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Autoimmune hepatitis in children

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https://www.readbyqxmd.com/read/29766815/old-and-new-treatments-for-pediatric-autoimmune-hepatitis
#1
Silvia Nastasio, Giuseppe Maggiore, Marco Sciveres, Lorenza Matarazzo
Autoimmune hepatitis is a rare inflammatory disease of the liver that most frequently affects children and young adults. It is a multifactorial disease of unknown etiology, characteristically progressive in nature, and if left untreated, may lead to cirrhosis and terminal liver failure. It has been known for several decades now that immunosuppressive treatment convincingly alters the outcome of most patients with autoimmune hepatitis and as such it should be started as soon as diagnosis is made. Primary goals of treatment are: normalization of hepatocellular function, extinction of the hepatic necroinflammatory process, and maintenance of a stable remission, thus preventing progression to cirrhosis and its complications...
May 16, 2018: Current Pediatric Reviews
https://www.readbyqxmd.com/read/29735782/an-overview-of-liver-transplant-pathology-data-from-a-tertiary-referral-centre-in-western-india
#2
Fiona Fonseca, Bijal Kulkarni, Meenal Hastak, Vinay Kumaran, Vibha Varma, Sorabh Kapoor
INTRODUCTION AND AIM: 1. Study of liver explants - Etiologic types of end-stage chronic liver disease (ESCLD) and acute liver failure (ALF) in adults and children. 2. Assessment of donor steatosis and incidental granulomas. 3. Post-transplant liver biopsies. MATERIAL AND METHODS: Specimens of 180 explant hepatectomies, 173 donor wedge and 30 core liver biopsies, and 58 post transplant liver biopsies received in our department from April 2013 to March 2017. RESULTS: 1...
April 9, 2018: Annals of Hepatology
https://www.readbyqxmd.com/read/29713609/accuracy-of-the-2008-simplified-criteria-for-the-diagnosis-of-autoimmune-hepatitis-in-children
#3
José Vicente Arcos-Machancoses, Cristina Molera Busoms, Ecaterina Julio Tatis, María Victoria Bovo, Jesús Quintero Bernabeu, Javier Juampérez Goñi, Vanessa Crujeiras Martínez, Javier Martin de Carpi
Purpose: Classical criteria for diagnosis of autoimmune hepatitis (AIH) are intended as research tool and are difficult to apply at patient's bedside. We aimed to study the accuracy of simplified criteria and the concordance with the expert diagnosis based on the original criteria. Methods: A cohort of children under study for liver disorder was selected through consecutive sampling to obtain the prevalence of AIH within the group of differential diagnoses. AIH was defined, based on classical criteria, through committee review of medical reports...
April 2018: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/29680189/vitamin-d-non-sufficiency-is-prevalent-in-children-with-chronic-liver-disease-in-a-tropical-country
#4
Way Seah Lee, Muhammad Yazid Jalaludin, Shin Yee Wong, Sik Yong Ong, Hee Wei Foo, Ruey Terng Ng
BACKGROUND: To determine vitamin D status in children with chronic liver disease (CLD) in a tropical country. METHODS: Cross-sectional study in Malaysian children with CLD. Factors affecting serum vitamin D level (definition: deficient < 30 nmol/L; insufficient 30-50 nmol/L; sufficient ≥ 50 nmol/L) was analyzed. RESULTS: Of the 59 children studied (males 32, 54%; median age 6.8 ± 5.3 years), the three most common causes were biliary atresia (n = 25), autoimmune hepatitis (n = 16) and sclerosing cholangitis (n = 6)...
April 5, 2018: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/29644994/autoimmune-hepatitis
#5
REVIEW
Giorgina Mieli-Vergani, Diego Vergani, Albert J Czaja, Michael P Manns, Edward L Krawitt, John M Vierling, Ansgar W Lohse, Aldo J Montano-Loza
Autoimmune hepatitis (AIH) is a severe liver disease that affects children and adults worldwide. The diagnosis of AIH relies on increased serum transaminase and immunoglobulin G levels, presence of autoantibodies and interface hepatitis on liver histology. AIH arises in genetically predisposed individuals when a trigger, such as exposure to a virus, leads to a T cell-mediated autoimmune response directed against liver autoantigens; this immune response is permitted by inadequate regulatory immune control leading to a loss of tolerance...
