keyword
https://read.qxmd.com/read/38291698/budd-chiari-syndrome-in-children-radiological-intervention-and-role-of-shear-wave-elastography-in-monitoring-response
#21
JOURNAL ARTICLE
Arghya Samanta, Anshu Srivastava, Rajnikant Yadav, Aditya Kapoor, Anindya Ghosh, Prabhakar Mishra, Moinak Sen Sarma, Ujjal Poddar
OBJECTIVES: Radiological intervention (RI) is the preferred treatment in children with Budd-Chiari syndrome (BCS). We studied the comparative long-term outcome of BCS children, with and without RI and utility of liver and splenic stiffness measurement (LSM, SSM) by 2-dimensional shear wave elastography (2D-SWE) in assessing response. METHODS: Sixty children (40 boys, median age 10.5 [6.5-15.25] years) with BCS (29 newly diagnosed, 31 follow-up) were evaluated. LSM and SSM by 2D-SWE and vascular patency were monitored pre- and postprocedure (≥ 6 months postprocedure) in those undergoing RI...
January 2024: Journal of Pediatric Gastroenterology and Nutrition
https://read.qxmd.com/read/38289778/portal-hypertension-among-patients-with-chronic-liver-disease-admitted-to-the-department-of-internal-medicine-of-a-tertiary-care-centre
#22
JOURNAL ARTICLE
Sabina Khadka, Ananda Aryal, Sagun Karki, Pratik Subedi, Kanchan Bogati, Sunil Baniya, Shrekshya Khadka, Kumar Roka, Asha Shahi
INTRODUCTION: Portal hypertension is increased pressure within the portal vein. A portal pressure gradient of more than 10 mmHg is defined as "clinically significant portal hypertension" due to manifestations such as splenomegaly, gastroesophageal varices, ascites, hepatorenal syndrome, hepatopulmonary syndrome, hepatic encephalopathy, and spontaneous bacterial peritonitis. The aim of this study was to find out the prevalence of portal hypertension among patients with chronic liver disease admitted to the Department of Internal Medicine of a tertiary care centre...
October 1, 2023: JNMA; Journal of the Nepal Medical Association
https://read.qxmd.com/read/38283536/study-of-cardiac-dysfunction-in-portal-hypertension-a-single-center-experience-from-eastern-india
#23
JOURNAL ARTICLE
Sambit Kumar Behera, Prajyoti Behera, Jyoti Ranjan Behera, Gayatri Behera
INTRODUCTION: Cardiac functional abnormalities are common in patients with cirrhosis of the liver. Nonetheless, the effect of portal hypertension and liver disorder on cardiac abnormalities is yet to be investigated. The current study evaluated the contribution of cirrhotic and non-cirrhotic portal hypertension as the potential cause of cardiac abnormalities. METHODS: The present study was a cross-sectional observational study. After excluding known heart diseases, 128 patients with portal hypertension from different causes were enrolled in the study...
December 2023: Curēus
https://read.qxmd.com/read/38273971/a-rare-association-hereditary-hemorrhagic-telangiectasia-with-liver-cirrhosis-causing-portal-hypertension
#24
Denisse Morales-Tovar, Froylan D Martínez-Sánchez, Alejandro Gabutti-Thomas, Rodolfo Rivera-Martínez, Jacqueline Córdova-Gallardo
Hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber syndrome, is a vascular disorder of autosomal dominant etiology. The hallmark clinical feature is the presence of recurrent episodes of epistaxis in patients with vascular malformations and a tendency to bleed. We present the case of a 71-year-old woman who presented to the emergency department with upper gastrointestinal bleeding caused by esophageal varices, in conjunction with gastric angiodysplasias. The presence of oronasopharyngeal telangiectasias and hepatomegaly raised suspicion of HHT...
2024: Case Reports in Gastrointestinal Medicine
https://read.qxmd.com/read/38273643/statin-use-in-cirrhosis-and-its-association-with-incidence-of-portal-vein-thrombosis
#25
JOURNAL ARTICLE
Waseem Amjad, Zhenghui Jiang, Michelle Lai
BACKGROUND AND AIM: Statin use has shown a reduction in hepatic decompensation and portal hypertension. Its association with portal vein thrombosis (PVT) incidence is unknown. We aim to compare the incidence of PVT in patients with and without statin use. METHODS: We excluded patients with a history of hepatocellular cancer, liver transplants, Budd-Chiari syndrome, and intra-abdominal malignancies. Patients with cirrhosis were followed from their first hepatologist clinical encounter (January 1, 2016, to January 31, 2021) for 180 days to determine PVT incidence...
