd.com/read/29115027/clinical-features-of-pediatric-autoimmune-hepatitis-in-japan-a-nationwide-survey
#1
Tsuyoshi Sogo, Atsushi Takahashi, Ayano Inui, Tomoo Fujisawa, Hiromasa Ohira, Hajime Takikawa
AIM: The purpose of this study was to determine the characteristics of children with autoimmune hepatitis (AIH) in Japan. METHODS: Questionnaires, which asked about patients newly diagnosed with AIH from 2009 to 2013 were sent to hospitals certified as training facilities for pediatrics in January 2015. RESULTS: A total of 35 patients were enrolled. The median age at diagnosis was 10 years (ranging from 3 months to 15 years), and the male-to-female ratio was 2:3...
November 8, 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/29112085/diagnosis-and-management-of-paediatric-autoimmune-liver-disease-espghan-hepatology-committee-position-statement
#2
Giorgina Mieli-Vergani, Diego Vergani, Ulrich Baumann, Piotr Czubkowski, Dominique Debray, Antal Dezsofi, Björn Fischler, Girish Gupte, Loreto Hierro, Giuseppe Indolfi, Jörg Jahnel, Françoise Smets, Henkjan J Verkade, Nedim Hadzic
Paediatric autoimmune liver disease is characterised by inflammatory liver histology, circulating autoantibodies and increased levels of IgG, in the absence of a known etiology. Three conditions have a likely autoimmune pathogenesis: autoimmune hepatitis (AIH), autoimmune sclerosing cholangitis (ASC), and de novo AIH after liver transplantation. Two types of paediatric AIH are recognized according to seropositivity for smooth muscle and/or antinuclear antibody (SMA/ANA, AIH-1) or liver kidney microsomal type 1 and/or anti-liver cytosol type 1 antibodies (anti-LKM-1/anti-LC-1; AIH-2)...
November 3, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29056396/serology-in-autoimmune-hepatitis-a-clinical-practice-approach
#3
REVIEW
Benedetta Terziroli Beretta-Piccoli, Giorgina Mieli-Vergani, Diego Vergani
Serology is key to the diagnosis of autoimmune hepatitis (AIH). Clinicians need to be aware of which tests to request, how to interpret the laboratory reports, and be familiar with the laboratory methodology. If correctly tested, >95% of AIH patients show some serological reactivity. Indirect immunofluorescence on triple rodent tissue is recommended as first screening step, since it allows the detection of all liver-relevant autoantibodies, except for anti-soluble liver antigen (SLA) antibody, which needs to be detected by molecular based assays...
October 19, 2017: European Journal of Internal Medicine
https://www.readbyqxmd.com/read/29045346/autoimmune-hepatitis-and-aih-overlap-with-sclerosing-cholangitis-immunophenotype-markers-in-children-and-adolescents
#4
Priscila Menezes Ferri, Ana Cristina Simões E Silva, Karen Cecília de Lima Torres, Soraya Luiza Campos Silva, Diego Júnior Queiroga de Aquino, Maria Luísa Marques Ferreira, Eleonora Druve Tavares Fagundes, Débora Marques de Miranda, Alexandre Rodrigues Ferreira
OBJECTIVE: The pathophysiology of autoimmune hepatitis (AIH) may involve the activation of immune cells and changes in the expression of cellular markers. The aim of this study was to characterize the immunophenotype markers of lymphocytes and monocytes in the peripheral blood of children and adolescents with type 1 AIH and AIH overlap with sclerosing cholangitis (overlap syndrome, OS). METHODS: This is a cross-sectional study of 20 children and adolescents diagnosed with type 1 AIH and 19 with OS...
October 17, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29019854/the-best-choice-for-second-line-treatment-in-standard-treatment-refractory-children-with-autoimmune-hepatitis
#5
Silvia Nastasio, Marco Sciveres, Giuseppe Maggiore
No abstract text is available yet for this article.
