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non cirrhotic portal hypertension

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https://www.readbyqxmd.com/read/29761167/phenotypic-spectrum-and-diagnostic-pitfalls-of-abcb4-deficiency-depending-on-age-of-onset
#1
Stephanie Barbara Schatz, Christoph Jüngst, Verena Keitel-Anselmo, Ralf Kubitz, Christina Becker, Patrick Gerner, Eva-Doreen Pfister, Imeke Goldschmidt, Norman Junge, Daniel Wenning, Stephan Gehring, Stefan Arens, Dirk Bretschneider, Dirk Grothues, Guido Engelmann, Frank Lammert, Ulrich Baumann
Genetic variants in the adenosine triphosphate-binding cassette subfamily B member 4 ( ABCB4 ) gene, which encodes hepatocanalicular phosphatidylcholine floppase, can lead to different phenotypes, such as progressive familial intrahepatic cholestasis (PFIC) type 3, low phospholipid-associated cholelithiasis, and intrahepatic cholestasis of pregnancy. The aim of this multicenter project was to collect information on onset and progression of this entity in different age groups and to assess the relevance of this disease for the differential diagnosis of chronic liver disease...
May 2018: Hepatology Communications
https://www.readbyqxmd.com/read/29651703/spectrum-of-histopathological-changes-in-patients-with-non-cirrhotic-portal-fibrosis
#2
Abhijit Chougule, Archana Rastogi, Rakhi Maiwall, Chhagan Bihari, Vikrant Sood, Shiv Kumar Sarin
BACKGROUND AND AIMS: Non-cirrhotic portal fibrosis (NCPF) is a clinical disorder characterized by features of portal hypertension in the absence of significant fibrosis. It is one of the commonest causes of portal hypertension in India. This study aimed to analyze histomorphological spectrum of NCPF in detail. METHODS AND RESULTS: There were 67 specimens from 66 patients which included 43 (65.2%) male and 23 (34.8%) female patients with a mean age of 31 years (range: 7-61 years)...
April 12, 2018: Hepatology International
https://www.readbyqxmd.com/read/29644531/splanchnic-vein-thrombosis-in-the-myeloproliferative-neoplasms
#3
REVIEW
Imo J Akpan, Brady Lee Stein
PURPOSE OF REVIEW: To review the epidemiology, diagnostic challenges, pathogenesis, and treatment strategies for patients with myeloproliferative neoplasm-associated splanchnic vein thrombosis. RECENT FINDINGS: The epidemiology of myeloproliferative neoplasm-associated splanchnic vein thrombosis (MPN-SVT) has been well characterized. While typical MPN-associated thrombosis affects older patients and involves the arterial circulation, MPN-SVT mostly impacts younger women...
April 11, 2018: Current Hematologic Malignancy Reports
https://www.readbyqxmd.com/read/29644430/pathology-of-idiopathic-non-cirrhotic-portal-hypertension
#4
REVIEW
Maria Guido, Samantha Sarcognato, Diana Sacchi, Guido Colloredo
Idiopathic non-cirrhotic portal hypertension is an under-recognized vascular liver disease of unknown etiology, characterized by clinical signs of portal hypertension in the absence of cirrhosis. By definition, any disorder known to cause portal hypertension in the absence of cirrhosis and any cause of chronic liver disease must be excluded to make a diagnosis of idiopathic non-cirrhotic portal hypertension. However, the diagnosis is often difficult because the disease resembles cirrhosis and there is no gold standard test...
April 12, 2018: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/29609064/massive-epitheloid-hemangioendothelioma-causing-non-cirrhotic-portal-hypertension
#5
Amir Taefi, Karen Matsukuma, Eric Chak
No abstract text is available yet for this article.
March 30, 2018: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/29564495/non-cirrhotic-portal-hypertension-an-imaging-review
#6
S Rajesh, Amar Mukund, Binit Sureka, Kalpana Bansal, Maxime Ronot, Ankur Arora
Non-cirrhotic portal hypertension (NCPH) comprises a group of diseases that are characterized by increased portal pressure in the absence of cirrhosis of the liver. It may arise as a result of either primary hepatic disease or the effects of systemic disorders on liver. The natural course of NCPH is largely indolent and benign with an overall better outcome than cirrhosis, as long as the complications of portal hypertension are properly managed and regular surveillance is carried out. However, due to the numerous and variable etiological factors and lack of standardized diagnostic criteria, this entity is under-recognized clinically as well as pathologically and often gets mislabeled as cryptogenic cirrhosis...
