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Sclerosing cholangitis 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/28302003/liver-transplant-for-nonhepatocellular-carcinoma-malignancy
#2
Nihan Haberal Reyhan
Liver transplant is now an acceptable and effective treatment for specific nonhepatocellular malignancies. Worldwide, hilar cholangiocarcinoma accounts for 3% of all primary gastrointestinal malignancies and for 10% of primary hepatobiliary malignancies. For patients who have early-stage, unresectable cholangiocarcinoma, liver transplant preceded by neoadjuvant radiotherapy can result in tumor-free margins, accomplish a radical resection, and treat the underlying primary sclerosing cholangitis when present...
March 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28281846/autoimmune-liver-diseases-and-inflammatory-bowel-diseases-in-children-current-issues-and-future-perspectives
#3
Sabrina Cardile, Tommaso Alterio, Manila Candusso, Andrea Pietrobattista, Daniela Liccardo, Maria Sole Basso, Bronislava Papadatou, Fiammetta Bracci, Daniela Knafelz, Giuliano Torre
Inflammatory bowel diseases (IBDs) represent a group of intestinal disorders with a chronic and relapsing inflammation of the gut, and with a potential risk of systemic involvement of other organs and systems. Over the pediatric age, an incidence higher than 20% of developing extraintestinal manifestation during follow-up has been reported. The liver and the biliary system are frequently involved, and primary sclerosing cholangitis (PSC) is the most predominant entity with an incidence rate of 6.4-7.8% in children...
June 2017: Scandinavian Journal of Gastroenterology
https://www.readbyqxmd.com/read/28188033/success-and-safety-of-endoscopic-retrograde-cholangiopancreatography-in-children
#4
Jordan D Rosen, Rebecca S Lane, Jose M Martinez, Eduardo A Perez, Jun Tashiro, Amy E Wagenaar, Robert M Van Haren, Ashwini Kumar, Juan E Sola
PURPOSE: Despite its diagnostic and therapeutic utility, endoscopic retrograde cholangiopancreatography (ERCP) is underutilized in children. METHODS: Patients younger than 18years undergoing ERCP from 2000 to 2014 at a children's hospital were identified. Patient characteristics and outcomes were evaluated. RESULTS: Overall, 215 ERCPs (78% therapeutic) were performed in 184 patients. Our cohort was 67% female, with a median age (IQR) of 14 (8) years...
February 1, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28143594/prevalence-of-paediatric-inflammatory-bowel-disease-in-sweden-a-nationwide-population-based-register-study
#5
J F Ludvigsson, K Büsch, O Olén, J Askling, K E Smedby, A Ekbom, E Lindberg, M Neovius
BACKGROUND: We evaluated the impact of different case definition algorithms on the prevalence of paediatric inflammatory bowel disease (IBD), Crohn's disease (CD) and ulcerative colitis (UC) and to compare the occurrence of certain diseases compared to matched controls. METHODS: Paediatric patients (<18 years) were identified via ICD codes for UC and CD in Swedish registers between 1993 and 2010 (n = 1432). Prevalence was defined as ≥2 IBD-related visits...
January 31, 2017: BMC Gastroenterology
https://www.readbyqxmd.com/read/28141916/justifying-nonstandard-exception-requests-for-pediatric-liver-transplant-candidates-an-analysis-of-narratives-submitted-to-the-united-network-for-organ-sharing-2009-2014
#6
E R Perito, H J Braun, J L Dodge, S Rhee, J P Roberts
Nonstandard exception requests (NSERs), for which transplant centers provide patient-specific narratives to support a higher Model for End-stage Liver Disease/Pediatric End-stage Liver Disease score, are made for >30% of pediatric liver transplant candidates. We describe the justifications used in pediatric NSER narratives 2009-2014 and identify justifications associated with NSER denial, waitlist mortality, and transplant. Using United Network for Organ Sharing data, 1272 NSER narratives from 1138 children with NSERs were coded for analysis...
January 31, 2017: American Journal of Transplantation
https://www.readbyqxmd.com/read/28141677/the-unique-inflammatory-bowel-disease-phenotype-of-pediatric-primary-sclerosing-cholangitis-a-single-center-study
#7
Henry Shiau, Faith D Ihekweazu, Mansi Amin, Tatiana Fofanova, Tamir Miloh, Richard Kellermayer
BACKGROUND: In adults, primary sclerosing cholangitis (PSC), a cholestatic liver disease characterized by inflammation/fibrosis of intra/extra-hepatic bile ducts, associates with a milder form of inflammatory bowel disease (IBD), particularly ulcerative colitis (UC). The pediatric PSC-IBD phenotype is less well characterized. METHODS: We performed a retrospective, single-center study examining PSC-IBD patients at Texas Children's Hospital (TCH) between 2000-2015...