April 12, 2018: Nature Reviews. Disease Primers
https://www.readbyqxmd.com/read/29470290/autoimmune-hepatitis-in-children-and-adolescents-effect-on-quality-of-life
#6
Isadora de Carvalho Trevizoli, Carolina Silva Pinedo, Vanessa Oliveira Teles, Renata Belém Pessoa de Melo Seixas, Elisa de Carvalho
OBJECTIVES: The aim of the to evaluate the effect of autoimmune hepatitis (AIH) on the quality of life of children and adolescents and to identify which variables effect health-related quality of life (HRQoL). METHODS: The Pediatric Quality of Life Inventory 4.0 (PedsQL 4.0) was used to evaluate HRQoL. In addition, a questionnaire was applied which included signs, symptoms, and use of medications. RESULTS: A total of 43 patients (mean age, 15...
February 21, 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29470284/evolving-practice-and-changing-phenotype-in-pediatric-autoimmune-liver-disease-outcomes-from-an-australian-center
#7
Harveen Singh, Fariha Balouch, Charlton Noble, Peter Lewindon
OBJECTIVES: Autoimmune liver disease (AILD) incorporates primary sclerosing cholangitis (PSC), autoimmune hepatitis (AIH), and autoimmune sclerosing cholangitis (ASC). ASC is a condition that includes overlap of AIH and PSC. We investigate changes in practice in relation to diagnosis and phenotype over 2 time periods. METHODS: Retrospective chart review was conducted from January 2000 to 2016. Data were divided into two 8-year cohorts, CI and C2. RESULTS: Data were collected in 75 children, 29 in 2000-2007 (C1) and 46 in 2008-2016 (C2)...
February 21, 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29464506/idiopathic-portal-hypertension-and-extrahepatic-portal-venous-obstruction
#8
REVIEW
Rajeev Khanna, Shiv Kumar Sarin
BACKGROUND: Idiopathic portal hypertension (IPH) and extrahepatic portal venous obstruction (EHPVO) are non-cirrhotic vascular causes of portal hypertension (PHT). Variceal bleed and splenomegaly are the commonest presentations. AIM: The present review is intended to provide the existing literature on etiopathogenesis, clinical profile, diagnosis, natural history and management of IPH and EHPVO. RESULTS: IPH and EHPVO are both characterized by normal hepatic venous pressure gradient, moderate to massive splenomegaly with preserved liver synthetic functions...
February 2018: Hepatology International
https://www.readbyqxmd.com/read/29446799/autoimmune-hepatitis-in-children
#9
Saumya Pathak, Deepak Kamat
Autoimmune hepatitis (AIH) is an immune-mediated, inflammatory liver disease. Clinical presentation of AIH in children is highly variable. It can present acutely, chronically, or silently. There are two main types of AIH-type 1 and type 2, which are differentiated and defined by the presence of specific autoantibodies. AIH eventually progresses to cirrhosis when left untreated, and occasionally even with treatment. AIH must be suspected and excluded in all children presenting with signs of acute, prolonged, or severe liver disease...
February 1, 2018: Pediatric Annals
https://www.readbyqxmd.com/read/29396050/association-of-interleukin-il-4-variable-number-of-tandem-repeats-vntrs-and-il-4-590-promoter-polymorphisms-with-susceptibility-to-pediatric-autoimmune-hepatitis-type-1
#10
Amira Ibrahim Mansour, Ola Galal Behairy, Eman Rateb Abd Almonaem, Rasha Mahmoud Abd-Rabuh, Inas Abd Elmonem Ahmed
OBJECTIVE: Autoimmune hepatitis (AIH) is a chronic inflammatory liver disease mediated by an autoimmune reaction to hepatocytes, the present study aimed to assess the possible associations between interleukin-4 (IL-4) variable number of tandem repeats (VNTRs) and IL-4-590 promoter polymorphisms and susceptibility to autoimmune hepatitis type 1 in children. SUBJECTS AND METHODS: The study was performed on 101 children diagnosed with AIH and 104 apparently healthy, age and sex-matched control children, diagnosis of AIH was based on the simplified score for the diagnosis of AIH...