October 10, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28993983/skin-manifestations-associated-with-autoimmune-liver-diseases-a-systematic-review
#6
REVIEW
Benedetta Terziroli Beretta-Piccoli, Pietro Invernizzi, M Eric Gershwin, Carlo Mainetti
Autoimmune liver diseases, which include mainly autoimmune hepatitis, primary biliary cholangitis, primary sclerosing cholangitis, and the variant syndromes, are often associated with extrahepatic autoimmune diseases. However, the association with cutaneous diseases is less well described. In the present article, we provide a systematic literature review on skin manifestations linked to each of these four autoimmune liver diseases, excluding skin manifestations of systemic diseases. The association of autoimmune hepatitis with vitiligo is well known, with a particular striking association with type 2 autoimmune hepatitis, a condition occurring almost entirely in children and adolescents, much rarer and more aggressive than type 1 autoimmune hepatitis; probable associations are also identified with alopecia areata, psoriasis, and pyoderma gangrenosum...
October 9, 2017: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/28977835/association-of-stat4-and-ptpn22-polymorphisms-and-their-interactions-with-type-1-autoimmune-hepatitis-susceptibility-in-chinese-han-children
#7
Xiaofeng Li, Huiqin Chen, Yun Cai, Pingping Zhang, Zhuanggui Chen
AIMS: To investigate the impact of signal transducer and activator of transcription 4 (STAT4) and the protein tyrosine phosphatase N22 (PTPN22) gene single nucleotide polymorphisms (SNPs), gene-gene interactions and haplotype on type-1 Autoimmune Hepatitis (AIH) risk. RESULTS: Logistic regression analysis showed that type 1 AIH was significantly higher in carriers of T allele of rs7574865 than those with GG genotype (P- value less than 0.001), higher in carriers of C allele of rs7582694 than those with GG genotype (P- value < 0...
September 22, 2017: Oncotarget
https://www.readbyqxmd.com/read/28970719/autoimmune-hepatitis-standard-treatment-and-systematic-review-of-alternative-treatments
#8
REVIEW
Benedetta Terziroli Beretta-Piccoli, Giorgina Mieli-Vergani, Diego Vergani
Autoimmune hepatitis is a rare chronic inflammatory liver disease, affecting all ages, characterised by elevated transaminase and immunoglobulin G levels, positive autoantibodies, interface hepatitis at liver histology and good response to immunosuppressive treatment. If untreated, it has a poor prognosis. The aim of this review is to summarize the evidence for standard treatment and to provide a systematic review on alternative treatments for adults and children. Standard treatment is based on steroids and azathioprine, and leads to disease remission in 80%-90% of patients...
September 7, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28953529/anti-tnf%C3%AE-treatment-in-children-and-adolescents-with-combined-inflammatory-bowel-disease-and-autoimmune-liver-disease
#9
Natalia Nedelkopoulou, Babu Vadamalayan, Diego Vergani, Giorgina Mieli-Vergani
OBJECTIVES: Inflammatory bowel disease (IBD) and autoimmune liver disease (AILD) are closely associated, the former often dictating progression of the latter. Antibodies to tumor necrosis factor alpha (anti- TNFα) are effective in the management of IBD, but may cause liver injury. METHODS: Retrospective review of medical records of patients with juvenile AILD who received anti-TNFα for IBD to evaluate the safety and efficacy of anti-TNFα. RESULTS: Eleven patients (6 males), aged 9-15y (median 13y) were identified...
September 26, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28953189/transient-hypothyroidism-and-autoimmune-thyroiditis-in-children-with-chronic-hepatitis-c-treated-with-pegylated-interferon-%C3%AE-2b-and-ribavirin
#10
Daniele Serranti, Giuseppe Indolfi, Gabriella Nebbia, Mara Cananzi, Lorenzo D'Antiga, Silvia Ricci, Stefano Stagi, Chiara Azzari, Massimo Resti
BACKGROUND: Autoimmune thyroid disease and thyroid dysfunction are common in adults receiving interferon-based treatment for chronic hepatitis C (CHC). Few data are available in children with CHC. This study is aimed to evaluate the appearance and timing of thyroid dysfunction and anti-thyroid autoimmunity in children with CHC treated with pegylated interferon-α-2b and ribavirin. METHODS: 61 otherwise healthy children with CHC, 3-17 years of age, infected perinatally and treatment naïve, receiving therapy with pegylated interferon-α-2b and ribavirin and 183 age- and sex-matched controls were included in a multicenter, prospective, case-control study...
