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Nodular regenerative hyperplasia

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https://www.readbyqxmd.com/read/29324586/ten-year-experience-of-transjugular-intrahepatic-portosystemic-shunt-for-noncirrhotic-portal-hypertension
#1
David Regnault, Louis d'Alteroche, Charlotte Nicolas, Fanny Dujardin, Jean Ayoub, Jean Marc Perarnau
BACKGROUND: Transjugular intrahepatic portosystemic shunt (TIPS) is considered to be well suited for the treatment of noncirrhotic portal hypertension (NCPHT) because of a usually severe portal hypertension (PHT) and a mild liver failure, but very less data are available. PATIENTS AND METHODS: Records of patients referred for TIPS between 2004 and 2015 for NCPHT were reviewed. No patient should have clinical or biological or histological features of cirrhosis. RESULTS: Twenty-five patients with a wide variety of histological lesions (sinusoidal dilatations, granulomatosis, regenerative nodular hyperplasia, obliterative portal venopathy, or subnormal liver) and a wide variety of associated diseases (thrombophilia, sarcoidosis, common variable immunodeficiency, scleroderma, Castleman's disease, early primitive biliary cirrhosis, congenital liver fibrosis, chemotherapy, purinethol intake, and congenital varices) were included...
January 10, 2018: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/29246416/hepatobiliary-involvement-in-systemic-sclerosis-and-the-cutaneous-subsets-characteristics-and-survival-of-patients-from-the-spanish-rescle-registry
#2
Begoña Marí-Alfonso, Carmen Pilar Simeón-Aznar, Alfredo Guillén-Del Castillo, Manuel Rubio-Rivas, Luis Trapiella-Martínez, José Antonio Todolí-Parra, Mónica Rodríguez Carballeira, Adela Marín-Ballvé, Nerea Iniesta-Arandia, Dolores Colunga-Argüelles, María Jesús Castillo-Palma, Luis Sáez-Comet, María Victoria Egurbide-Arberas, Norberto Ortego-Centeno, Mayka Freire, José Antonio Vargas Hitos, Antonio-J Chamorro, Ana Belen Madroñero-Vuelta, Isabel Perales-Fraile, Xavier Pla-Salas, Rafael A Fernández-De-La-Puebla, Vicent Fonollosa-Pla, Carles Tolosa-Vilella
OBJECTIVE: To assess the prevalence and causes of hepatobiliary involvement (HBI) in systemic sclerosis (SSc), to investigate the clinical characteristics and prognosis of SSc patients with HBI (SSc-HBI) and without HBI (SSc-non-HBI), and to compare both groups according to the cutaneous SSc subsets. METHODS: In all, 1572 SSc patients were collected in the RESCLE registry up to January 2015, and all hepatobiliary disturbances were recorded. We investigated the HBI-related characteristics and survival from the entire SSc cohort and according to the following cutaneous subsets: diffuse cutaneous SSc (dcSSc), limited cutaneous SSc (lcSSc), and SSc sine scleroderma (ssSSc)...
October 6, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/29156507/liver-transplantation-in-adults-with-liver-disease-due-to-common-variable-immunodeficiency-leads-to-early-recurrent-disease-and-poor-outcome
#3
Vian Azzu, Joshua E Elias, Adam Duckworth, Susan Davies, Rebecca Brais, Dinakantha S Kumararatne, Alexander E S Gimson, William J H Griffiths
Common variable immunodeficiency (CVID) is the commonest from of primary immunodeficiency characterized by antibody deficiency, recurrent bacterial infections and autoimmunity. Advanced chronic liver disease occurs in a subset of patients with CVID and manifests with various histological features such as nodular regenerative hyperplasia, inflammation, fibrosis and cholangiopathy. We present a case series characterizing the outcomes in adult patients transplanted for primary CVID-related liver disease. We discuss the unique transplantation challenges faced in this primary immunodeficiency group including susceptibility to infections and early disease recurrence...
November 20, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/29131270/imaging-assessment-of-portal-venous-system-pictorial-essay-of-normal-anatomy-anatomic-variants-and-congenital-anomalies
#4
A Guerra, A M De Gaetano, A Infante, C Mele, M G Marini, E Rinninella, R Inchingolo, L Bonomo
The purpose of this pictorial essay is to describe anatomic variants and congenital anomalies of portal venous system and related liver parenchymal alterations. The imaging findings of some of these entities have been previously described in other articles, however this work encompasses all congenital anomalies of portal venous system with attention to their features on various imaging modalities; in particular we illustrated with detailed pictures all the main portal vein variants, congenital extra- and intra-hepatic porto-systemic venous shunts and portal vein aneurysm...
