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Nodular regenerative hyperplasia

N Vicente, M Pérez, R Gander, A Segarra, C Leganés, J Bueno
AIM: Congenital portosistemic shunt (CPSS) is an uncommon condition that can cause serious complications such as encephalopathy and liver tumors at risk of malignant degeneration. Occlusion of the shunt by surgery or interventional radiology can prevent and even improve such complications. In some cases, liver transplantation is the only curative option. We describe our experience with this condition. PATIENTS AND METHODS: Between 1992 and 2013, eight children (four male and four female) were diagnosed with CPSS (four extrahepatic and four intrahepatic) in our center, of which seven were diagnosed after 2007...
January 15, 2016: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
Norihide Yoneda, Osamu Matsui, Azusa Kitao, Kazuto Kozaka, Satoshi Kobayashi, Motoko Sasaki, Kotaro Yoshida, Dai Inoue, Tetsuya Minami, Toshifumi Gabata
Gadoxetic acid is a contrast agent for magnetic resonance (MR) imaging with hepatocyte-specific properties and is becoming increasingly important in detection and characterization of hepatocellular carcinoma and benign hepatocellular nodules, including focal nodular hyperplasia (FNH), nodular regenerative hyperplasia (NRH), hepatocellular adenoma (HCA), and dysplastic nodule. In these hepatocellular nodules, a positive correlation between the grade of membranous uptake transporter organic anion-transporting polypeptide (OATP) 1B3 expression and signal intensity in the hepatobiliary (HB) phase has been verified...
October 14, 2016: Radiographics: a Review Publication of the Radiological Society of North America, Inc
Oliviero Riggio, Stefania Gioia, Ilaria Pentassuglio, Valeria Nicoletti, Michele Valente, Giulia d'Amati
The term idiopathic noncirrhotic portal hypertension (INCPH) has been recently proposed to replace terms, such as hepatoportal sclerosis, idiopathic portal hypertension, incomplete septal cirrhosis, and nodular regenerative hyperplasia, used to describe patients with a hepatic presinusoidal cause of portal hypertension of unknown etiology, characterized by features of portal hypertension (esophageal varices, nonmalignant ascites, porto-venous collaterals), splenomegaly, patent portal, and hepatic veins and no clinical and histological signs of cirrhosis...
2016: Hepatic Medicine: Evidence and Research
Adeline Furudoï, Audrey Gros, Sophie Stanislas, Mohamed Hamidou, Enio Furudoï, Éric Oksenhendler, Jean-Philippe Merlio, Jean-François Viallard, Marie Parrens
Histologic and phenotypic analyses of splenectomy samples from 17 patients with common variable immunodeficiency (CVID) showed the following nonspecific, evocative, white-pulp lesions: white-pulp hyperplasia (WPH) with reactive follicles, giant follicles (GFs), marginal zone hyperplasia, periarteriolar T-zone hyperplasia (PATH) and/or granulomas, which enabled us to discern 2 groups: the first (n=6) composed of WPH with reactive follicles without granulomas, and the second (n=9) characterized by the presence of granulomas with or without WPH...
July 2016: American Journal of Surgical Pathology
Shunsuke Hayano, Atsushi Naganuma, Yudai Okano, Yuhei Suzuki, Keisuke Shiina, Haruka Yoshida, Eri Hayashi, Sanae Uehara, Takashi Hoshino, Naomi Miyamae, Tomohiro Kudo, Hiroshi Ishihara, Akira Ogawa, Ken Sato, Satoru Kakizaki
A 51-year-old woman was diagnosed with mixed connective tissue disease (MCTD) in 2011. She underwent treatment with prednisolone. Her hepatobiliary enzyme level increased, and multiple nodules were found in both liver lobes in abdominal imaging studies. Ultrasonography revealed large and small hyperechoic lesions with indistinct or well-defined borders. No findings of classic hepatocellular carcinoma or liver cirrhosis were observed on contrast-enhanced computed tomography, but some nodules showed an enhanced effect of the central lesion that was characteristic of focal nodular hyperplasia (FNH) in an arterial phase...
May 2016: Nihon Shokakibyo Gakkai Zasshi, the Japanese Journal of Gastro-enterology
Mark Benedict, Manuel Rodriguez-Davalos, Sukru Emre, Zenta Walther, Raffaella A Morotti
Abernethy malformation, also termed congenital portosystemic shunt and congenital absence of portal vein (CAPV) 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...
April 26, 2016: Pediatric and Developmental Pathology
Ioanna Delladetsima, Stratigoula Sakellariou, Aikaterini Kokkori, Dina Tiniakos
AIM: To investigate atrophic parenchymal changes in ischemic liver conditions. DESIGN: We studied 18 cases of hepatic lesions with atrophic changes due to altered blood flow (hepatic venous congestion n=15 including 4 cases with additional nodular regenerative hyperplasia-NRH, NRH n=1, and antiphospholipid syndrome with patchy parenchymal atrophy n=2). Metaplastic hepatocellular changes, hepatocyte proliferation, hepatic stellate cell (HSC) activation, and sinusoidal capillarization were examined immunohistochemically with antibodies to keratins (K) 7 and 19, Ki67, αSMA and CD34, respectively...