January 25, 2024: Journal of Gastroenterology and Hepatology
https://read.qxmd.com/read/38261889/bloody-lips-gluing-bleeding-lower-lip-spider-angioma-in-decompensated-cirrhosis
#26
JOURNAL ARTICLE
Akash Gandotra, Sunil Taneja, Madhumita Premkumar, Nipun Verma, Arka De, Sahaj Rathi, Ajay Duseja
Spider angiomas are dilated vascular channels in the skin. They have a central arteriole with surrounding vascular channels resembling legs of a spider, hence the name. They are frequently associated with liver cirrhosis, thyrotoxicosis, and pregnancy. We present the case of a 49-year-old gentleman who was referred to our liver clinic with complaints of jaundice and ascites of one-month duration. The patient was a chronic alcohol consumer, consuming country-made liquor, 80-100 grams/day for past 8-10 years...
2024: Journal of Clinical and Experimental Hepatology
https://read.qxmd.com/read/38255164/b-blockers-in-liver-cirrhosis-a-wonder-drug-for-every-stage-of-portal-hypertension-a-narrative-review
#27
REVIEW
Dimitrios S Karagiannakis, Nikolaos D Karakousis, Theodoros Androutsakos
In cirrhotic patients, non-selective b-blockers (NSBBs) constitute the reference treatment of choice as monotherapy or combined with band ligation for the prevention of first variceal bleeding and rebleeding, respectively. Furthermore, the last Baveno VII guidelines recommended carvedilol, a b-blocker with additional anti-a1 receptor activity, in all compensated cirrhotics with clinically significant portal hypertension, to prevent liver decompensation. Interestingly enough, NSBBs have been reported to have a potentially positive impact on the short-term mortality of patients with acute-on-chronic liver failure...
December 25, 2023: Biomedicines
https://read.qxmd.com/read/38249206/caroli-s-syndrome-a-case-report-and-literature-review
#28
Muhammad Nabeel Shafqat, Muhammad Yousuf Y Memon, Salman Javed, Sai Gautham Kanagala, Momina Saleem
Synonymous with congenital non-obstructive saccular or fusiform intra-hepatic duct dilatation and congenital communicating cavernous ectasia of the intra-hepatic biliary tract, Caroli's syndrome (CS) is an extremely rare fibro-polycystic liver disorder characterized by ductal plate malformation and consequent peri-portal fibrosis due to segmental intra-hepatic duct dilatation. No more than 200 cases of the syndrome have been reported since 1958. CS may affect one or both lobes of the liver, but more commonly it affects the left hepatic lobe...
December 2023: Curēus
https://read.qxmd.com/read/38235907/transjugular-intrahepatic-portosystemic-shunts-for-adults-with-hepatorenal-syndrome
#29
JOURNAL ARTICLE
Alejandro G Gonzalez-Garay, Aurora E Serralde-Zúñiga, Liliana Velasco Hidalgo, Nayelli Cointa Flores García, Ma Isabel Aguirre-Salgado
BACKGROUND: Hepatorenal syndrome is a condition that occurs in people with chronic liver disease (such as alcoholic hepatitis, advanced cirrhosis, or fulminant liver failure) and portal hypertension. The prognosis is dismal, often with a survival of weeks to months. Hepatorenal syndrome is characterised by the development of intense splanchnic vasodilation favouring ascites and hypotension leading to renal vasoconstriction and acute renal failure. Therefore, treatment attempts focus on improving arterial pressure through the use of vasopressors, paracentesis, and increasing renal perfusion pressure...
January 18, 2024: Cochrane Database of Systematic Reviews
https://read.qxmd.com/read/38190840/caroli-disease-combined-with-banti-syndrome-in-a-woman-a-case-report
#30
JOURNAL ARTICLE
Shian Yu, Hang Yuan, Yong Cao
Caroli disease is a rare congenital malformation that predisposes to segmental cystic dilatation of the intrahepatic bile ducts. Banti syndrome is characterized by persistent splenomegaly due to chronic congestion, resulting in a low hematocrit and ultimately leading to pancytopenia. In this report, we describe a 29-year-old woman who presented with a >20-year history of hepatitis B surface antigen positivity and a >1-year history of recurrent fatigue and malaise. On examination, the patient had abdominal distension with marked splenomegaly (7 cm below the ribs) and ascites with tenderness of the abdominal muscles to palpation...
