keyword
https://read.qxmd.com/read/38073401/a-rare-case-of-hepatic-sinusoidal-occlusive-syndrome-in-a-premature-neonate-with-trisomy-21
#21
JOURNAL ARTICLE
A J Alenchery, N K Yeaney, C B Chen, R Talati, E Vogelius, C Tan, K Radhakrishnan
Trisomy 21 (Down Syndrome) may lead to multiple hematological and hepatobiliary manifestations including the development of transient abnormal myelopoiesis. While many cases resolve, transient abnormal myelopoiesis may lead to significant morbidity and mortality in a small percentage of patients. This condition may present a diagnostic challenge for physicians and currently there is only limited data on effective treatments, particularly with low blast percent transient abnormal myelopoiesis. We present a case of a neonate with trisomy 21 and multiple congenital anomalies who consequently developed hepatic failure with evidence of non-cirrhotic portal hypertension likely due to transient abnormal myelopoiesis...
December 7, 2023: Journal of Neonatal-perinatal Medicine
https://read.qxmd.com/read/38025487/portal-hypertension-in-children-a-tertiary-center-experience-in-turkey
#22
JOURNAL ARTICLE
Emine Nur Sunar Yayla, Sinan Sarı, Neslihan Gürcan Kaya, Ödül Eğrİtaş Gürkan, Hakan Sözen, İbrahim Onur Özen, Aydın Dalgıç, Buket Dalgıç
PURPOSE: Portal hypertension (PH) and its complications have a significant impact on morbidity and mortality. This study aimed to evaluate the etiology; clinical, laboratory, and endoscopic findings; treatment approaches; long-term outcomes; and prognosis of pediatric PH. METHODS: This retrospective study included 222 pediatric patients diagnosed with PH between 1998 and 2016, and data encompassing clinical, laboratory, and radiological features; treatments; and complications were analyzed...
November 2023: Pediatric Gastroenterology, Hepatology & Nutrition
https://read.qxmd.com/read/38018771/portal-hypertension-features-are-associated-with-ascites-occurrence-and-survival-in-patients-with-hepatocellular-carcinoma-treated-by-external-radiotherapy
#23
JOURNAL ARTICLE
Héloïse Giudicelli, Mickaël Andraud, Mathilde Wagner, Rémi Bourdais, Claire Goumard, Olivier Scatton, Dominique Thabut, Jean-Marc Simon, Manon Allaire
BACKGROUND AND AIMS: We studied the impact of Portal hypertension (PHT) on ascites occurrence and on radiotherapy outcome in cirrhotic patients with hepatocellular carcinoma (HCC). METHOD: All cirrhotic patients that received radiotherapy for HCC between 2012 and 2022 were included. Portal hypertension-Score was built using univariate analysis with the presence of esophageal varices (EV), platelet count, history of acute variceal bleeding (AVB) and spleen size. Time-to-events data were estimated using Kaplan-Meier method with log-rank and Cox-models...
November 29, 2023: United European Gastroenterology Journal
https://read.qxmd.com/read/38018555/the-role-of-elastography-in-clinically-significant-portal-hypertension
#24
JOURNAL ARTICLE
Angelo Alves de Mattos, Angelo Zambam de Mattos, Giovana Dal Pozzo Sartori, Gustavo Tovo Both, Cristiane Valle Tovo
• In compensated cirrhosis, using non-invasive methods would exempt the patient from the need of an endoscopy. • The Baveno VII presented the "rule of 5" for Vibration-Controlled Transient Elastography; liver stiffness measurement ≤15 kPa and platelets >150.000/mm3 exclude clinically significant portal hypertension (CSPH), while when ≥25 kPa is highly suggestive of CSPH. • Spleen stiffness measurement has been proposed as a more specific technique to predict the presence of CSPH...
