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https://www.readbyqxmd.com/read/28219566/immunoglobulin-g4-related-periaortitis-involving-the-aortic-arch-mimicking-a-mediastinal-tumor
#1
Min Jae Cha, Semin Chong, Yang Soo Kim, Byungjoon Park, Ja Hee Seo, Eun Sung Lee
Immunoglobulin G4 (IgG4)-related sclerosing disease is a recently proposed systemic fibroinflammatory disease entity, with periaortitis as a representative manifestation in the cardiovascular system. We present a case of IgG4-related periaortitis involving the aortic arch and proximal great vessels, mimicking an anterior mediastinal tumor on computed tomography. In patients with a soft tissue mass along the aortic wall, IgG4-related periaortitis should be included as a differential diagnosis and timely serologic investigation should be performed to prevent unnecessary surgery, because the disease shows a remarkable response to corticosteroids...
March 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28176451/epidemiology-and-risk-factors-for-infection-after-living-donor-liver-transplantation
#2
Cybele Lara R Abad, Brian D Lahr, Raymund R Razonable
BACKGROUND: The epidemiology of infections after living donor liver transplantation is limited. We aimed to study the epidemiology and risk factors of infections after living donor liver transplantation. METHODS: The medical records of 223 adult patients who underwent living donor liver transplantation from January 1, 2000 - August 31, 2015 were reviewed for all infections occurring up to 1 year. We estimated the cumulative incidence of infection using the Kaplan Meier product limit method...
February 8, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28146446/immunogenetics-in-primary-sclerosing-cholangitis
#3
Brian K Chung, Gideon M Hirschfield
PURPOSE OF REVIEW: Primary sclerosing cholangitis (PSC) is a progressive biliary liver disorder strongly associated with inflammatory bowel disease (PSC-IBD). We summarize the genetics of PSC-IBD and highlight recent findings that further differentiate PSC-IBD as a unique disease. RECENT FINDINGS: To date, genome-wide studies have uncovered 23 susceptibility loci for PSC-IBD, the majority of which have been previously reported as risk factors in other immune-mediated disorders...
March 2017: Current Opinion in Gastroenterology
https://www.readbyqxmd.com/read/28141916/justifying-non-standard-exception-requests-for-pediatric-liver-transplant-candidates-an-analysis-of-narratives-submitted-to-the-united-network-for-organ-sharing-2009-2014
#4
Emily R Perito, Hillary J Braun, Jennifer L Dodge, Sue Rhee, John P Roberts
Non-standard exception requests (NSERs), for which transplant centers provide patient-specific narratives to support a higher MELD/PELD 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 UNOS data, 1,272 NSER narratives from 1,138 children with NSERs were coded for analysis. The most common NSER justifications were failure-to-thrive (48%) and risk of death (40%); both associated with approval...
January 31, 2017: American Journal of Transplantation
https://www.readbyqxmd.com/read/28111336/clinical-features-response-to-treatment-and-outcomes-of-igg4-related-sclerosing-cholangitis
#5
Atsushi Tanaka, Susumu Tazuma, Kazuichi Okazaki, Takahiro Nakazawa, Kazuo Inui, Tsutomu Chiba, Hajime Takikawa
BACKGROUND & AIMS: Immunoglobulin G4 sclerosing cholangitis (IgG4SC) is a biliary tract manifestation of IgG4-related systemic disease. We investigated the demographics, clinical features at presentation, treatment response, and outcomes of IgG4-SC using data from a large-scale survey in Japan. METHODS: We performed a retrospective cohort study of 527 patients with IgG4-SC (436 female; median age, 66.2 years; range, 23-89 years) in Japan from 2000 to 2015. Data on patient demographics, presentation, treatment response, and outcomes were collected from questionnaires given to patients at 211 referral centers in Japan in 2015...