October 2017: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/29119002/mechanisms-of-liver-disease-in-patients-infected-with-hiv
#5
REVIEW
Matthew B Kaspar, Richard K Sterling
Objective: To describe the various mechanisms of liver disease in patients with HIV infection, and to link these mechanisms to disease states which may utilise them. Background: Non-AIDS causes of morbidity and mortality are becoming increasingly common in patients chronically infected with HIV. In particular, liver-related diseases have risen to become one of the leading causes of non-AIDS-related death. A thorough understanding of the mechanisms driving the development of liver disease in these patients is essential when evaluating and caring for these patients...
2017: BMJ Open Gastroenterology
https://www.readbyqxmd.com/read/29093156/nodular-regenerative-hyperplasia-of-the-liver-a-rare-vascular-complication-in-systemic-sclerosis
#6
Laura Graf, Rucsandra Dobrota, Suzana Jordan, Lukas Martin Wildi, Oliver Distler, Britta Maurer
OBJECTIVE: To investigate nodular regenerative hyperplasia (NRH) as a vascular complication of systemic sclerosis (SSc) with microvasculopathy as a common denominator. METHODS: Cases of SSc-NRH were identified by systematic literature review and by screening the Zurich cohort. NRH had to be diagnosed by liver biopsy. RESULTS: Literature review retrieved 22 cases. In our cohort, 1.4% of patients with SSc were diagnosed with NRH. Most had vasculopathy, were positive for anticentromere antibodies, had elevated alkaline phosphatase and gamma-glutamyl transferase levels, normal liver morphology on ultrasound yet increased stiffness on ultrasound elastography, and had portal hypertension...
November 1, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28987257/the-liver-in-oncology
#7
REVIEW
Renu Dhanasekaran, Paul Y Kwo
Gastroenterologists and hepatologists will encounter oncology patients who develop abnormal liver tests, patients with hepatic malignancies, and patients with acute and chronic liver disease who require chemotherapy or immediate evaluation. Chemotherapy can cause liver injury owing to toxic effects or idiosyncratic reactions. Immune checkpoint inhibitors may be associated with autoimmune-mediated liver toxicities. Venoocclusive disease requires immediate evaluation. Nodular regenerative hyperplasia is a chronic progressive disorder...
November 2017: Clinics in Liver Disease
https://www.readbyqxmd.com/read/28922441/evaluation-of-2d-shear-wave-elastography-for-characterisation-of-focal-liver-lesions
#8
Ludmila Gerber, Daniel Fitting, Kajana Srikantharajah, Nina Weiler, Georgia Kyriakidou, Joerg Bojunga, Falko Schulze, Dimitra Bon, Stefan Zeuzem, Mireen Friedrich-Rust
BACKGROUND AND AIM: This is a prospective study for evaluation of 2D-shear wave elastography (2D-SWE) for characterisation and differentiation of benign und malignant focal liver lesions (FLLs). METHODS: The patients referred to our ultrasound unit were prospectively included. B-mode ultrasound and 2D-SWE (Aixplorer® France) were performed for one FLL in each patient. Liver histology and/or contrast-enhanced imaging were used as a reference method. RESULTS: 140 patients with FLL were included...
September 2017: Journal of Gastrointestinal and Liver Diseases: JGLD
https://www.readbyqxmd.com/read/28834232/critical-assessment-of-thioguanine-treatment-for-inflammatory-bowel-diseases-is-it-time-to-rehabilitate-this-treatment
#9
Ramya Movva, Alison Haywood, Sohil A Khan, Timothy Hj Florin, Iulia Oancea
OBJECTIVE: The potential therapeutic effect of thioguanine in the management of inflammatory bowel disease (IBD) is hindered due to association with vascular hepatotoxicity. The study aimed to assess the evidence for efficacy of thioguanine in IBD management and the association with nodular regenerative hyperplasia (NRH) and other thioguanine-related hepatotoxicities. METHODS: Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines were used for literature search...
September 2017: Journal of Digestive Diseases
https://www.readbyqxmd.com/read/28815029/nodular-regenerative-hyperplasia-rarely-leads-to-liver-transplantation-a-20-year-cohort-study-in-all-dutch-liver-transplant-units
#10
Berrie Meijer, Melek Simsek, Hans Blokzijl, Robert A de Man, Minneke J Coenraad, Gerard Dijkstra, Carin Mj van Nieuwkerk, Chris Jj Mulder, Nanne Kh de Boer
BACKGROUND: Nodular regenerative hyperplasia is an uncommon liver condition associated with several autoimmune disorders and drugs. The clinical symptoms of nodular regenerative hyperplasia vary from asymptomatic to severe complications of portal hypertension (nodular regenerative hyperplasia-syndrome). OBJECTIVE: The purpose of this study was to identify the prognosis and optimal management, as well as the role of liver transplantation, in nodular regenerative hyperplasia...