October 2016: Histology and Histopathology
Dália Fernandes, Sónia Vilaça, Carla Rolanda
No abstract text is available yet for this article.
March 2016: Gastroenterology
Vanessa Szablewski, Céline René, Valérie Costes
Patients with common variable immunodeficiency disorder (CVID) are subject to lymphoproliferative disorders and predisposed to lymphoma. Some patients may also develop liver lesions. The purpose of this study was to define clinical and histopathological features of patients with CVID presenting with liver lesions suspicious of lymphoma. Four CVID cases corresponding to these criteria were retrieved from our files. Liver biopsy specimens were subjected to morphologic, immunophenotypic and molecular analysis...
October 22, 2015: Virchows Archiv: An International Journal of Pathology
Timothy B Lautz, Sonam A Shah, Riccardo A Superina
Two children developed hepatoblastoma concurrent with congenital portosystemic shunts (PSSs) (Abernethy malformations). Both underwent operative ligation of their PSSs. One received concurrent tumor resection, whereas the other was deemed initially unresectable and underwent biopsy followed by neoadjuvant chemotherapy. Although benign hepatic masses, such as focal nodular hyperplasia and nodular regenerative hyperplasia, are common in patients with Abernethy malformations, malignant tumors have also been documented and should always be considered in the differential diagnosis of a patient with a congenital PSS and a hepatic mass...
April 2016: Journal of Pediatric Gastroenterology and Nutrition
Jonathan G Stine, Naga Chalasani
To examine the available literature and summarize what is known about chronic drug-induced liver injury. We reviewed PubMed/MEDLINE through March 2015. We developed a MEDLINE search strategy using PubMed medical subject heading terms chronic liver injury, hepatotoxicity, drug-induced liver injury, cirrhosis and chronic liver disease. We reviewed the reference list of included articles to identify articles missed in the database search. Chronic liver injury from drugs is more common than once thought with prevalence as high as 18% based on large national registries...
November 2015: Liver International: Official Journal of the International Association for the Study of the Liver
Majed Almotairi, Kamaldine Oudjhane, Govind B Chavhan
OBJECTIVE: The purpose of this study is to present our experience with MRI evaluation of multifocal liver lesions in children and describe the MRI characteristics of these lesions. PATIENTS AND METHODS: A retrospective review of consecutive MRI exams performed for the evaluation of multiple liver lesions between 1 January 2007 and 31 December 2012 was done to note the number of lesions, the size of the largest lesion, MR signal characteristics, and background liver...
July 2015: Indian Journal of Radiology & Imaging
Maria Guido, Samantha Sarcognato, Aurelio Sonzogni, Maria Grazia Lucà, Marco Senzolo, Stefano Fagiuoli, Alberto Ferrarese, Marco Pizzi, Luciano Giacomelli, Guido Colloredo
BACKGROUND & AIMS: Obliterative portal venopathy without portal hypertension has been described by a single study in a limited number of patients, thus very little is known about this clinical condition. This study aimed to investigate the prevalence of obliterative portal venopathy and its clinical-pathological correlations in patients with cryptogenic chronic liver test abnormalities without clinical signs of portal hypertension. METHODS: We analysed 482 liver biopsies from adults with non-cirrhotic cryptogenic chronic liver disorders and without any clinical signs of portal hypertension, consecutively enrolled in a 5-year period...
March 2016: Liver International: Official Journal of the International Association for the Study of the Liver
Sanket Srinivasa, Wai G Lee, Ali Aldameh, Jonathan B Koea
BACKGROUND: A spontaneous hepatic haemorrhage (SHH) is a rare condition that presents acutely to both hepatobiliary and general surgeons. Management of the condition is challenging because of the emergent presentation requiring immediate intervention, the presence of underlying chronic liver disease and the multiple potential underlying aetiological conditions. METHODS: A literature search on a spontaneous hepatic haemorrhage was instituted on Medline (1966-2014), Cochrane Register of Controlled Trials, EMBASE (1947-2014), PubMed, Web of Science and Google Scholar...
October 2015: HPB: the Official Journal of the International Hepato Pancreato Biliary Association
Liliana Chiorean, Xin-Wu Cui, Andrea Tannapfel, Doris Franke, Martin Stenzel, Wojciech Kosiak, Dagmar Schreiber-Dietrich, Jörg Jüngert, Jian-Min Chang, Christoph F Dietrich
Benign hepatic tumors are commonly observed in adults, but rarely reported in children. The reasons for this remain speculative and the exact data concerning the incidence of these lesions are lacking. Benign hepatic tumors represent a diverse group of epithelial and mesenchymal tumors. In pediatric patients, most benign focal liver lesions are inborn and may grow like the rest of the body. Knowledge of pediatric liver diseases and their imaging appearances is essential in order to make an appropriate differential diagnosis...
