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Autoimmune hepatitis in children

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https://www.readbyqxmd.com/read/28390159/the-natural-history-of-primary-sclerosing-cholangitis-in-781-children-a-multicenter-international-collaboration
#1
Mark R Deneau, Wael El-Matary, Pamela L Valentino, Reham Abdou, Khaled Alqoaer, Mansi Amin, Achiya Z Amir, Marcus Auth, Fateh Bazerbachi, Annemarie Broderick, Albert Chan, Jillian Cotter, Sylvia Doan, Mounif El-Youssef, Federica Ferrari, Katryn N Furuya, Madeleine Gottrand, Frederic Gottrand, Nitika Gupta, Matjaz Homan, M K Jensen, Binita M Kamath, Kyung Mo Kim, Kaija-Leena Kolho, Anastasia Konidari, Bart Koot, Raffaele Iorio, Oren Ledder, Cara Mack, Mercedes Martinez, Tamir Miloh, Parvathi Mohan, Niamh O'Cathain, Alexandra Papadopoulou, Amanda Ricciuto, Lawrence Saubermann, Pushpa Sathya, Eyal Shteyer, Vratislav Smolka, Atushi Tanaka, Raghu Varier, Veena Venkat, Bernadette Vitola, Miriam B Vos, Marek Woynarowski, Jason Yap
BACKGROUND: There are limited data on the natural history of primary sclerosing cholangitis (PSC) in children. We aimed to describe the disease characteristics and long-term outcomes of pediatric PSC. METHODS: We retrospectively collected all pediatric PSC cases from 36 participating institutions and conducted a survival analysis from the date of PSC diagnosis to dates of diagnosis of portal hypertensive or biliary complications, cholangiocarcinoma, liver transplantation, or death...
April 8, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28348471/role-of-allopurinol-in-optimizing-thiopurine-therapy-in-patients-with-autoimmune-hepatitis-a-review
#2
REVIEW
Shivani Deswal, Anshu Srivastava
Autoimmune hepatitis (AIH) is a chronic immune mediated liver disease characterized by elevated transaminases, hyper gammaglobulinemia, presence of autoantibodies and interface hepatitis in the absence of a known etiology of liver disease. Thiopurines (azathioprine [AZA]/6-mercaptopurine [6MP]) and steroids remain the first line of treatment of AIH in both children and adults. However, a small proportion of AIH patients are either non-responders or develop side effects with AZA. The metabolism of AZA is complex and mediated by multiple enzymes...
March 2017: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/28326205/shigella-sonnei-bacteremia-presenting-with-profound-hepatic-dysfunction
#3
Oluwaseun Shogbesan, Andrew Rettew, Bilal Shaikh, Abdullateef Abdulkareem, Anthony Donato
Worldwide, Shigellosis is a significant public health issue, associated with nearly one million deaths annually. About half a million cases of Shigella infection are reported annually in the United States. Shigella bacteremia is uncommon and generally seen in children and immunocompromised adults. We present a case of a Shigella sonnei bacteremia with marked hepatic derangement in a 27-year-old previously healthy homosexual male with history of Roux-en-Y gastric bypass, who presented to the emergency room with a 4-day history of loose watery stool, abdominal cramps, nausea and vomiting, and yellow skin of 2-day duration...
2017: Case Reports in Gastrointestinal Medicine
https://www.readbyqxmd.com/read/28272159/outcomes-in-pediatric-autoimmune-hepatitis-and-significance-of-azathioprine-metabolites
#4
Melissa A Sheiko, Shikha S Sundaram, Kelley E Capocelli, Zhaoxing Pan, Annette M McCoy, Cara L Mack
OBJECTIVES: Autoimmune hepatitis (AIH) is a common pediatric liver disease and long-term remission is usually maintained with azathioprine (AZA). There is no consensus on the target range for AZA active metabolite 6-thioguanine (6-TGN) levels in pediatric AIH. The aim of this study was to characterize the outcomes of pediatric patients with AIH and determine correlations between AZA dosing or 6-TGN metabolite levels and biochemical remission. METHODS: A retrospective chart review was performed and data on presentation, laboratories including AZA metabolite levels, medication use and outcomes was collected...