January 2024: Journal of International Medical Research
https://read.qxmd.com/read/38176129/subcutaneous-therapy-for-portal-hypertension-phin-214-a-partial-vasopressin-receptor-1a-agonist
#31
JOURNAL ARTICLE
Gerardo M Castillo, Yao Yao, Rebecca E Guerra, Han Jiang, Akiko Nishimoto-Ashfield, Alexander V Lyubimov, Joshua F Alfaro, Kali A Striker, Nikolay Buynov, Phillip Schwabl, Elijah M Bolotin
Cirrhosis is a liver disease that leads to increased intrahepatic resistance, portal hypertension (PH), and splanchnic hyperemia resulting in ascites, variceal bleeding, and hepatorenal syndrome. Terlipressin, a prodrug that converts to a short half-life vasopressin receptor 1 A (V1a) full agonist [8-Lys]-Vasopressin (LVP), is an intravenous treatment for PH complications, but hyponatremia and ischemic side effects require close monitoring. We developed PHIN-214 which converts into PHIN-156, a more biologically stable V1a partial agonist...
January 3, 2024: Biomedicine & Pharmacotherapy
https://read.qxmd.com/read/38163189/transjugular-intrahepatic-portosystemic-shunt-for-the-treatment-of-hepatic-sinusoidal-obstruction-syndrome-caused-by-pyrrolizidine-alkaloids-a-multicenter-retrospective-study
#32
JOURNAL ARTICLE
Chaoyang Wang, Yingliang Wang, Jianbo Zhao, Chongtu Yang, Xiaoli Zhu, Huanzhang Niu, Junhui Sun, Bin Xiong
PURPOSE: To assess the impact of transjugular intrahepatic portosystemic shunt (TIPS) on clinical outcomes and liver histology in patients with hepatic sinusoidal obstruction syndrome (HSOS) caused by pyrrolizidine alkaloids (PA), and compare these results with those of patients who received supportive treatment alone. MATERIALS AND METHODS: From June 2015 to August 2022, 164 patients diagnosed with PA-HSOS in six tertiary care centers were retrospectively included in this study and divided into TIPS group (n = 69) and supportive treatment (ST) group (n = 95)...
January 15, 2024: Heliyon
https://read.qxmd.com/read/38158116/cediranib-ameliorates-portal-hypertensive-syndrome-via-inhibition-of-vegfr-2-signaling-in-cirrhotic-rats
#33
JOURNAL ARTICLE
Qiang Fan, Guangbo Wu, Min Chen, Guqing Luo, Zhenghao Wu, Haizhong Huo, Hongjie Li, Lei Zheng, Meng Luo
Portal hypertension (PHT) is a syndrome caused by systemic and portal hemodynamic disturbances with the progression of cirrhosis. However, the exact mechanisms regulating angiogenesis-related responses in PHT remain unclear. Cediranib is a potent inhibitor of vascular endothelial growth factor receptor (VEGFR) tyrosine kinases, exhibiting a greater affinity for VEGFR-2. Liver cirrhosis was induced by common bile duct ligation (BDL) in Sprague-Dawley rats. Sham-operated rats were controls. BDL and sham rats were randomly allocated to receive Cediranib or vehicle after BDL...
December 27, 2023: European Journal of Pharmacology
https://read.qxmd.com/read/38154981/-budd-chiari-syndrome-with-hepatopulmonary-syndrome-a-case-report-and-literature-review
#34
REVIEW
F Y Tian, X Dong, X H Hou, R Y Yuan, Y W Pan, D Zhang
Objective: To summarize the clinical features and prognosis of Budd-Chiari syndrome with hepatopulmonary syndrome (HPS) in children. Methods: The clinical data of a child who had Budd-Chiari syndrome with HPS treated at the Department of Pediatrics of the First Affiliated Hospital of Zhengzhou University in December 2016 was analyzed retrospectively. Taking "Budd-Chiari syndrome" and "hepatopulmonary syndrome" in Chinese or English as the keywords, literature was searched at CNKI, Wanfang, China Biomedical Literature Database and PubMed up to July 2023...