2023: Arquivos de Gastroenterologia
https://read.qxmd.com/read/37975050/von-willebrand-factor-as-a-biomarker-for-liver-disease-an-update
#25
REVIEW
Anshuman Elhence, Shalimar
The von Willebrand factor (vWF) is best known for its role in the hemostatic pathway, aiding platelet adhesion and aggregation, as well as circulating along with coagulation factor VIII, prolonging its half-life. However, vWF is more than a hemostatic protein and is a marker of endothelial dysfunction in patients with cirrhosis. The levels of vWF increase progressively as cirrhosis progresses. Despite its qualitative defects, it can support and carry out its hemostatic role and contribute to a pro-coagulant disbalance...
2023: Journal of Clinical and Experimental Hepatology
https://read.qxmd.com/read/37940774/non-cirrhotic-intrahepatic-portal-hypertension-is-the-gun-loaded
#26
EDITORIAL
Suprabhat Giri, Anil Chandra Anand
No abstract text is available yet for this article.
November 9, 2023: Indian Journal of Gastroenterology: Official Journal of the Indian Society of Gastroenterology
https://read.qxmd.com/read/37936020/impact-of-liver-cirrhosis-severity-of-cirrhosis-and-portal-hypertension-on-the-difficulty-and-outcomes-of-laparoscopic-and-robotic-major-liver-resections-for-primary-liver-malignancies
#27
JOURNAL ARTICLE
Federica Cipriani, Luca Aldrighetti, Francesca Ratti, Andrew G R Wu, Tousif Kabir, Olivier Scatton, Chetana Lim, Wanguang Zhang, Jasper Sijberden, Davit L Aghayan, Tiing-Foong Siow, Safi Dokmak, Fabricio Ferreira Coelho, Paulo Herman, Marco V Marino, Vincenzo Mazzaferro, Adrian K H Chiow, Iswanto Sucandy, Arpad Ivanecz, Sung-Hoon Choi, Jae Hoon Lee, Mikel Gastaca, Marco Vivarelli, Felice Giuliante, Andrea Ruzzenente, Chee-Chien Yong, Mengqiu Yin, Constantino Fondevila, Mikhail Efanov, Zenichi Morise, Fabrizio Di Benedetto, Raffaele Brustia, Raffaele Dalla Valle, Ugo Boggi, David Geller, Andrea Belli, Riccardo Memeo, Salvatore Gruttadauria, Alejandro Mejia, James O Park, Fernando Rotellar, Gi-Hong Choi, Ricardo Robles-Campos, Xiaoying Wang, Robert P Sutcliffe, Johann Pratschke, Eric C H Lai, Charing C N Chong, Mathieu D'Hondt, Kazuteru Monden, Santiago Lopez-Ben, T Peter Kingham, Alessandro Ferrero, Giuseppe Maria Ettorre, Daniel Cherqui, Xiao Liang, Olivier Soubrane, Go Wakabayashi, Roberto I Troisi, Tan-To Cheung, Yutaro Kato, Atsushi Sugioka, Ho-Seong Han, Tran Cong Duy Long, Qu Liu, Rong Liu, Bjørn Edwin, David Fuks, Kuo-Hsin Chen, Mohammad Abu Hilal, Brian K P Goh
BACKGROUND: Minimally invasive liver resections (MILR) offer potential benefits such as reduced blood loss and morbidity compared with open liver resections. Several studies have suggested that the impact of cirrhosis differs according to the extent and complexity of resection. Our aim was to investigate the impact of cirrhosis on the difficulty and outcomes of MILR, focusing on major hepatectomies. METHODS: A total of 2534 patients undergoing minimally invasive major hepatectomies (MIMH) for primary malignancies across 58 centers worldwide were retrospectively reviewed...