March 7, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28219489/associations-between-autoimmune-diseases-and-attention-deficit-hyperactivity-disorder-a%C3%A2-nationwide-study
#5
Philip Rising Nielsen, Michael Eriksen Benros, Søren Dalsgaard
OBJECTIVE: Recent studies have suggested that autoimmune diseases and immune activation play a part in the pathogenesis of different neurodevelopmental disorders. This study investigated the association between a personal history and a family history of autoimmune disease and the risk of developing attention-deficit/hyperactivity disorder (ADHD). METHOD: A cohort was formed of all singletons born in Denmark from 1990 to 2007, resulting in a study population of 983,680 individuals followed from 1995 to 2012...
March 2017: Journal of the American Academy of Child and Adolescent Psychiatry
https://www.readbyqxmd.com/read/28204893/gastrointestinal-system-manifestations-in-juvenile-systemic-lupus-erythematosus
#6
Hafize Emine Sönmez, Asuman Nur Karhan, Ezgi Deniz Batu, Yelda Bilginer, Ersin Gümüş, Hülya Demir, Aysel Yüce, Seza Özen
Systemic lupus erythematosus (SLE) is an autoimmune disease which may involve gastrointestinal system (GIS). The aim of this study was to present GIS manifestations of pediatric SLE patients. The medical files of 69 children with SLE followed between January 2011 and January 2016 were reviewed. All fulfilled the Systemic Lupus International Collaborating Clinics criteria. All patients (≤18 years of age) with GIS manifestations were included. GIS manifestations were observed in 19 (27.5%) out of 69 SLE patients and present at the time of SLE diagnosis in 13 (68...
February 16, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28199778/a-national-retrospective-study-of-paediatric-end-stage-liver-disease-as-a-predictor-of-change-to-second-line-therapy-in-children-with-autoimmune-hepatitis
#7
Andréanne N Zizzo, Carolina Jimenez-Rivera, Joseph Kim, Richard A Schreiber, Simon C Ling, Jason Yap, Jeff Critch, Najma Ahmed, Fernando Alvarez, Binita M Kamath
BACKGROUND & AIMS: Adult studies of autoimmune hepatitis (AIH) have shown that the model of end-stage liver disease is associated with resistance to first-line treatment. Using a multicentre retrospective database, we sought to determine if the paediatric end-stage liver disease (PELD) score would similarly predict treatment resistance in paediatric AIH. METHODS: One hundred and seventy-one children from 13 Canadian centres who fulfilled the International Autoimmune Hepatitis Group (IAIHG) criteria were included and assessed for change to second-line therapy within 24 months of primary treatment onset...
February 15, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28172972/p486-immunization-status-of-children-and-adolescents-with-inflammatory-bowel-disease-or-autoimmune-hepatitis-in-germany
#8
L Cagol, K Frivolt, A Krahl, N Förster, E Lainka, P Gerner, D Ney, J Vermehren, S Trenkel, M Radke, S Koletzko, C Posovszky
No abstract text is available yet for this article.
February 1, 2017: Journal of Crohn's & Colitis
https://www.readbyqxmd.com/read/28169970/second-line-agents-in-pediatric-patients-with-autoimmune-hepatitis-a-systematic-review-and-meta-analysis
#9
Andréanne N Zizzo, Pamela L Valentino, Prakesh S Shah, Binita M Kamath
BACKGROUND AND AIMS: Ten to 20% of children with autoimmune hepatitis 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 employed to identify potential studies for inclusion...
February 4, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28154539/temporal-association-of-certain-neuropsychiatric-disorders-following-vaccination-of-children-and-adolescents-a-pilot-case-control-study
#10
Douglas L Leslie, Robert A Kobre, Brian J Richmand, Selin Aktan Guloksuz, James F Leckman
BACKGROUND: Although the association of the measles, mumps, and rubella vaccine with autism spectrum disorder has been convincingly disproven, the onset of certain brain-related autoimmune and inflammatory disorders has been found to be temporally associated with the antecedent administration of various vaccines. This study examines whether antecedent vaccinations are associated with increased incidence of obsessive-compulsive disorder (OCD), anorexia nervosa (AN), anxiety disorder, chronic tic disorder, attention deficit hyperactivity disorder, major depressive disorder, and bipolar disorder in a national sample of privately insured children...