March 21, 2018: Abdominal Radiology
https://www.readbyqxmd.com/read/29516016/portal-cavernoma-mimicking-pancreatic-malignancy
#7
Vivian M Ussui, Lavinia Goldstein, Enrico Souto, Cynthia Levy
Portal cavernoma colangiopathy (PCC) is an uncommon cause of portal hypertension, and it is an important differential diagnosis of pancreatic malignancy given the expanded network of collateral vessels. On imaging studies, portal cavernoma can be seen as a hypoechoic mass, possibly associated with distal common bile duct obstruction. Most cases occur in non-cirrhotic patients. During the symptomatic phase, these patients carry a high-risk of complications related to sustained biliary obstruction. We report a unique patient with obstructive jaundice and a presumed pancreatic mass that proved to be a portal cavernoma complicated by PCC in the setting of nodular regenerative hyperplasia of the liver...
2018: ACG Case Reports Journal
https://www.readbyqxmd.com/read/29507470/propranolol-reduces-systemic-oxidative-stress-and-endotoxemia-in-cirrhotic-patients-with-esophageal-varices
#8
Dimitra Taprantzi, Dimitrios Zisimopoulos, Konstantinos C Thomopoulos, Iris Spiliopoulou, Christos D Georgiou, Georgios Tsiaoussis, Christos Triantos, Charalambos A Gogos, Chrisoula Labropoulou-Karatza, Stelios F Assimakopoulos
Background: The aim of the study was to investigate the effect of propranolol on systemic oxidative stress and endotoxemia in patients with liver cirrhosis and clinically significant portal hypertension evidenced by the presence of esophageal varices. Methods: Fourteen patients with liver cirrhosis and esophageal varices, not previously been treated with non-selective beta-blockers (NSBB), were prospectively started on propranolol and followed up for three months...
March 2018: Annals of Gastroenterology: Quarterly Publication of the Hellenic Society of Gastroenterology
https://www.readbyqxmd.com/read/29464506/idiopathic-portal-hypertension-and-extrahepatic-portal-venous-obstruction
#9
REVIEW
Rajeev Khanna, Shiv Kumar Sarin
BACKGROUND: Idiopathic portal hypertension (IPH) and extrahepatic portal venous obstruction (EHPVO) are non-cirrhotic vascular causes of portal hypertension (PHT). Variceal bleed and splenomegaly are the commonest presentations. AIM: The present review is intended to provide the existing literature on etiopathogenesis, clinical profile, diagnosis, natural history and management of IPH and EHPVO. RESULTS: IPH and EHPVO are both characterized by normal hepatic venous pressure gradient, moderate to massive splenomegaly with preserved liver synthetic functions...
February 2018: Hepatology International
https://www.readbyqxmd.com/read/29456411/clinical-utility-of-hepatitis-b-surface-antigen-kinetics-in-treatment-na%C3%A3-ve-chronic-hepatitis-b-patients-during-long-term-entecavir-therapy
#10
Tien-Ching Lin, Yen-Cheng Chiu, Hung-Chih Chiu, Wen-Chun Liu, Pin-Nan Cheng, Chiung-Yu Chen, Ting-Tsung Chang, I-Chin Wu
AIM: To investigate the utility of hepatitis B surface antigen (HBsAg) kinetics in chronic hepatitis B patients during long-term entecavir treatment. METHODS: This retrospective study included treatment-naïve chronic hepatitis B patients who received at least 2 years of consecutive entecavir treatment. Patients were followed up at three to six month intervals with liver biochemistry, hepatitis B virus DNA, and abdominal sonography. In hepatitis B e antigen (HBeAg)-positive patients, HBeAg levels were assessed every three to six month until results became negative...