November 7, 2023: Annals of Surgical Oncology
https://read.qxmd.com/read/37908069/predictors-of-long-term-rebleeding-risk-in-cirrhotic-patients-undergoing-esophagogastric-devascularization-and-splenectomy-impact-of-portal-vein-thrombosis-and-hemoglobin-levels
#28
JOURNAL ARTICLE
Jian-Bo Han, Qing-Hua Shu, Yong-Xiang Yi, Bei-Cheng Sun
BACKGROUND Esophagogastric devascularization and splenectomy (EGDS) is widely used to treat patients with portal hypertension in China. This study aimed to determine risk factors that increase risk of rebleeding after EGDS and evaluate the effect of portal vein thrombosis (PVT) on rebleeding rates after EGDS. MATERIAL AND METHODS Clinical data of patients with cirrhosis (n=138) who underwent EGDS between December 2010 and January 2016 were retrospectively analyzed. Patients were assigned to rebleeding or non-rebleeding groups and followed up...
November 1, 2023: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://read.qxmd.com/read/37892084/a-narrative-review-on-non-cirrohotic-portal-hypertension-not-all-portal-hypertensions-mean-cirrhosis
#29
REVIEW
Michele Fiordaliso, Giuseppe Marincola, Barbara Pala, Raffaella Muraro, Mariangela Mazzone, Maria Carmela Di Marcantonio, Gabriella Mincione
Non-cirrhotic portal hypertension (NCPH), also known as idiopathic non-cirrhotic portal hypertension (INCPH) and porto-sinusoidal vascular disorder (PSVD), is a rare disease characterized by intrahepatic portal hypertension (IPH) in the absence of cirrhosis. The precise etiopathogenesis of IPH is an area of ongoing research. NCPH diagnosis is challenging, as there are no specific tests available to confirm the disease, and a high-quality liver biopsy, detailed clinical information, and an expert pathologist are necessary for diagnosis...
October 20, 2023: Diagnostics
https://read.qxmd.com/read/37880457/ct-and-mr-imaging-of-primary-biliary-cholangitis-a-pictorial-review
#30
REVIEW
Yun Zhang, Tianying Zheng, Zixing Huang, Bin Song
Primary biliary cholangitis (PBC) is a rare chronic autoimmune-mediated cholestatic liver disease involving medium and small bile ducts that can lead to liver fibrosis and cirrhosis. To date, the pathogenesis of PBC remains elusive, and there is currently no curative medical treatment. Computed tomography (CT) and magnetic resonance (MR) imaging, as common technical tools that allow non-invasive monitoring of liver tissue in vivo, play crucial roles in the diagnosis, staging, and prognosis prediction in PBC by enabling assessment of abnormalities in liver morphology and parenchyma, irregular configuration of bile ducts, lymphadenopathy, portal hypertension, and complications of cirrhosis...
October 26, 2023: Insights Into Imaging
https://read.qxmd.com/read/37869072/management-of-liver-disease-and-portal-hypertension-in-common-variable-immunodeficiency-cvid
#31
REVIEW
Lukas S Baumert, Angela Shih, Raymond T Chung
Patients with common variable immunodeficiency (CVID) frequently develop liver disease and associated complications, which represent an increasingly prevalent unmet medical need. The main hepatic manifestation of CVID is nodular regenerative hyperplasia (NRH), resulting in non-cirrhotic portal hypertension (NCPH). Liver disease is often underdiagnosed, leading to poor outcomes and decreased survival. The increasing numbers of patients with CVID who are diagnosed late with progressive liver disease underscores the importance of appropriate clinical management and treatment of liver complications...
November 2023: JHEP reports: innovation in hepatology
https://read.qxmd.com/read/37847236/the-early-initiation-of-perioperative-anticoagulation-therapy-in-cirrhotic-patients-with-portal-hypertension-after-laparoscopic-splenectomy-plus-esophagogastric-devascularization-a-10-year-single-center-experience
#32
JOURNAL ARTICLE
Dong Wang, Xiao Chen, Ling Lv, Tao Yang, Bo Huang, Yanlong Cao, Jianguo Lu, Jikai Yin
Background: Portal veinous system thrombosis (PVST) is a common complication after laparoscopic splenectomy plus esophagogastric devascularization (LSED). Anticoagulation therapy was still in debate in LSED perioperation. This study aimed to determine the postoperative risk factors of PVST, the efficacy and safety of the anticoagulation therapy after LSED procedure, and the potential impact of anticoagulation on patients' liver function and overall survival (OS). Methods: Three hundred patients who underwent LSED were retrospectively enrolled and analyzed in the study...