January 26, 2018: Cytokine
https://www.readbyqxmd.com/read/29356770/diagnosis-and-management-of-pediatric-autoimmune-liver-disease-espghan-hepatology-committee-position-statement
#11
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 Hadžić
Paediatric autoimmune liver disease is characterized 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, and de novo AIH after liver transplantation. Two types of pediatric AIH are recognized according to seropositivity for smooth muscle and/or antinuclear antibody (AIH-1) or liver kidney microsomal type 1 and/or anti-liver cytosol type 1 antibodies (AIH-2)...
February 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29352427/preserved-in-vitro-immunoreactivity-in-children-receiving-long-term-immunosuppressive-therapy-due-to-inflammatory-bowel-disease-or-autoimmune-hepatitis
#12
Teresa Schleker, Eva-Maria Jacobsen, Benjamin Mayer, Gudrun Strauss, Klaus-Michael Debatin, Carsten Posovszky
BACKGROUND: Children with inflammatory bowel disease (IBD) or autoimmune hepatitis (AIH) are at risk for severe infections. This is partially a result of their chronic disease condition but, moreover, a side effect of their immunosuppressive therapy. Currently, vaccinations with live vaccines are regarded as contraindicated under immunosuppressive therapy, mainly because of concerns about side effects and a lack of data showing an adequate immune reaction. As there is no systematic study on the individual immunoreactivity under immunosuppressive therapy in this patient group, we analyzed the lymphocyte subgroups and immunoreactivity of lymphocytes in children with IBD or AIH with and without immunosuppressive therapy in vitro...
January 19, 2018: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/29343274/coexistent-sickle-cell-anemia-and-autoimmune-disease-in-eight-children-pitfalls-and-challenges
#13
Valerie Li-Thiao-Te, Florence Uettwiller, Pierre Quartier, Florence Lacaille, Brigitte Bader-Meunier, Valentine Brousse, Mariane de Montalembert
BACKGROUND: Patients with sickle cell disease (SCD) present a defective activation of the alternate complement pathway that increases the risk of infection and is thought to predispose to autoimmune disease (AID). However, coexisting AID and SCD is rarely reported, suggesting possible underdiagnosis due to an overlapping of the symptoms. STUDY DESIGN: Among 603 patients with SCD followed between 1999 and June 2016, we retrospectively searched for patients with coexisting SCD and AID...
January 17, 2018: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29331407/decreased-health-related-quality-of-life-in-children-and-adolescents-with-autoimmune-hepatitis
#14
Ana Beatriz Bozzini, Luciana Neder, Clovis A Silva, Gilda Porta
OBJECTIVE: The objective of this study was to evaluate the health-related quality of life in children and adolescents with autoimmune hepatitis. METHODS: A cross-sectional assessment with the Pediatric Quality of Life Inventory 4.0 (PedsQL 4.0) was completed for 80 patients with autoimmune hepatitis and 45 healthy controls. Demographic data, prednisone dose, disease remission state, disease severity, and abdominal pain were also evaluated. RESULTS: Based on the child self-reports, physical, emotional, school, and total scores were significantly lower in autoimmune hepatitis patients when compared with controls (p<0...
January 10, 2018: Jornal de Pediatria
https://www.readbyqxmd.com/read/29327413/itpa-activity-in-children-treated-by-azathioprine-relationship-to-the-occurrence-of-adverse-drug-reactions-and-inflammatory-response
#15
Antony Citterio-Quentin, Mustapha Moulsma, Marie-Paule Gustin, Alain Lachaux, Roselyne Boulieu
Azathioprine (AZA), a thiopurine drug, is widely used in the treatment of children with immunological diseases such as inflammatory bowel disease (IBD) and autoimmune hepatitis (AIH); however, interindividual variability in the occurrence of adverse drug reactions (ADRs) and drug response is observed. This study investigated (i) the relationships between inosine triphosphate pyrophosphatase (ITPA) activity, an enzyme involved in thiopurine metabolism, and the occurrence of ADRs in children with immunological disease on AZA therapy, and (ii) the relationship between ITPA activity and the inflammatory activity observed in children with IBD...