February 14, 2018: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/29429910/natural-history-of-patients-with-non-cirrhotic-portal-hypertension-comparison-with-patients-with-compensated-cirrhosis
#11
Stefania Gioia, Silvia Nardelli, Chiara Pasquale, Ilaria Pentassuglio, Valeria Nicoletti, Francesca Aprile, Manuela Merli, Oliviero Riggio
BACKGROUND: The knowledge of natural history of patients with portal hypertension (PH) not due to cirrhosis is less well known than that of cirrhotic patients. AIM: To describe the clinical presentation and the outcomes of 89 patients with non-cirrhotic PH (25 with non-cirrhotic portal hypertension, INCPH, and 64 with chronic portal vein thrombosis, PVT) in comparison with 77 patients with Child A cirrhosis. METHODS: The patients were submitted to a standardized clinical, laboratory, ultrasonographic and endoscopic follow-up...
January 31, 2018: Digestive and Liver Disease
https://www.readbyqxmd.com/read/29399280/collagen-proportionate-area-correlates-to-hepatic-venous-pressure-gradient-in-non-abstinent-cirrhotic-patients-with-alcoholic-liver-disease
#12
Sophie Restellini, Nicolas Goossens, Sophie Clément, Nicolas Lanthier, Francesco Negro, Laura Rubbia-Brandt, Laurent Spahr
AIM: To explore the relationship between collagen proportionate area (CPA) and portal hypertension-related clinical manifestations in alcoholic liver disease (ALD). METHODS: Retrospective study with chart review of patients with ALD adressed to our center between January 2012 and December 2013 for a transjugular liver biopsy (TJLB) and hepatic hemodynamic study. Patients were included if they met the following criteria: (1) Medical indication for a liver biopsy in the setting of ALD; (2) recent (< 15 d) clinical, radiological, endoscopic and biological data available; and (3) estimated follow-up of at least 6 mo...
January 27, 2018: World Journal of Hepatology
https://www.readbyqxmd.com/read/29391766/beneficial-long-term-effect-of-a-phosphodiesterase-5-inhibitor-in-cirrhotic-portal-hypertension-a-case-report-with-8-years-follow-up
#13
Peter Deibert, Adhara Lazaro, Zoran Stankovic, Denise Schaffner, Martin Rössle, Wolfgang Kreisel
Non-selective beta-blockers are the mainstay of medical therapy for portal hypertension in liver cirrhosis. Inhibitors of phosphodiesterase-5 (PDE-5-inhibitors) reduce portal pressure in the acute setting by > 10% which may suggest a long-term beneficial effect. Currently, there is no available data on long-term treatment of portal hypertension with PDE-5-inhibitors. This case of a patient with liver cirrhosis secondary to autoimmune liver disease with episodes of bleeding from esophageal varices is the first documented case in which a treatment with a PDE-5-inhibitor for eight years was monitored...
January 21, 2018: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/29377486/liver-atrophy-and-regeneration-in-noncirrhotic-portal-vein-thrombosis-effect-of-surgical-shunts
#14
Abdulrhman S Elnaggar, Adam D Griesemer, Stuart Bentley-Hibbert, Robert S Brown, Mercedes Martinez, Steven J Lobritto, Tomoaki Kato, Jean C Emond
The goal of the study is to characterize the relationship between portal vein thrombosis (PVT) and hepatic atrophy in patients without cirrhosis and the effect of various types of surgical shunts on liver regeneration and splenomegaly. Patients without cirrhosis with PVT suffer from presinusoidal portal hypertension, and often hepatic atrophy is a topic that has received little attention. We hypothesized that patients with PVT have decreased liver volumes, and shunts that preserve intrahepatic portal flow enhance liver regeneration...
January 29, 2018: Liver Transplantation
https://www.readbyqxmd.com/read/29352618/point-shear-wave-elastography-of-the-spleen-its-role-in-patients-with-portal-hypertension
#15
Natalie Lucchina, Chiara Recaldini, Monica Macchi, Valeria Molinelli, Mario Montanari, Sergio Segato, Raffaele Novario, Carlo Fugazzola
The goals of the study described here were to evaluate the feasibility and reproducibility of measuring spleen stiffness (SS) using point shear wave elastography in a series of cirrhotic patients and to investigate whether SS, liver stiffness (LS) and other non-invasive parameters are correlated with the presence of esophagogastric varices (EVs). Fifty-four cirrhotic patients with low-grade EVs or without EVs at esophago-gastro-duodenoscopy were enrolled. They underwent abdominal ultrasound and Doppler ultrasound of hepatic vessels simultaneously with p-SWE of the liver and spleen...
