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https://www.readbyqxmd.com/read/28884036/severe-hepatopulmonary-syndrome-in-a-child-with-caroli-syndrome
#1
W De Jesus-Rojas, K McBeth, A Yadav, J M Stark, R A Mosquera, C Jon
Hepatopulmonary Syndrome (HPS) is a potential complication of chronic liver disease and is more commonly seen in the adult population. Caroli Syndrome is a rare inherited disorder characterized by intrahepatic ductal dilation and liver fibrosis that leads to portal hypertension. In children with liver disease, HPS should be considered in the differential diagnosis of prolonged, otherwise unexplained, hypoxemia. The presence of HPS can improve patient priority on the liver transplantation wait list, despite their Pediatric End-Stage Liver Disease (PELD) score...
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/28881059/pancreas-preserving-duodenectomy-after-living-donor-liver-transplantation-for-invasive-cytomegalovirus-disease
#2
Rodrigo Vincenzi, Eduardo A Fonseca, Paulo Chapchap, Marcel C C Machado, Karina M O Roda, Helry L Candido, Marcel R Benavides, Marco A D'Assuncao, Rogerio C Afonso, Plinio Turine, Fernando P Marson, João Seda Neto
CMV infection plays an important role in the postoperative course following solid organ transplantation. We present the case of an 11-year-old male patient who underwent LDLT due to severe hepatopulmonary syndrome and biliary cirrhosis. Four weeks after LDLT, he developed persistent GI bleeding and was subjected to repeated endoscopic treatment and radiological arterial embolization to stop the bleeding from duodenal ulcers. Diagnostic workup was negative for CMV disease. Because the bleeding persisted, surgical treatment was indicated, and a pancreas-preserving duodenectomy was performed...
September 7, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28871319/onset-ages-of-hepatopulmonary-syndrome-and-pulmonary-hypertension-in-patients-with-biliary-atresia
#3
Takehisa Ueno, Ryuta Saka, Yuichi Takama, Hiroaki Yamanaka, Yuko Tazuke, Kazuhiko Bessho, Hiroomi Okuyama
PURPOSE: Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PoPH) are extrahepatic complications of biliary atresia (BA). Their detection is sometimes delayed, which may result in missed opportunities for liver transplantation. The aim of this study was to determine the onset ages of HPS and PoPH in BA patients. METHODS: BA patients followed at our institution were identified. Patients visited our clinic for routine blood work, as well as regular electrocardiography, chest X-rays, and arterial blood gas tests...
September 4, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28853802/-hepatopulmonary-syndrome-and-portopulmonary-hypertension
#4
Cristina Marcu, Eduardo Schiffer, John-David Aubert, Julien Vionnet, Patrick Yerly, Pierre Deltenre, Astrid Marot
Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH) are two frequent pulmonary complications of liver disease. Portal hypertension is a key element in the pathogenesis of both disorders, which are however distinct in terms of pathogenesis, diagnosis and treatment. HPS corresponds to an abnormal arterial oxygenation in relation with the development of intrapulmonary vascular dilatations. POPH is a pulmonary arterial hypertension in the setting of portal hypertension and elevated pulmonary vascular resistance...
August 30, 2017: Revue Médicale Suisse
https://www.readbyqxmd.com/read/28840954/assessing-the-kinetics-of-microbubble-appearance-in-cirrhotic-patients-using-transthoracic-saline-contrast-enhanced-echocardiography
#5
Adriano R Tonelli, Tawfeq Naal, Wael Dakkak, Margaret M Park, Raed A Dweik, James K Stoller
BACKGROUND: The number of cardiac cycles that need to be reviewed by echocardiography before a significant intrapulmonary shunt can be excluded remains unclear. METHODS: We retrospectively identified patients with cirrhosis who underwent technetium-99 m-labeled macroaggregated albumin scanning. The kinetics of bubble appearance after the injection of agitated saline during transthoracic echocardiograms were assessed in these patients. RESULTS: For the 64 eligible patients, the mean ± SD age was 56 ± 9 years...
August 25, 2017: Echocardiography
https://www.readbyqxmd.com/read/28833992/successful-venoarterial-extracorporeal-membrane-oxygenation-for-prolonged-hepatopulmonary-syndrome-following-pediatric-liver-transplantation-a-case-report-and-review-of-the-literature
#6
Hamza Hassan Khan, Laura Schroeder, Megha S Fitzpatrick, Stuart S Kaufman, Nada A Yazigi, Gregory K Yurasek, David M Steinhorn, Thomas M Fishbein, Khalid M Khan
HPS is a major complicating feature of end-stage liver disease. Diagnosis is clinical, and LT is the only definitive treatment. While the general impression is that HPS improves quickly after transplantation, it may not always be the case. We describe the smallest reported child with HPS prior to LT and requiring prolonged venoarterial extracorporeal membrane oxygenation after LT; especially as it is a rare occurrence, physician managing such cases should be aware of the circumstances under which HPS may require specific treatment...