January 2, 2024: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://read.qxmd.com/read/38151450/histological-evaluation-of-pyrrolizidine-alkaloid-induced-hepatic-sinusoidal-obstruction-syndrome-correlation-with-drum-tower-severity-scoring
#35
JOURNAL ARTICLE
Ruiqi Li, Lin Li, Zihao Cai, Jun Chen, Han Zhang, Si Zhao, Jingjing Tu, Chang Sun, Yani Jin, Ming Zhang, Feng Zhang, Wei Zhang, Qin Yin, Hui Xu, Hao Han, Taishun Li, Yuzheng Zhuge, Jiangqiang Xiao
BACKGROUND: Hepatic sinusoidal obstruction syndrome induced by pyrrolizidine alkaloids (PA-HSOS) is a complication of drug-induced liver damage. Few studies have examined the relationship between pathological changes and clinical circumstances in PA-HSOS. The Drum Tower Severity Scoring System (DTSS) was developed using prognostic indicators from clinical treatment outcomes. We hypothesized that the severity of pathological damage is consistent with DTSS. AIMS: We aimed to improve our understanding and assessment of vascular liver injury disease histopathology by studying larger sample sizes of human histopathological samples...
December 26, 2023: Digestive and Liver Disease
https://read.qxmd.com/read/38146932/event-free-survival-of-maralixibat-treated-patients-with-alagille-syndrome-compared-to-a-real-world-cohort-from-gala
#36
JOURNAL ARTICLE
Bettina E Hansen, Shannon M Vandriel, Pamela Vig, Will Garner, Douglas B Mogul, Kathleen M Loomes, David A Piccoli, Elizabeth B Rand, Irena Jankowska, Piotr Czubkowski, Dorota Gliwicz-Miedzińska, Emmanuel M Gonzales, Emmanuel Jacquemin, Jérôme Bouligand, Lorenzo D'Antiga, Emanuele Nicastro, Henrik Arnell, Björn Fischler, Étienne Sokal, Tanguy Demaret, Susan Siew, Michael Stormon, Saul J Karpen, Rene Romero, Noelle H Ebel, Jeffrey A Feinstein, Amin J Roberts, Helen M Evans, Shikha S Sundaram, Alexander Chaidez, Winita Hardikar, Sahana Shankar, Ryan T Fischer, Florence Lacaille, Dominique Debray, Henry C Lin, M Kyle Jensen, Catalina Jaramillo, Palaniswamy Karthikeyan, Giuseppe Indolfi, Henkjan J Verkade, Catherine Larson-Nath, Ruben E Quiros-Tejeira, Pamela L Valentino, Maria Rogalidou, Antal Dezsőfi, James E Squires, Kathleen Schwarz, Pier Luigi Calvo, Jesus Quintero Bernabeu, Andréanne N Zizzo, Gabriella Nebbia, Pinar Bulut, Ermelinda Santos-Silva, Rima Fawaz, Silvia Nastasio, Wikrom Karnsakul, María Legarda Tamara, Cristina Molera Busoms, Deirdre Kelly, Thomas Damgaard Sandahl, Carolina Jimenez-Rivera, Jesus M Banales, Quais Mujawar, Li-Ting Li, Huiyu She, Jian-She Wang, Kyung Mo Kim, Seak Hee Oh, Maria Camila Sanchez, Maria Lorena Cavalieri, Way Seah Lee, Christina Hajinicolaou, Chatmanee Lertudomphonwanit, Orith Waisbourd-Zinman, Cigdem Arikan, Seema Alam, Elisa Carvalho, Melina Melere, John Eshun, Zerrin Önal, Dev M Desai, Sabina Wiecek, Raquel Borges Pinto, Victorien M Wolters, Jennifer Garcia, Marisa Beretta, Nanda Kerkar, Jernej Brecelj, Nathalie Rock, Eberhard Lurz, Niviann Blondet, Uzma Shah, Richard J Thompson, Binita M Kamath
BACKGROUND AND AIMS: Alagille syndrome (ALGS) is characterized by chronic cholestasis with associated pruritus and extrahepatic anomalies. Maralixibat, an ileal bile acid transporter inhibitor, is the first-approved pharmacologic therapy for cholestatic pruritus in ALGS. Since long-term placebo-controlled studies are not feasible or ethical in children with rare diseases, a novel approach was taken comparing 6-year outcomes from maralixibat trials with an aligned and harmonized natural history cohort from the Global ALagille Alliance (GALA) study...