October 17, 2023: Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A
https://read.qxmd.com/read/37830057/case-report-novel-dguok-variants-associated-with-idiopathic-non-cirrhotic-portal-hypertension-in-a-han-chinese-child
#33
Jia-Qi Li, Jia-Yan Feng, Ying Gong, Wang-Qiang Li, Teng Liu
DGUOK deficiency has primarily been associated with lethal hepatic failure with or without hypotonia, nystagmus, and psychomotor retardation, features typical of mitochondrial disease. A study in 3 Turkish children identified homozygosity for a variant in DGUOK as associated with idiopathic non-cirrhotic portal hypertension (INCPH). However, no further instances of INCPH associated with DGUOK variants have been reported. We here describe a fourth patient with DGUOK variants and childhood-onset INCPH, a 12-year-old Han Chinese boy, reporting clinical manifestations, histopathologic findings, and results of genetic studies...
2023: Frontiers in Pediatrics
https://read.qxmd.com/read/37827507/platelet-activation-is-upregulated-in-cirrhotic-patients-with-portal-vein-thrombosis
#34
JOURNAL ARTICLE
Jingnuo Ding, Fazhi Zhao, Juanjuan Cui, Mingqing Zhu, Weifeng Zhao
OBJECTIVE: The alteration of platelet function plays a key role in thrombosis formation. This study aimed to explore platelet function alterations in the formation of portal vein thrombosis (PVT) in cirrhosis. METHODS: Cirrhotic patients admitted to hospital between October 2021 and April 2023 were recruited and divided into PVT and non-PVT groups. Flow cytometry was used to detect the expression of CD62p, CD63, monocyte-platelet aggregates (MPAs), neutrophil-platelet aggregates (NPAs) and vWF-Ag to evaluate platelet activation and adhesion function...
October 12, 2023: Thrombosis and Haemostasis
https://read.qxmd.com/read/37796423/focused-panel-sequencing-points-to-genetic-predisposition-in-non-cirrhotic-intrahepatic-portal-hypertension-patients-in-india
#35
JOURNAL ARTICLE
Rekha Aaron, Kalpana Premkumar, Aaron Chapla, B Vijayalekshmi, Uday Zachariah, Elwyn Elias, Thomas Alex Kodiatte, Dolly Daniel, John Jude, K A Balasubramanian, Sukesh C Nair, Nihal Thomas, Banumathi Ramakrishna, C E Eapen, Ashish Goel
OBJECTIVE: Non-cirrhotic intrahepatic portal hypertension (NCIPH), a portal microangiopathy affecting small portal vein radicles, is a disease of Indian sub-continent. NCIPH appears to be a complex disease with interactions between inherited and acquired factors, though the exact pathophysiological mechanism is unknown. We aimed at investigating the genetic variants that might contribute to susceptibility to NCIPH. METHODS: In this case-control study, we analyzed genes associated with microangiopathy-VWF-ADAMTS13 (von Willebrand factor and its cleavase enzyme - a disintegrin and matrix metalloprotease with thrombospondin type-1 motifs member 13) and alternative complement system vitamin B12 metabolism and with familial NCIPH...