January 11, 2018: Basic & Clinical Pharmacology & Toxicology
https://www.readbyqxmd.com/read/29323192/baseline-il-2-and-the-aih-score-can-predict-the-response-to-standard-therapy-in-paediatric-autoimmune-hepatitis
#16
Jana Diestelhorst, Norman Junge, Danny Jonigk, Jerome Schlue, Christine S Falk, Michael P Manns, Ulrich Baumann, Elmar Jaeckel, Richard Taubert
Although autoimmune hepatitis (AIH) can be treated with corticosteroid-based first-line therapy, incomplete remission is associated with progressive liver fibrosis. So far accepted predictors of the subsequent treatment response of AIH patients are lacking. Therefore, we analysed baseline parameters, including iron homeostasis and cytokine levels, in 60 children with paediatric AIH (pAIH). In contrast to adults, elevated serum markers indicating iron overload were not commonly found in children. Therefore, ferritin was not predictive of the treatment response in pAIH...
January 11, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29316057/a-promising-medium-term-follow-up-of-pediatric-sclerosing-cholangitis-mild-phenotype-or-early-diagnosis
#17
Federica Ferrari, Giusy Ranucci, Marina Aloi, Luca Della Volpe, Franca Viola, Erasmo Miele, Salvatore Cucchiara, Raffaele Iorio
AIM: Sclerosing cholangitis (SC) is a chronic cholestatic liver disease that is being increasingly diagnosed in childhood. The long-term course and prognosis of pediatric SC are poorly described. METHODS: We reviewed data of pediatric SC patients, followed in two referral centers, during a period of up to 20 years. We aimed to evaluate long-term outcomes according to SC phenotype. RESULTS: Among 45 patients (median age, 10.4 years; male patients, 73...
January 9, 2018: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/29274889/new-onset-autoimmune-hepatitis-during-anti-tumor-necrosis-factor-alpha-treatment-in-children
#18
Amanda Ricciuto, Binita M Kamath, Thomas D Walters, Karen Frost, Nicholas Carman, Peter C Church, Simon C Ling, Anne M Griffiths
OBJECTIVES: To evaluate a large anti-tumor necrosis factor (TNF)-treated pediatric inflammatory bowel disease cohort for drug-induced liver injury (DILI) following presentation of an index case with suspected DILI with autoimmune features after infliximab exposure. To characterize the incidence, natural history, and risk factors for liver enzyme elevation with anti-TNF use. STUDY DESIGN: We reviewed the index case and performed a retrospective cohort study of 659 children receiving anti-TNF therapy between 2000 and 2015 at a tertiary pediatric inflammatory bowel disease center...
March 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29233784/altered-natural-killer-cell-cytokine-profile-in-type-2-autoimmune-hepatitis
#19
Dalila Mele, Grazia Bossi, Giuseppe Maggiore, Barbara Oliviero, Stefania Mantovani, Beatrice Bonelli, Mario U Mondelli, Stefania Varchetta
Type 2 autoimmune hepatitis (AIH-2) is a rare disease presenting in early childhood. The immunopathogenetic mechanisms are poorly characterized, although a defect of regulatory T cells (Treg) has been shown. There is virtually no information on innate immune responses and natural killer (NK) cells in particular. We have performed an extended immunophenotypic and functional analysis of NK cells in children with AIH-2. We show that NK cell frequency is reduced in this setting and that the balance between NK activating and inhibitory receptors is skewed toward activation...
March 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/29207801/autoimmune-haemolytic-anaemia-a-spectrum-of-presentation-in-children
#20
Amitabh Singh, Anirban Mandal, Ankita Patel, Sataroopa Mishra
Autoimmune Haemolytic Anaemias (AIHAs) are rare in children. They can be either a primary disease or secondary to/triggered by a host of other clinical conditions. We present five interesting cases of paediatric AIHA associated with infections (viral, bacterial and atypical) and autoimmune diseases {Systemic Lupus Erythematosus (SLE) and Autoimmune Hepatitis (AIH)}. The H1N1 influenza associated AIHA responded to oseltamivir and Intravenous Immunoglobulin (IvIg) while the cases secondary to Mycoplasma pneumoniae and pneumococcal bacteraemia required only treatment of the primary infection...
September 2017: Journal of Clinical and Diagnostic Research: JCDR
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