September 22, 2017: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28914695/autoimmune-acute-liver-failure-and-seronegative-autoimmune-liver-disease-in-children-are-they-different-from-classical-disease
#11
Vikas Jain, Anshu Srivastava, Surender K Yachha, Niraj Kumari, Rachana Kathuria, Moinak S Sarma, Ujjal Poddar, Narendra Krishnani
OBJECTIVES: Presentation as autoimmune acute liver failure (AI-ALF) and seronegative autoimmune liver disease (SN-AILD) represents two uncommon variants of AILD. We compared the clinical profile and outcome of AI-ALF with autoimmune-non-acute liver failure (AI-non-ALF) and also SN-AILD with seropositive autoimmune liver disease (SP-AILD). MATERIALS AND METHODS: Children managed as AI-ALF and AI-non-ALF including SN-AILD and SP-AILD were enrolled and compared. AI-non-ALF was diagnosed by simplified diagnostic criteria and AI-ALF by Pediatric Acute Liver Failure Study Group criteria with positive autoantibody, exclusion of other etiologies, elevated immunoglobulin G and histology when available...
September 13, 2017: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28878866/association-of-autoimmune-hepatitis-type-1-in-a-child-with-evans-syndrome
#12
Chaowapong Jarasvaraparn, Hamayun Imran, Abdul Siddiqui, Felicia Wilson, David A Gremse
Autoimmune hepatitis (AIH) is a progressive liver disease that is often associated with extrahepatic autoimmune disorders. Evans syndrome (ES) is a rare autoimmune disorder, which is characterized by immune thrombocytopenia and autoimmune hemolytic anemia. Association of AIH with ES is rare, especially in children. We report a 3-year-old female with a past medical history of ES who presented with jaundice and significant transaminitis due to AIH type 1. She required multiple treatments with steroids as well as azathioprine, intravenous immunoglobulin and a course of rituximab...
August 18, 2017: World Journal of Hepatology
https://www.readbyqxmd.com/read/28828056/autoimmune-hepatitis-with-autoimmune-haemolytic-anemia-triggered-by-varicella-a-rare-presentation
#13
Deepak Jain, Harpreet Singh, Gaganpreet Singh, Promil Jain
Varicella is a common infectious exanthematous disease of children. Infection manifests as polymorphic maculopapulovesicular along with low grade fever, malaise and headache. Adults are less likely to be infected with varicella infection but once infected suffer disproportionately from serious complications like pneumonia, encephalitis, hepatitis and thrombocytopenia. Varicella hepatitis is generally a self-limiting disease with only a temporary subclinical rise in hepatic enzymes and are usually encountered however autoimmune hepatitis (AIH) which is an autoimmune disorder of unknown etiology affecting the liver is a very rare association of Varicella infection ...
December 2016: Mædica
https://www.readbyqxmd.com/read/28781316/histological-changes-in-autoimmune-hepatitis-with-graves-disease-a-child-case-report
#14
Mamiko Yamada, Hironori Shibata, Yohei Masugi, Tomohiro Ishi, Kaori Kameyama, Hirotoshi Ebinuma, Tomonobu Hasegawa
We herein report a child case of autoimmune hepatitis (AIH) accompanied with Graves' disease. Elevated aminotransferase levels were found in a 12-year-old Japanese girl with Graves' disease. In her first liver biopsy, necrosis and inflammation was limited to the centrilobular area, while the second biopsy showed different findings. Namely, portal injury newly appeared, including interface hepatitis, which represents the histological characteristics of AIH. As the histological findings at the onset of AIH do not always show typical findings, a re-biopsy is considered to be important in individuals suspected to have AIH...
August 15, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28735981/autoimmune-liver-disease-in-children-with-sickle-cell-disease
#15
Suttiruk Jitraruch, Emer Fitzpatrick, Maesha Deheragoda, Annamaria Deganello, Giorgina Mieli-Vergani, Susan Height, David Rees, Nedim Hadzic, Marianne Samyn
OBJECTIVE: To assess the incidence, clinical features, and outcome of autoimmune liver disease (AILD) in patients with sickle cell disease (SCD). STUDY DESIGN: Single center retrospective review of patients with SCD with AILD referred between 1999 and 2015. RESULTS: Thirteen of 77 (17%) patients with SCD with hepatic dysfunction were diagnosed with AILD (median age 11, range, 3.4-16 years) with a female preponderance (77%). Acute hepatitis and insidious onset were the commonest presentations...