January 19, 2017: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28109714/biliary-tumors-with-pancreatic-counterparts
#6
Yasuni Nakanuma, Yoshiko Sudo
Some biliary diseases mimic pancreatic diseases pathologically as well as pathogenetically. Such diseases can be called "biliary diseases with pancreatic counterparts". Biliary intraepithelial neoplasm (BilIN), intraductal papillary neoplasm of bile ducts (IPNB), hepatobiliary mucinous cystic neoplasm (hMCN), and IgG4-inflammatory pseudotumor represent the biliary counterparts of pancreatic intraepithelial neoplasm (PanIN), intraductal papillary mucinous neoplasm of pancreas (IPMN), pancreatic MCN, and mass forming type 1 autoimmune pancreatitis (AIP), respectively...
December 20, 2016: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/28105357/immunoglobulin-g4-mediated-sclerosing-cholangitis-as-a-risk-factor-for-cholangiocarcinoma-a-case-report
#7
Karin E Koopman, Elisabeth Bloemena, Geert Kazemier, Michael Klemt-Kropp
Immunoglobulin (Ig)G4-mediated disease is a systemic autoimmune disease, which occasionally presents solely as sclerosing cholangitis (SC). IgG4-mediated SC is challenging to diagnose, as it may mimic cholangiocarcinoma radiologically, and carcinoma cells may produce IgG4. The diagnosis of IgG4-mediated disease is based on histological consensus criteria and response to corticosteroids. In addition to the radiological and histological overlap between IgG4-mediated SC and cholangiocarcinoma, IgG4-mediated SC may be considered as a risk factor for the development of cholangiocarcinoma...
December 2016: Molecular and Clinical Oncology
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/28060780/18f-fdg-pet-ct-features-of-chronic-sclerosing-sialadenitis-presenting-as-localized-igg4-related-disease
#9
Nobuo Kashiwagi, Eisuke Enoki, Chisa Hosokawa, Kenta Sakaguchi, Takamichi Murakami
Chronic sclerosing sialadenitis is a benign inflammatory condition that most commonly affects the submandibular gland in elderly individuals. It is currently known to belong to the spectrum of IgG4-related systemic diseases, which is reflected by systemic involvement on F-FDG PET/CT images. We presented a case of a 73-year-old man with histologically proven IgG4-related chronic sclerosing sialadenitis, unilateral localized form, on whole-body F-FDG PET/CT images that mimicked submandibular carcinoma with lymph node metastasis...
February 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28055006/biliary-tract-instillation-of-a-smac-mimetic-induces-trail-dependent-acute-sclerosing-cholangitis-like-injury-in-mice
#10
Maria Eugenia Guicciardi, Anuradha Krishnan, Steven F Bronk, Petra Hirsova, Thomas S Griffith, Gregory J Gores
Primary sclerosing cholangitis (PSC) is a cholestatic liver disease of unknown etiopathogenesis characterized by fibrous cholangiopathy of large and small bile ducts. Systemic administration of a murine TNF-related apoptosis-inducing ligand (TRAIL) receptor agonist induces a sclerosing cholangitis injury in C57BL/6 mice, suggesting endogenous TRAIL may contribute to sclerosing cholangitis syndromes. Cellular inhibitor of apoptosis proteins (cIAP-1 and cIAP-2) are negative regulators of inflammation and TRAIL receptor signaling...
January 5, 2017: Cell Death & Disease
https://www.readbyqxmd.com/read/28040756/current-practices-in-the-management-of-malignant-pleural-effusions-a-survey-among-members-of-the-european-society-of-thoracic-surgeons
#11
Marco Scarci, Edward Caruana, Luca Bertolaccini, Benedetta Bedetti, Alessandro Brunelli, Gonzalo Varela, Kostas Papagiannopoulos, Jaroslaw Kuzdzal, Gilbert Massard, Enrico Ruffini, Pierre Emmanuel Falcoz, Isabelle Opitz, Hasan Batirel, Alper Toker, Gaetano Rocco
OBJECTIVES: Malignant pleural effusion (MPE) commonly complicates advanced malignancy and their exact management is still undefined. We undertook a survey to determine the current practice among members of the European Society of Thoracic Surgeons (ESTS). METHODS: A cross-sectional survey focused on the current practice of management of MPE was developed by the authors. The questions were outlined after a review of the literature and circulated in an Internet-based survey format...