July 28, 2015: World Journal of Gastroenterology: WJG
Simona Gurzu, Ioan Jung, Anca Otilia Contac, Mihai Turcu, Adrian Tudor
Atypical hepatocellular neoplasm (AHN) is an adenoma-like hepatic tumor that even occurs in noncirrhotic liver of males (any age) or females ≥ 50 years old, or associates focal atypical features. In this article, 2 unusual cases diagnosed in elderly cirrhotic patients, unrelated to steroids, are presented. The first case was incidentally diagnosed in an 83-year-old female. During laparoscopic surgery for cholecystectomy, hemoperitoneum was installed and laparotomy was necessary to remove a 70-mm nodular encapsulated hepatic tumor that was microscopically composed by hepatocyte-like cells with clear cytoplasm, arranged in 1- to 2-cell-thick plates and intermingled with areas of peliosis, negative for alpha fetoprotein (αFP), p53, and keratin 7, with low Ki67 index and intact reticulin framework...
July 2015: Medicine (Baltimore)
Amany I Gorgy, Naudia L Jonassaint, Susan E Stanley, Ayman Koteish, Amy E DeZern, Jolan E Walter, Sabrina C Sopha, James P Hamilton, Julie Hoover-Fong, Allen R Chen, Robert A Anders, Ihab R Kamel, Mary Armanios
BACKGROUND: Telomere syndromes have their most common manifestation in idiopathic pulmonary fibrosis and emphysema. The short telomere defect in these patients may manifest systemically as bone marrow failure and liver disease. We sought to understand the causes of dyspnea in telomerase and telomere gene mutation carriers who have no parenchymal lung disease. METHODS: Clinical and pathologic data were reviewed as part of a Johns Hopkins-based natural history study of short telomere syndromes including dyskeratosis congenita...
October 2015: Chest
Makiko Yasuda, Angelika L Erwin, Lawrence U Liu, Manisha Balwani, Brenden Chen, Senkottuvelan Kadirvel, Lin Gan, M Isabel Fiel, Ronald E Gordon, Chunli Yu, Sonia Clavero, Antonios Arvelakis, Hetanshi Naik, L David Martin, John D Phillips, Karl E Anderson, Vaithamanithi M Sadagoparamanujam, Sander S Florman, Robert J Desnick
Acute intermittent porphyria (AIP) is an autosomal-dominant hepatic disorder caused by the half-normal activity of hydroxymethylbilane (HMB) synthase. Symptomatic individuals experience life-threatening acute neurovisceral attacks that are precipitated by factors that induce the hepatic expression of 5-aminolevulinic acid synthase 1 (ALAS1), resulting in the marked accumulation of the putative neurotoxic porphyrin precursors 5-aminolevulinic acid (ALA) and porphobilinogen (PBG). Here, we provide the first detailed description of the biochemical and pathologic alterations in the explanted liver of an AIP patient who underwent orthotopic liver transplantation (OLT) due to untreatable and debilitating chronic attacks...
2015: Molecular Medicine
Bindia Jharap, Dirk P van Asseldonk, Nanne K H de Boer, Pierre Bedossa, Joachim Diebold, A Mieke Jonker, Emmanuelle Leteurtre, Joanne Verheij, Dominique Wendum, Fritz Wrba, Pieter E Zondervan, Jean-Frédéric Colombel, Walter Reinisch, Chris J J Mulder, Elisabeth Bloemena, Adriaan A van Bodegraven
BACKGROUND AND AIMS: Nodular regenerative hyperplasia (NRH) of the liver is associated with several diseases and drugs. Clinical symptoms of NRH may vary from absence of symptoms to full-blown (non-cirrhotic) portal hypertension. However, diagnosing NRH is challenging. The objective of this study was to determine inter- and intraobserver agreement on the histopathologic diagnosis of NRH. METHODS: Liver specimens (n=48) previously diagnosed as NRH, were reviewed for the presence of NRH by seven pathologists without prior knowledge of the original diagnosis or clinical background...
2015: PloS One
Juan Putra, Arifa Toor, Todd A Noce, Swan N Thung, Arief A Suriawinata, Mikhail Lisovsky
Focal nodular hyperplasia and nodular regenerative hyperplasia are occasionally seen in patients with hepatic venous outflow obstruction as a consequence of circulatory stress in the liver. In addition, neoplastic processes such as hepatic adenoma, hepatocellular carcinoma, and metastatic disease may arise in these patients. Histologic evaluation is necessary when imaging modalities are unable to distinguish these lesions. We present a case of multiple hepatic lesions, suspicious for metastases, in a patient with Budd-Chiari syndrome secondary to polycythemia vera...
July 2015: Annals of Hepatology
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