August 19, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28759737/differential-diagnosis-of-hepatopulmonary-syndrome-hps-portopulmonary-hypertension-pph-and-hereditary-hemorrhagic-telangiectasia-hht
#7
REVIEW
Inna Krynytska, Mariya Marushchak, Anna Mikolenko, Anzhela Bob, Iryna Smachylo, Ludmyla Radetska, Olga Sopel
Hepatopulmonary syndrome (HPS) is a severe complication of advanced liver disease associated with an extremely poor prognosis. HPS is diagnosed in 4-47% of patients with cirrhosis and in 15-20% of candidates for liver transplantation. In addition, severe hypoxia is associated with a high risk of complications of liver transplantation (a 30% chance during the first 90 days) and increases the gap between transplantation and improving arterial oxygenation. The pathogenesis of HPS is not fully understood, and no effective pharmacological treatment has been developed yet...
July 31, 2017: Bosnian Journal of Basic Medical Sciences
https://www.readbyqxmd.com/read/28729092/annexin-a2-modulated-proliferation-of-pulmonary-arterial-smooth-muscle-cells-depends-on-caveolae-and-caveolin-1-in-hepatopulmonary-syndrome
#8
Lin Liao, Binwu Zheng, Bin Yi, Chang Liu, Lin Chen, Ziyang Zeng, Jing Gao
We have established that annexin A2 (ANXA2) is an important factor in the experimental hepatopulmonary syndrome (HPS) serum-induced proliferation of pulmonary arterial smooth muscle cells (PASMCs). However, the detailed mechanism remains unclear. ANXA2 translocated to the caveolin-enriched microdomains (caveolae) in PASMCs upon HPS serum stimulation. The disruption of caveolae by Methyl-β-cyclodextrin (MβCD) alleviated the caveolae recruitment of ANXA2 and the ANXA2-mediated activation of ERK1/2 and NF-κB, so that ANXA2-modulated PASMC proliferation was suppressed...
July 17, 2017: Experimental Cell Research
https://www.readbyqxmd.com/read/28705726/dual-organ-beneficial-effects-of-metformin-in-cirrhotic-rats-with-hepatopulmonary-syndrome
#9
EDITORIAL
Chih-Wei Liu, Ying-Ying Yang
No abstract text is available yet for this article.
July 10, 2017: Journal of the Chinese Medical Association: JCMA
https://www.readbyqxmd.com/read/28676445/metformin-reduces-intrahepatic-fibrosis-and-intrapulmonary-shunts-in-biliary-cirrhotic-rats
#10
Mu-Tzu Ko, Hui-Chun Huang, Wen-Shin Lee, Chiao-Lin Chuang, I-Fang Hsin, Shao-Jung Hsu, Fa-Yauh Lee, Ching-Chih Chang, Shou-Dong Lee
BACKGROUND: Liver fibrosis causes portal hypertension which dilates collateral vasculature and enhances extra-hepatic angiogenesis including intrapulmonary shunts, which subsequently complicates with hepatopulmonary syndrome. Metformin is an anti-diabetic agent which possesses anti-inflammation and anti-angiogenesis properties. This study evaluated the effect of metformin treatment on liver and lung in a non-diabetic rat model with biliary cirrhosis induced via common bile duct ligation (CBDL)...
July 1, 2017: Journal of the Chinese Medical Association: JCMA
https://www.readbyqxmd.com/read/28642034/cyclooxygenase-2-promotes-pulmonary-intravascular-macrophage-accumulation-by-exacerbating-bmp-signaling-in-rat-experimental-hepatopulmonary-syndrome
#11
Chang Liu, Jing Gao, Bing Chen, Lin Chen, Karine Belguise, Weifeng Yu, Kaizhi Lu, Xiaobo Wang, Bin Yi
BACKGROUND AND AIMS: One central factor in hepatopulmonary syndrome (HPS) pathogenesis is intravascular accumulation of activated macrophages in small pulmonary arteries. However, molecular mechanism underlying the macrophage accumulation in HPS is unknown. In this study, we aimed to explore whether elevated COX-2 induces the Bone morphogenic protein-2 (BMP-2)/Crossveinless-2 (CV-2) imbalance and then activation of BMP signaling pathway promotes the macrophage accumulation in Common Bile Duct Ligation (CBDL) rat lung...