December 31, 2016: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/27938792/surveillance-in-cholangiocellular-carcinoma
#12
REVIEW
Thomas C Wirth, Arndt Vogel
Cholangiocellular carcinoma is the most frequent malignant neoplasm originating from the epithelium of intra- or extrahepatic bile ducts. In the past decades, the incidence of cholangiocarcinoma has been shown to increase while overall mortality has remained high with an approximate 5-year overall survival below 20%. Surgery remains the only curative option while systemic treatment is limited to palliative chemotherapy. Therefore, surveillance strategies for patients at risk of developing cholangiocarcinoma are urgently needed, particularly in patients with primary sclerosing cholangitis and patients infected with liver flukes...
December 2016: Best Practice & Research. Clinical Gastroenterology
https://www.readbyqxmd.com/read/27924081/liver-guardian-modifier-and-target-of-sepsis
#13
REVIEW
Pavel Strnad, Frank Tacke, Alexander Koch, Christian Trautwein
Sepsis and septic shock are characterized by life-threatening organ dysfunction caused by a dysregulated host response to infection. The liver has a central role during sepsis, and is essential to the regulation of immune defence during systemic infections by mechanisms such as bacterial clearance, acute-phase protein or cytokine production and metabolic adaptation to inflammation. However, the liver is also a target for sepsis-related injury, including hypoxic hepatitis due to ischaemia and shock, cholestasis due to altered bile metabolism, hepatocellular injury due to drug toxicity or overwhelming inflammation, as well as distinct pathologies such as secondary sclerosing cholangitis in critically ill patients...
January 2017: Nature Reviews. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/27921051/systemic-mastocytosis-causing-refractory-pruritus-in-a-liver-disease-patient
#14
Naga S Addepally, Jagpal S Klair, Mohit Girotra, Johnny Jones, Farshad Aduli
Systemic mastocytosis (SM) results from clonal, neoplastic proliferation of abnormal mast cells. Patients become susceptible to itching, urticaria, and anaphylactic shock, which occurs due to histamine release from mast cells. SM may coexist alongside other systemic diseases, thus confounding the overall clinical presentation. We discuss a 23-year-old woman with refractory pruritus, which was initially attributed to primary sclerosing cholangitis but had a nonresponse to antihistaminics, ursodiol, and cholestyramine...
August 2016: ACG Case Reports Journal
https://www.readbyqxmd.com/read/27882046/the-role-of-genetic-and-immune-factors-for-the-pathogenesis-of-primary-sclerosing-cholangitis-in-childhood
#15
REVIEW
Priscila Menezes Ferri, Ana Cristina Simões E Silva, Soraya Luiza Campos Silva, Diego Junior Queiroga de Aquino, Eleonora Druve Tavares Fagundes, Débora Marques de Miranda, Alexandre Rodrigues Ferreira
Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease characterized by chronic inflammation of the biliary tree resulting in liver fibrosis. PSC is more common in male less than 40 years of age. The diagnosis of PSC is based on clinical, laboratory, image, and histological findings. A biochemical profile of mild to severe chronic cholestasis can be observed. Endoscopic retrograde cholangiography is the golden standard method for diagnosis, but magnetic resonance cholangiography is currently also considered a first-line method of investigation...
2016: Gastroenterology Research and Practice
https://www.readbyqxmd.com/read/27880989/validation-of-the-prognostic-value-of-histologic-scoring-systems-in-primary-sclerosing-cholangitis-an-international-cohort-study
#16
Elisabeth M G de Vries, Manon de Krijger, Martti Färkkilä, Johanna Arola, Peter Schirmacher, Daniel Gotthardt, Benjamin Goeppert, Palak J Trivedi, Gideon M Hirschfield, Henriette Ytting, Ben Vainer, Henk R van Buuren, Katharina Biermann, Maren H Harms, Olivier Chazouilleres, Dominique Wendum, Astrid D Kemgang, Roger W Chapman, Lai Mun Wang, Kate D Williamson, Annette S H Gouw, Valerie Paradis, Christine Sempoux, Ulrich Beuers, Stefan G Hübscher, Joanne Verheij, Cyriel Y Ponsioen
: Histologic scoring systems specific for primary sclerosing cholangitis (PSC) are not validated. We recently determined the applicability and prognostic value of three histological scoring systems in a single PSC cohort. The aim of this study was to validate their prognostic use and reproducibility across a multicenter PSC cohort. Liver biopsies from PSC patients were collected from seven European institutions. Histologic scoring was performed using the Nakanuma, Ishak, and Ludwig scoring systems...