August 2017: United European Gastroenterology Journal
https://www.readbyqxmd.com/read/28727971/congenital-extrahepatic-portosystemic-shunt-abernethy-malformation-type-ib-with-associated-hepatocellular-carcinoma-case-report-and-literature-review
#11
Mark Benedict, Manuel Rodriguez-Davalos, Sukru Emre, Zenta Walther, Raffaella Morotti
Abernethy malformation, also termed congenital portosystemic shunt and congenital absence of portal vein is the result of malformation of the splanchnic venous system. Congenital portosystemic shunts are divided into extra- and intrahepatic shunts. Two shunts have been defined: Type I is characterized by the complete diversion of portal blood into the vena cava with an associated congenital absence of the portal vein. Type II is defined by an intact but diverted portal vein through a side-to-side, extrahepatic connection to the vena cava...
July 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28626210/clinical-correspondence-to-hepatocellular-carcinoma-related-lesions-with-atypical-radiological-pattern
#12
Tokio Higaki, Yutaka Midorikawa, Yosuke Nakashima, Hisashi Nakayama, Shunichi Matsuoka, Mitsuhiko Moriyama, Masahiko Sugitani, Tadatoshi Takayama
In patients at risk of hepatocarcinogenesis, tumors are frequently detected with atypical radiological patterns related to hepatocellular carcinoma (HCC) on imaging studies. Despite their high potential for malignancy, whether to resect such lesions immediately is controversial. Based on histological findings, patients with non-enhanced tumors or enhanced tumors without washout were divided into two groups: those with tumors that should be treated containing well, moderately, and poorly differentiated HCC (Group 1), and those that can be observed containing early HCC, hepatocellular adenoma, focal nodular hyperplasia, dysplastic nodules, and regenerative nodules (Group 2), and we elucidated the clinical correspondence to these tumors...
June 18, 2017: Bioscience Trends
https://www.readbyqxmd.com/read/28614198/the-almost-normal-liver-biopsy-presentation-clinical-associations-and-outcome
#13
MULTICENTER STUDY
Thomas W Czeczok, John S Van Arnam, Laura D Wood, Michael S Torbenson, Taofic Mounajjed
Liver biopsies obtained for abnormal liver enzymes or unexplained ascites occasionally appear histologically almost normal. The differential diagnosis for these cases is challenging because literature addressing this topic is lacking. We aimed to establish a differential diagnosis and determine clinical associations and outcomes for almost-normal liver biopsies. Ninety-seven histologically almost-normal liver biopsies were collected from 2 institutions. All cases lacked significant inflammation, fatty change, biliary tract disease, vascular disease, nodular regenerative hyperplasia, iron overload, inherited metabolic or storage disorder, viral hepatitis, or fibrosis...
September 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28588888/thioguanine-in-inflammatory-bowel-disease-long-term-efficacy-and-safety
#14
Mark G Ward, Kamal V Patel, Viraj C Kariyawasam, Rishi Goel, Ben Warner, Tim R Elliott, Paul A Blaker, Peter M Irving, Anthony M Marinaki, Jeremy D Sanderson
BACKGROUND: Thioguanine (TG) is efficacious in inflammatory bowel disease (IBD), but its toxicity, particularly nodular regenerative hyperplasia (NRH) of the liver, has limited its use. We assessed the long-term clinical outcomes and safety of TG in patients whom were intolerant or refractory to conventional immunomodulators. METHODS: This is a retrospective, single-centre study of IBD patients treated with TG from 2001-2013. Response was defined as clinical remission (Harvey-Bradshaw Index < 5 for Crohn's disease (CD), Simple Clinical Colitis Activity Index < 4 for ulcerative colitis (UC)) without corticosteroids or, if receiving anti-tumour-necrosis-factor (anti-TNF) therapy, absence of dose escalation...
June 2017: United European Gastroenterology Journal
https://www.readbyqxmd.com/read/28491877/nodular-regenerative-hyperplasia-a-case-of-rare-prognosis
#15
Mindy Lee, Manhal Izzy, Ashwin Akki, Kathryn Tanaka, Harmit Kalia
Introduction: Nodular regenerative hyperplasia (NRH) is a known etiology of noncirrhotic portal hypertension. Cases of biopsy-proven NRH in human immunodeficiency virus (HIV)-positive patients have been described. While these patients often have normal synthetic liver function, several reports described disease progression to liver failure. Case: We here present a 26-year-old woman with history of congenital HIV on antiretroviral therapy complicated by Pneumocystis carinii pneumonia at age 14. CD4 counts have been >300 with undetectable viral load...