January 28, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28087208/-child-langerhans-cell-histiocytosis
#8
REVIEW
Jean Donadieu, Sébastien Héritier
DEFINITION: Langerhans cell histiocytosis (LCH) is defined by the association of a clinical and radiological involvement and a biopsy of a pathological tissue. Extension: it can affect any organ or system of the body but most commonly the bone (80% of cases), the skin (33%) and the pituitary (25%). Other organs are concerned such as liver, spleen, hematopoietic system and the lungs (15% each), lymph nodes (5-10%) and central nervous system (CNS) excluding the pituitary (2-4%). Natural history: the natural history of the disease is very heterogeneous, ranging from auto-regressive lesions to a disease affecting multiple organs with fatal consequences, while some lesions may be responsible for permanent sequels...
January 2017: La Presse Médicale
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
#9
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
#10
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/27504812/the-natural-history-of-primary-sclerosing-cholangitis-in-children-a-large-single-center-longitudinal-cohort-study
#11
Pamela L Valentino, Shanna Wiggins, Sarah Harney, Roshan Raza, Christine K Lee, Maureen M Jonas
OBJECTIVES: Data regarding pediatric primary sclerosing cholangitis (PSC) natural history are limited. We describe a large pediatric PSC cohort with longitudinal follow-up. METHODS: The present study records review of pediatric patients with PSC diagnosed between 1984 and 2014. RESULTS: N = 120 (63% M) ages 1 to 21 years (median 14 years) at diagnosis. 27% (31/113) had autoimmune sclerosing cholangitis (ASC), 24% had exclusive small duct PSC, METAVIR stage was F3-F4 in 41%...
December 2016: 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
#12
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
https://www.readbyqxmd.com/read/27469900/mutations-in-dcdc2-doublecortin-domain-containing-protein-2-in-neonatal-sclerosing-cholangitis
#13
Tassos Grammatikopoulos, Melissa Sambrotta, Sandra Strautnieks, Pierre Foskett, A S Knisely, Bart Wagner, Maesha Deheragoda, Chris Starling, Giorgina Mieli-Vergani, Joshua Smith, Laura Bull, Richard J Thompson
BACKGROUND & AIMS: Neonatal sclerosing cholangitis (NSC) is a severe neonatal-onset cholangiopathy commonly leading to liver transplantation (LT) for end-stage liver disease in childhood. Liver biopsy findings histopathologically resemble those in biliary atresia (BA); however, in NSC extrahepatic bile ducts are patent, whilst in BA their lumina are obliterated. NSC is commonly seen in consanguineous kindreds, suggesting autosomal recessive inheritance. METHODS: From 29 NSC patients (24 families) identified, DNA was available in 24 (21 families)...
December 2016: Journal of Hepatology
https://www.readbyqxmd.com/read/27319779/dcdc2-mutations-cause-neonatal-sclerosing-cholangitis
#14
Muriel Girard, Albane A Bizet, Alain Lachaux, Emmanuel Gonzales, Emilie Filhol, Sophie Collardeau-Frachon, Cécile Jeanpierre, Charline Henry, Monique Fabre, Loic Viremouneix, Louise Galmiche, Dominique Debray, Christine Bole-Feysot, Patrick Nitschke, Danièle Pariente, Catherine Guettier, Stanislas Lyonnet, Laurence Heidet, Aurelia Bertholet, Emmanuel Jacquemin, Alexandra Henrion-Caude, Sophie Saunier
Neonatal sclerosing cholangitis (NSC) is a rare biliary disease leading to liver transplantation in childhood. Patients with NSC and ichtyosis have already been identified with a CLDN1 mutation, encoding a tight-junction protein. However, for the majority of patients, the molecular basis of NSC remains unknown. We identified biallelic missense mutations or in-frame deletion in DCDC2 in four affected children. Mutations involve highly conserved amino acids in the doublecortin domains of the protein. In cholangiocytes, DCDC2 protein is normally located in the cytoplasm and cilia, whereas in patients the mutated protein is accumulated in the cytoplasm, absent from cilia, and associated with ciliogenesis defect...