November 23, 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/27853829/-hepatopancreaticobiliary-diseases-in-igg4-associated-autoimmune-diseases
#17
L Grenacher
BACKGROUND: IgG4-related diseases are rare systemic multiorgan diseases and can thus affect any organ system. The incidence of diagnosis has significantly increased due to increasing awareness. OBJECTIVE: In the abdomen the hepatopancreaticobiliary system provides an essential organ system for the expression of IgG4-associated autoimmune diseases. The focus here is autoimmune pancreatitis type 1 but IgG4-associated sclerosing cholangitis and IgG4-associated hepatopathy, which can also occur in combination are less well-known...
December 2016: Der Radiologe
https://www.readbyqxmd.com/read/27852547/prevalence-of-igg-4-associated-cholangiopathy-based-on-serum-igg-4-levels-in-patients-with-primary-sclerosing-cholangitis-and-its-relationship-with-inflammatory-bowel-disease
#18
Seyed Alireza Taghavi, Shaghayegh Kaedi Majd, Mahnoosh Sianati, Masood Sepehrimanesh
BACKGROUND/AIMS: Autoimmune cholangiopathy is part of a fibro-inflammatory immunoglobulin G-4 (IgG-4)-related systemic disease that causes biliary tract strictures. Its clinical presentation is quite similar to that of more common diseases such as primary sclerosing cholangitis (PSC) and pancreatobilliary malignancies. The aims of the present study were to evaluate the prevalence of IgG-4-associated cholangiopathy (IAC) in patients diagnosed with PSC and its relationship with inflammatory bowel disease (IBD)...
November 2016: Turkish Journal of Gastroenterology: the Official Journal of Turkish Society of Gastroenterology
https://www.readbyqxmd.com/read/27802157/gut-microbiome-and-liver-diseases
#19
REVIEW
Herbert Tilg, Patrice D Cani, Emeran A Mayer
The gut microbiota has recently evolved as a new important player in the pathophysiology of many intestinal and extraintestinal diseases. The liver is the organ which is in closest contact with the intestinal tract, and is exposed to a substantial amount of bacterial components and metabolites. Various liver disorders such as alcoholic liver disease, non-alcoholic liver disease and primary sclerosing cholangitis have been associated with an altered microbiome. This dysbiosis may influence the degree of hepatic steatosis, inflammation and fibrosis through multiple interactions with the host's immune system and other cell types...
December 2016: Gut
https://www.readbyqxmd.com/read/27801954/the-world-health-organization-2016-classification-of-testicular-non-germ-cell-tumours-a-review-and-update-from-the-international-society-of-urological-pathology-testis-consultation-panel
#20
REVIEW
Muhammad T Idrees, Thomas M Ulbright, Esther Oliva, Robert H Young, Rodolfo Montironi, Lars Egevad, Daniel Berney, John R Srigley, Jonathan I Epstein, Satish K Tickoo
The World Health Organization (WHO) released a new tumour classification for the genitourinary system in early 2016 after consensus by pathologists with expertise in these organs. It utilized the framework of the 2004 classification, and incorporated the most up-to-date information concerning these tumours. In testicular tumours, the majority of the changes occurred in the nomenclature and classification of germ cell tumours; however, several modifications were also made for non-germ cell tumours. Among sex cord-stromal tumours, sclerosing Sertoli cell tumour (SCT) is no longer recognized as a separate entity but as a morphological variant of SCT not otherwise specified (NOS), as CTNNB1 gene mutations have been noted in both neoplasms but not in the other forms of SCT...
March 2017: Histopathology
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