October 5, 2023: Indian Journal of Gastroenterology: Official Journal of the Indian Society of Gastroenterology
https://read.qxmd.com/read/37795099/rituximab-and-improved-nodular-regenerative-hyperplasia-associated-non-cirrhotic-liver-disease-in-common-variable-immunodeficiency-a-case-report-and-literature-study
#36
REVIEW
Willem Roosens, Frederik Staels, Sien Van Loo, Stephanie Humblet-Baron, Isabelle Meyts, Hadewijch De Samblanx, Chris Verslype, Hannah van Malenstein, Schalk van der Merwe, Wim Laleman, Rik Schrijvers
Common variable immunodeficiency (CVID) associated liver disease is an underrecognized and poorly studied non-infectious complication that lacks an established treatment. We describe a CVID patient with severe multiorgan complications, including non-cirrhotic portal hypertension secondary to nodular regenerative hyperplasia leading to diuretic-refractory ascites. Remarkably, treatment with rituximab, administered for concomitant immune thrombocytopenia, resulted in the complete and sustained resolution of portal hypertension and ascites...
2023: Frontiers in Immunology
https://read.qxmd.com/read/37779802/a-case-of-non-cirrhotic-portal-hypertension-in-a-patient-with-primary-myelofibrosis-disease
#37
Ruchi Mangal, Maria Jamil, Zeinab Nasser, James P Purtell
Idiopathic non-cirrhotic portal hypertension can emerge due to a varied spectrum of underlying and contributory factors, presenting in the form of abdominal distention as the initial symptom encountered. Often, a patient remains asymptomatic to the underlying cause and seeks medical care for their abdominal enlargement. As the portal hypertension continues to progress, ascites begins to develop due to a history of portal vein thrombosis being sufficient to increase splanchnic blood flow in a portal hypertensive pattern...
August 2023: Curēus
https://read.qxmd.com/read/37773790/case-report-successful-pregnancy-complicated-with-non-cirrhotic-portal-hypertension-in-a-lady-who-suffered-from-postpartum-hemorrhage-previously
#38
JOURNAL ARTICLE
Xiaoxi Niu, Yanmin Gong, Xia Luo
RATIONALE: Non-cirrhotic portal hypertension (NCPH) is characterized by the absence of cirrhotic modification of the liver and the patency of the portal and hepatic veins. When compared to the general population, NCPH is associated with an increased risk of maternal and perinatal morbidity and mortality during pregnancy. NCPH was present in the majority (74.1%) of pregnant women with portal hypertension. One (25%) out of every 4 pregnancies was complicated by variceal hemorrhage while pregnant...
September 29, 2023: Medicine (Baltimore)
https://read.qxmd.com/read/37770804/how-non-alcoholic-fatty-liver-disease-and-cirrhosis-affect-the-heart
#39
REVIEW
Søren Møller, Signe Wiese, Mads Barløse, Jens D Hove
Liver diseases affect the heart and the vascular system. Cardiovascular complications appear to be a leading cause of death in patients with non-alcoholic fatty liver disease (NAFLD) and cirrhosis. The predominant histological changes in the liver range from steatosis to fibrosis to cirrhosis, which can each affect the cardiovascular system differently. Patients with cirrhotic cardiomyopathy (CCM) and NAFLD are at increased risk of impaired systolic and diastolic dysfunction and for suffering major cardiovascular events...
September 28, 2023: Hepatology International
https://read.qxmd.com/read/37731994/direct-oral-anticoagulants-for-the-treatment-of-splanchnic-vein-thrombosis-a-state-of-art
#40
REVIEW
Giovanni Monaco, Luca Bucherini, Bernardo Stefanini, Fabio Piscaglia, Francesco Giuseppe Foschi, Luca Ielasi
Splanchnic vein thrombosis (SVT) is a manifestation of venous thromboembolism in an unusual site. Portal, mesenteric, and splenic veins are the most common vessels involved in SVT which occurs mainly in patients with liver cirrhosis, although non-cirrhotic patients could be affected as well. Thrombosis of hepatic veins, also known as Budd-Chiari syndrome, is another manifestation of SVT. Prompt diagnosis and intervention are mandatory in order to increase the recalization rate and reduce the risk of thrombus progression and hypertensive complications...
September 7, 2023: World Journal of Gastroenterology: WJG
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