April 2018: Ultrasound in Medicine & Biology
https://www.readbyqxmd.com/read/29282635/endoscopic-submucosal-dissection-of-early-gastric-cancer-in-patients-with-liver-cirrhosis
#16
Won Hyeok Choe, Jeong Hwan Kim, Jung Ho Park, Heung Up Kim, Dae Hyeon Cho, Sang Pyo Lee, Tae Yoon Lee, Sun-Young Lee, In Kyung Sung, Hyung Seok Park, Chan Sup Shim
BACKGROUND AND OBJECTIVES: Endoscopic submucosal dissection (ESD) has become widely accepted method for treating early gastric cancer (EGC), but little is known about its role in patients with liver cirrhosis (LC). The aims of this study were to evaluate the outcomes of ESD in treating EGC in patients with LC. METHODS: The multicenter retrospective study identified 43 patients with chronic hepatitis B (CHB)-related cirrhosis undergoing ESD for treating EGC. The patients (LC group) were subdivided into two groups based on their Child-Pugh classification (CP-class): 32 with CP-class A (LC-A) and 11 with CP-class B (LC-B)...
February 2018: Digestive Diseases and Sciences
https://www.readbyqxmd.com/read/29218665/benefits-of-laparoscopic-liver-resection-in-patients-with-hepatocellular-carcinoma-and-portal-hypertension-a-case-matched-study
#17
Víctor Molina, Jaime Sampson-Dávila, Joana Ferrer, Constantino Fondevila, Rafael Díaz Del Gobbo, David Calatayud, Jordi Bruix, Juan Carlos García-Valdecasas, Josep Fuster
BACKGROUND: The advantages of laparoscopy over open liver resection in patients with cirrhosis have been widely demonstrated. On the other hand, information on the role of minimally invasive liver surgery in the presence of clinically significant portal hypertension (CSPH) is scarce. The aim of this study was to evaluate the role of laparoscopic liver resection in selected cirrhotic patients with CSPH. METHODS: A retrospective case-control study of cirrhotic patients with hepatocellular carcinoma who were treated with laparoscopic liver resection was conducted from December 2005 to April 2016...
May 2018: Surgical Endoscopy
https://www.readbyqxmd.com/read/29183424/surgery-for-portal-hypertension-in-children-a-12-year-review
#18
N Patel, A Grieve, J Hiddema, J Botha, J Loveland
BACKGROUND: Portal hypertension is a common and potentially devastating condition in children. Notwithstanding advances in the nonsurgical management of portal hypertension, surgery remains an important treatment modality in select patients. We report here on our experience in the past 12 years. OBJECTIVES: To describe the profile of, indication for, and complications of shunt surgery in children with portal hypertension. METHODS: Twelve children underwent shunt surgery between 2005 and 2017...
November 6, 2017: South African Medical Journal, Suid-Afrikaanse Tydskrif Vir Geneeskunde
https://www.readbyqxmd.com/read/29145760/non-cirrhotic-portal-hypertension-associated-with-multicentric-castleman-s-disease-a-case-report
#19
Ana Luísa Pinto, Marília Gomes, Maria Augusta Cipriano, Maria Letícia Ribeiro
No abstract text is available yet for this article.
November 17, 2017: Acta Oncologica
https://www.readbyqxmd.com/read/29124669/shunt-occlusion-for-portosystemic-shunt-syndrome-related-refractory-hepatic-encephalopathy-a-single-center-experience-in-21-patients-from-kerala
#20
Cyriac Abby Philips, Lijesh Kumar, Philip Augustine
INTRODUCTION: Large spontaneous portosystemic shunts (SPSS) are seen in a subset of patients with liver disease and medically refractory recurrent/persistent hepatic encephalopathy (MRHE). Shunt occlusion has been shown to improve clinical outcomes. METHODS: We retrospectively analyzed patient characteristics, SPSS attributes, procedural features, baseline clinical and investigational parameters, neurological outcomes, adverse effects (procedure and portal hypertension related), and risk factors predicting outcomes in liver disease patients undergoing shunt occlusion procedure for MRHE...
September 2017: Indian Journal of Gastroenterology: Official Journal of the Indian Society of Gastroenterology
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