December 25, 2023: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://read.qxmd.com/read/38139297/hepatorenal-syndrome-novel-insights-into-diagnostics-and-treatment
#37
REVIEW
Krzysztof Badura, Weronika Frąk, Joanna Hajdys, Gabriela Majchrowicz, Ewelina Młynarska, Jacek Rysz, Beata Franczyk
Hepatorenal syndrome (HRS) is a disorder associated with cirrhosis and renal impairment, with portal hypertension as its major underlying cause. Moreover, HRS is the third most common cause of acute kidney injury, thus creating a major public health concern. This review summarizes the available information on the pathophysiological implications of HRS. We discuss pathogenesis associated with HRS. Mechanisms such as dysfunction of the circulatory system, bacterial infection, inflammation, impaired renal autoregulation, circulatory, and others, which have been identified as critical pathways for development of HRS, have become easier to diagnose in recent years...
December 14, 2023: International Journal of Molecular Sciences
https://read.qxmd.com/read/38125301/lower-free-triiodothyronine-ft3-levels-in-cirrhosis-are-linked-to-systemic-inflammation-higher-risk-of-acute-on-chronic-liver-failure-and-mortality
#38
JOURNAL ARTICLE
Lukas Hartl, Benedikt Simbrunner, Mathias Jachs, Peter Wolf, David Josef Maria Bauer, Bernhard Scheiner, Lorenz Balcar, Georg Semmler, Michael Schwarz, Rodrig Marculescu, Varius Dannenberg, Michael Trauner, Mattias Mandorfer, Thomas Reiberger
BACKGROUND & AIMS: Advanced chronic liver disease (ACLD) may affect thyroid hormone homeostasis. We aimed to analyze the pituitary-thyroid axis in ACLD and the prognostic value of free triiodothyronine (fT3). METHODS: Patients with ACLD (liver stiffness measurement [LSM] ≥10 kPa) undergoing hepatic venous pressure gradient (HVPG) measurement between June 2009 and September 2022 and available fT3 levels were included. Clinical stages of ACLD were defined as follows: probable ACLD (pACLD; LSM ≥10 kPa and HVPG ≤5 mmHg), S0 (mild portal hypertension [PH]; HVPG 6-9 mmHg), S1 (clinically significant PH), S2 (clinically significant PH with varices), S3 (past variceal bleeding), S4 (past/current non-bleeding hepatic decompensation), and S5 (further decompensation)...
January 2024: JHEP reports: innovation in hepatology
https://read.qxmd.com/read/38117438/liver-transplantation-for-zellweger-syndrome
#39
JOURNAL ARTICLE
Jagadeesh Menon, Naresh Shanmugam, Mukul Vij, Ashwin Rammohan, Mohamed Rela
Zellweger syndrome or cerebrohepatorenal syndrome is a rare, multisystem disorder occurring due to defect in metabolic pathway within the peroxisomes. Cirrhosis with portal hypertension is an important presentation of these patients. Given its progressive, multisystem nature, the role of liver transplantation (LT) in Zellweger syndrome remains undefined and controversial. An 11-y-old boy diagnosed with Zellweger syndrome presented to the authors with decompensated cirrhosis along with bilateral proptosis...
December 20, 2023: Indian Journal of Pediatrics
https://read.qxmd.com/read/38115240/caroli-disease-with-subcutaneous-hemorrhage-as-the-sole-clinical-manifestation-a-case-report
#40
JOURNAL ARTICLE
Wentai Yang, Qing Jin
RATIONAL: The disease of Caroli is a rare congenital disorder, characterized by the dilated intrahepatic bile ducts, resulting from mutations in the PKHD1 gene. Caroli syndrome, characterized by dilated intrahepatic bile ducts with congenital hepatic fibrosis, is linked to autosomal recessive polycystic kidney disease. The clinical manifestations of Caroli disease are not typical, and Caroli disease is easy to be missed and misdiagnosed. Therefore, we reported this case in the hope of raising awareness of the disease among clinicians...
December 15, 2023: Medicine (Baltimore)
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