2017: Frontiers in Psychiatry
https://www.readbyqxmd.com/read/28097735/autoimmune-enteropathy-and-hepatitis-in-pediatric-heart-transplant-recipient
#11
Kimberly Lewis, Ryan Butts, J Antonio Quiros, Michelle Hudspeth, Katherine Twombley, Andrew Savage, Sally Self, Ali Burnette, Shaoli Sun
AIE is a rare disorder in children that presents with severe diarrhea and malabsorption, caused by immune-mediated damage to intestinal mucosa. AIE is often associated with various syndromes of immunodeficiency including IPEX syndrome (immune dysregulation, polyendocrinopathy and enteropathy, X-linked). Dysfunctional T regulatory cells are the source of pathology in both IPEX syndrome and AIE as they are essential in maintaining tolerance to self-antigens and eliminating autoreactive B cells. This case report describes a 10-year-old cardiac transplant and total thymectomy patient on chronic immunosuppression with tacrolimus that presented with AIE and extraintestinal manifestations of cyclical hepatitis...
January 17, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/27914924/the-management-of-childhood-liver-diseases-in-adulthood
#12
REVIEW
Deepak Joshi, Nitika Gupta, Marianne Samyn, Maesha Deheragoda, Fabienne Dobbels, Michael A Heneghan
An increasing number of patients with childhood liver disease survive into adulthood. These young adults are now entering adult services and require ongoing management. Aetiologies can be divided into liver diseases that develop in young adults which present to adult hepatologists i.e., biliary atresia and Alagille syndrome or liver diseases that occur in children/adolescents and adults i.e., autoimmune hepatitis or Wilson's disease. To successfully manage these young adults, a dynamic and responsive transition service is essential...
March 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/27759901/human-leucocyte-antigens-b-08-drb1-03-and-drb1-13-are-significantly-associated-with-autoimmune-liver-and-biliary-diseases-in-finnish-children
#13
E Ylinen, L Salmela, J Peräsaari, T Jaatinen, A Tenca, O Vapalahti, M Färkkilä, H Jalanko, K-L Kolho
AIM: The human leucocyte 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 three years to 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 four years and three months to 14...
February 2017: Acta Paediatrica
https://www.readbyqxmd.com/read/27755342/liver-transplantation-for-children-with-primary-sclerosing-cholangitis-and-autoimmune-hepatitis-unos-database-analysis
#14
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 era. We aimed to characterize the outcome of LT in children with AIH and PSC. METHODS: Children 18 years or younger with PSC or AIH who had a first, isolated LT from 2002 to 2012 were identified from the United Network for Organ Sharing database...
April 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27709857/distribution-of-diseases-causing-liver-function-test-abnormality-in-children-and-natural-recovery-time-of-the-abnormal-liver-function
#15
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
https://www.readbyqxmd.com/read/27688687/incidence-and-characteristics-of-hepatitis-e-virus-infection-in-children-in-assiut-upper-egypt
#16
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. Results A total of 123 patients (73 boys, 50 girls) were included in the analysis...
October 2016: Journal of International Medical Research
https://www.readbyqxmd.com/read/27660052/c14orf132-gene-is-possibly-related-to-extremely-low-birth-weight
#17
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
https://www.readbyqxmd.com/read/27633047/the-use-of-tacrolimus-in-refractory-autoimmune-hepatitis-in-children-and-adults-a-single-center-experience
#18
Haider Al Taii, Mohamad A Hanouneh, Ibrahim Hanouneh, Rocio Lopez, Nizar Zein, Naim Alkhouri
No abstract text is available yet for this article.
February 2017: Scandinavian Journal of Gastroenterology
https://www.readbyqxmd.com/read/27540711/hepatic-inflammation-may-influence-liver-stiffness-measurements-by-transient-elastography-in-children-and-young-adults
#19
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 (LSMs) in children/young adults. METHODS: This was a cohort analysis of children/young adults who underwent TE within 1 year of liver biopsy. Alanine aminotransferase (ALT) was obtained within 30 days of the biopsy and LSM...
April 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27498582/long-term-follow-up-of-children-and-adolescents-with-primary-sclerosing-cholangitis-and-autoimmune-sclerosing-cholangitis
#20
COMPARATIVE STUDY
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
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