October 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28673589/hepatitis-b-vaccine-non-response-a-predictor-of-latent-autoimmunity
#16
M D Raffaella Mormile
Unresponsiveness to Hepatitis B virus (HBV) vaccine has been associated with interleukins involved with Th1 functioning including Interleukin-8 (IL-18) and Interferon-γ (IFN-γ). IL-18 and IFN-γ have also been implicated in the onset of different types of immune-mediate inflammatory conditions such as Type 1 Diabetes (T1D), Celiac disease (CD), rheumatoid arthritis (RA), obesity and systemic lupus erythematosus (SLE). Taking into account that HBV vaccination is provided in the 1st year of life worldwide, I propose that all babies should be tested for anti-HBs response after completion of the vaccine series...
July 2017: Medical Hypotheses
https://www.readbyqxmd.com/read/28663066/successful-management-of-acute-liver-failure-in-italian-children-a-16-year-experience-at-a-referral-centre-for-paediatric-liver-transplantation
#17
A Di Giorgio, A Sonzogni, A Piccichè, G Alessio, E Bonanomi, M Colledan, L D'Antiga
BACKGROUND: Identifying the causes of acute liver failure (ALF) and predictors of death or liver transplantation (LTX) is crucial to decide its management. We aimed to describe features and outcome of ALF in Italian children. METHODS: Retrospective review of cases presenting between 1996-2012. ALF was defined by high transaminases, INR ≥2.0 regardless of hepatic encephalopathy (HE), no evidence of underlying chronic liver disease. RESULTS: 55 children (median age 2...
June 12, 2017: Digestive and Liver Disease
https://www.readbyqxmd.com/read/28644343/second-line-agents-in-pediatric-patients-with-autoimmune-hepatitis-a-systematic-review-and-meta-analysis
#18
Andréanne N Zizzo, Pamela L Valentino, Prakesh S Shah, Binita M Kamath
BACKGROUND AND AIMS: Ten percent to 20% of children with autoimmune hepatitis (AIH) require second-line therapy to achieve remission. Although current guidelines exist on first-line management, evidence for second-line therapy in treatment-refractory patients is lacking. Our aim was to perform a systematic review and meta-analysis of the efficacy and safety of second-line treatments used in this population. METHODS: Electronic and manual searches were used to identify potential studies for inclusion...
July 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28603203/neonatal-lupus-erythematosus
#19
REVIEW
Naoto Yokogawa, Naofumi Sumitomo, Masaru Miura, Kazuhiko Shibuya, Hiroshi Nagai, Mikako Goto, Atsuko Murashima
Neonatal lupus (NL), a passively-acquired autoimmune disease associated with maternal anti-SSA antibody, presents both cardiac manifestations such as cardiac NL and non-cardiac manifestations including rashes, cytopenia, and hepatic abnormalities. Cardiac NL, occurring in 1-2% of anti-SS-A antibody-positive mothers, is a life-threatening complication with a mortality rate of 20% and a pacemaker implantation rate of 70%. In contrast, cutaneous NL, which is more common than cardiac NL, usually resolves in six months...
2017: Nihon Rinshō Men'eki Gakkai Kaishi, Japanese Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28595203/parameters-of-ventricular-repolarization-in-patients-with-autoimmune-hepatitis
#20
Burak Açar, Mahmut Yüksel, Çağrı Yayla, Özgür Kırbaş, Sefa Ünal, Ahmet Göktuğ Ertem, Koray Demirtaş, Mustafa Kaplan, Meral Akdoğan, Sabite Kaçar, Ertuğrul Kayaçetin, Sinan Aydoğdu
OBJECTIVE: Autoimmune hepatitis (AIH) is a liver disorder that affects both children and adults. It is characterized by inflammatory liver histology, elevated transaminase level, circulating nonorgan-specific autoantibodies, and increased level of immunoglobulin G in the absence of known etiology. Ventricular repolarization has been evaluated using T wave and QT interval measurements in patients with hepatic cirrhosis. Ventricular repolarization may be defined using QT interval, QT dispersion, and T wave measurements...
June 2017: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
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