January 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28489843/-non-cirrhotic-portal-hypertension-due-to-didanosina-a-rare-case
#16
María T Gamero, María Susana Gallardo, Víctor Aguilar, Eduar Bravo, Julissa Guevara, Fernando Mejia
Liver involvement is usually seen in patients infected with the human immunodeficiency virus (HIV), especially in patients coinfected with hepatitis B or C, in alcohol abuse, etc. However, there is a group of patients who develop liver involvement and portal hypertension of unspecified cause. Non-cirrhotic portal hypertension (NCPH) is a liver disorder recently described, but potentially serious. It has been reported in HIV-infected patients with highly active antiretroviral therapy (HAART), specifically didanosine (DDI)...
January 2017: Revista de Gastroenterología del Perú: órgano Oficial de la Sociedad de Gastroenterología del Perú
https://www.readbyqxmd.com/read/28465872/a-rare-cause-of-portal-hypertension-behcet-s-disease-and-nodular-regenerative-hyperplasia-of-the-liver
#17
Cyriac Philips, Rajaguru Paramaguru, Lijesh Kumar, Padmanabha Shenoy, Philip Augustine
Nodular regenerative hyperplasia (NRH) is a rare liver condition in which widespread benign transformation of the hepatic parenchyma into small regenerative nodules occur, leading to development of non-cirrhotic portal hypertension. Conditions associated with NRH include rheumatologic, hematological, autoimmune, infectious, neoplastic, or drug-related etiology. Accurate diagnosis is made on liver biopsy, showing diffuse micronodular transformation in the absence of fibrosis. Here, we present the second case in world literature of a middle-aged man presenting with recent onset sleep reversal and memory loss with darkening of skin for four years associated with systemic manifestations, in whom porto-systemic shunt syndrome due to NRH secondary to Behcet's disease was eventually diagnosed...
March 29, 2017: Curēus
https://www.readbyqxmd.com/read/28422310/adult-onset-cystic-fibrosis-liver-disease-diagnosis-and-characterization-of-an-underappreciated-entity
#18
COMPARATIVE STUDY
Christopher Koh, Sasan Sakiani, Pallavi Surana, Xiongce Zhao, Jason Eccleston, David E Kleiner, David Herion, T Jake Liang, Jay H Hoofnagle, Milica Chernick, Theo Heller
Cystic fibrosis (CF) liver disease (CFLD), a leading cause of death in CF, is mostly described in pediatric populations. Adult-onset CFLD lacks sufficient characterization and diagnostic tools. A cohort of CF patients without CFLD during childhood were followed for up to 38 years with serologic testing, imaging, and noninvasive fibrosis markers. Historical CFLD diagnostic criteria were compared with newly proposed CFLD criteria. Thirty-six CF patients were followed for a median of 24.5 years (interquartile range 15...
August 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28302006/transplantation-for-large-liver-tumors-in-the-setting-of-abernethy-malformation
#19
İlgin Özden, Ayşen Yavru, Mine Güllüoğlu, Aydin Alper, Orhan Bilge, Serdar Cantez, Özlem Durmaz
In this study, we report our experiences on the role of transplantation in 2 patients with large liver tumors in the setting of Abernethy malformation. Patient 1 was a 17-year-old boy who was referred for hepatic masses and recurrent hepatic encephalopathy episodes. Computed tomography and magnetic resonance imaging showed 2 large tumors (4 and 8 cm) in the liver. The portal vein drained directly into the vena cava. Core biopsy of the larger mass revealed fibrosis and regenerative hyperplasia. There were hyperintense signals in the T1-weighted images in the globus pallidus...
March 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28284915/reversibility-of-chemotherapy-related-liver-injury
#20
Luca Vigano, Giovanni De Rosa, Christian Toso, Axel Andres, Alessandro Ferrero, Arnaud Roth, Elisa Sperti, Pietro Majno, Laura Rubbia-Brandt
BACKGROUND & AIMS: Chemotherapy-associated liver injury (CALI) increases the risk of liver resection and may prejudice further surgery and chemotherapy. The reversibility of CALI is therefore important; however, no data concerning this are available. This study aimed to retrospectively analyze the reversibility of CALI in patients undergoing liver resection for colorectal metastases. METHODS: All resections of colorectal liver metastases after oxaliplatin and/or irinotecan-based chemotherapy were included...
July 2017: Journal of Hepatology
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