October 2016: Human Mutation
https://www.readbyqxmd.com/read/27294341/extrarenal-manifestations-in-shigatoxin-associated-haemolytic-uremic-syndrome
#15
J Matthies, C Hünseler, R Ehren, R Volland, F Körber, B Hoppe, L T Weber, S Habbig
BACKGROUND: Shigatoxin-associated haemolytic uremic syndrome (STEC-HUS) is the most frequent cause of acute kidney injury in children worldwide. Extrarenal manifestations are the main determinants for both, short- and long-term prognosis of patients with STEC-HUS. PATIENTS: 46 patients treated over the last 10 years for STEC-HUS in a single center. METHODS: This retrospective study analysed the incidence and outcome of extrarenal manifestations in our cohort of children with STEC-HUS...
July 2016: Klinische Pädiatrie
https://www.readbyqxmd.com/read/27257963/recurrence-of-autoimmune-liver-disease-and-inflammatory-bowel-disease-after-pediatric-liver-transplantation
#16
REVIEW
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
https://www.readbyqxmd.com/read/26932879/child-with-jaundice-and-pruritus-how-to-evaluate
#17
REVIEW
Barath Jagadisan, Anshu Srivastava
Jaundice with pruritus is a manifestation of cholestasis. The defective biliary drainage causes accumulation of substances that are usually excreted in bile, which in turn causes pruritus. The exact nature of the pruritogen is under evaluation. However, lysophosphatidic acid is the current favourite. The causes of cholestasis can be broadly classified as intra or extrahepatic, with intrahepatic disorders being more often associated with pruritus. Cholestatic phase of acute viral hepatitis, progressive familial intrahepatic cholestasis, syndromic and non-syndromic paucity of intralobular bile ductules, drug induced cholestasis and sclerosing cholangitis (SC) are the common causes in children...
November 2016: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/26891438/-coexistence-of-coeliac-disease-and-inflammatory-bowel-disease-in-children
#18
REVIEW
Paulina Krawiec, Agnieszka Pawłowska-Kamieniak, Elżbieta Pac-Kożuchowska, Agnieszka Mroczkowska-Juchkiewcz, Katarzyna Kominek
Coeliac disease and inflammatory bowel disease are chronic inflammatory conditions of gastrointestinal tract with complex aetiology with genetic, environmental and immunological factors contributing to its pathogenesis. It was noted that immune-mediated disorders often coexist. There is well-known association between coeliac disease and type 1 diabetes and ulcerative colitis and primary sclerosing cholangitis. However, growing body of literature suggests the association between coeliac disease and inflammatory bowel disease, particularly ulcerative colitis...
January 2016: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/26835903/infliximab-associated-immunomediated-hepatitis-in-children-with-inflammatory-bowel-disease
#19
Shikib Mostamand, Shauna Schroeder, Jacqueline Schenkein, Tamir Miloh
Infliximab (IFX) is commonly used to induce and maintain remission in inflammatory bowel disease (IBD). We report the first 2 cases of children with ulcerative colitis who had normal liver transaminases before IFX and were diagnosed with immunomediated hepatitis after IFX induction. Both the cases had negative antibodies for antinuclear, smooth muscle, and liver kidney microsome, with 1 patient having positive autoimmune serology (dsDNA) and overlap primary sclerosing cholangitis. IFX was discontinued and transaminases normalized without steroid administration...
July 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/26825768/clinical-characteristics-and-prognosis-in-children-and-adolescents-with-autoimmune-hepatitis-and-overlap-syndrome
#20
Adriana T Rodrigues, Priscila M F Liu, Eleonora D T Fagundes, Thaís C N Queiroz, Pamela de Souza Haueisen Barbosa, Soraya L C Silva, Ana C Simões E Silva, Débora M Miranda, Alexandre R Ferreira, Luiz R Alberti
OBJECTIVES: This is a cohort study of 134 children and adolescents with a known diagnosis of autoimmune hepatitis (AIH). During follow-up, some of them developed autoimmune sclerosing cholangitis (ASC). This study describes the characteristics of the patients upon diagnosis, and their response to treatment and any complications, and compares the patients who developed ASC during follow-up (ASC group) with those who did not (AIH group). METHODS: A total of 73.1% of the patients were girls with a median age upon diagnosis of 10...
July 2016: Journal of Pediatric Gastroenterology and Nutrition
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