August 15, 2017: Biochemical Pharmacology
https://www.readbyqxmd.com/read/28641354/-acute-liver-failure-acute-on-chronic-liver-failure-hepatorenal-syndrome-hepatopulmonary-syndrome-and-portopulmonary-hypertension-artificial-liver-support-on-the-icu
#12
Uwe Lodes, David Jacob, Frank Meyer
Background Acute hepatic dysfunction in the form of acute liver failure (ALF) or acute-on-chronic liver failure (ACLF) is a disease with a high risk of mortality and requires interdisciplinary intensive care. Aim This article explains the nomenclature, pathophysiology, prognosis and possible treatment options of ALF and ACLF, including the possibilities of extracorporeal liver support therapy at the point of liver transplantation (LTx). Method Narrative review with a selective literature review and representative case studies...
June 2017: Zentralblatt Für Chirurgie
https://www.readbyqxmd.com/read/28632747/selective-cyclooxygenase-inhibition-by-sc-560-improves-hepatopulmonary-syndrome-in-cirrhotic-rats
#13
Ching-Chih Chang, Wen-Shin Lee, Hsian-Guey Hsieh, Chiao-Lin Chuang, Hui-Chun Huang, Fa-Yauh Lee, Shou-Dong Lee
OBJECTIVE: Hepatopulmonary syndrome (HPS) is characterized by hypoxia in patients with chronic liver disease. The mechanism of HPS includes pulmonary vasodilatation, inflammation, and angiogenesis. Prostaglandins synthesized by cyclooxygenases (COX) participate in vascular responsiveness, inflammation and angiogenesis, which can be modulated by COX inhibitors. We therefore evaluated the impact of COX inhibition in rats with common bile duct ligation (CBDL)-induced liver cirrhosis and HPS...
2017: PloS One
https://www.readbyqxmd.com/read/28612753/do-late-bubbles-correspond-to-early-hepatopulmonary-syndrome
#14
Hilary M DuBrock, Michael J Krowka
No abstract text is available yet for this article.
August 1, 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/28611257/isolated-intrapulmonary-vascular-dilatations-and-the-risk-of-developing-hepatopulmonary-syndrome-in-liver-transplant-candidates
#15
Manuel Mendizabal, David Goldberg, Federico Piñero, Diego Arufe, María José de la Fuente, Pablo Testa, Matías Coronel, Sergio Baratta, Luis Podestá, Michael Fallon, Marcelo Silva
BACKGROUND: The natural history of intrapulmonary vascular dilations (IPVD) and their impact on patient outcomes in the setting of portal hypertension has only been described in small series. AIMS: To assess the development of hepatopulmonary síndrome (HPS) in patients with isolated IPVD and to evaluate outcomes of IPVD and HPS among patients evaluated for liver transplantation (LT). MATERIAL AND METHODS: Data from a prospective cohort of patients evaluated for LT with standardized screening for HPS were analyzed...
August 1, 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/28604464/liver-transplantation-in-a-patient-with-antiphospholipid-syndrome-a-case-report
#16
Rachel C Steckelberg, Zarah D Antongiorgi, Randolph H Steadman
Antiphospholipid syndrome (APS) is an acquired thrombophilic disorder characterized by autoantibodies to cell membrane phospholipids. While altered coagulation can complicate end-stage liver disease, there are few reports describing the perioperative management for liver transplantation in recipients with a preexisting hypercoagulable disorder, such as APS. We present a patient with a history of APS, Budd-Chiari syndrome with cirrhosis, hepatopulmonary syndrome, and heparin-induced thrombocytopenia who underwent liver transplantation complicated by hepatic artery thrombosis...
September 1, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28591307/schistosomiasis-and-hepatopulmonary-syndrome-the-role-of-concomitant-liver-cirrhosis
#17
Liana Gonçalves-Macedo, Edmundo Pessoa Lopes, Ana Lucia Coutinho Domingues, Brivaldo Markman, Vitor Gomes Mota, Carlos Feitosa Luna
BACKGROUND: Hepatopulmonary syndrome (HPS) is defined as an oxygenation defect induced by intrapulmonary vasodilation in patients with liver disease or portal hypertension. It is investigated in patients with liver cirrhosis and less frequently in those with portal hypertension without liver cirrhosis, as may occur in hepatosplenic schistosomiasis (HSS). OBJECTIVES: To investigate the prevalence of HPS in patients with HSS, and to determine whether the occurrence of HPS is influenced by concomitant cirrhosis...
July 2017: Memórias do Instituto Oswaldo Cruz
https://www.readbyqxmd.com/read/28589869/-hepatopulmonary-syndrome-a-consideration-in-patients-with-liver-disease-accompanied-by-dyspnoea-and-hypoxaemia
#18
P V van Hirtum, G P Bootsma, R A de Man, C Th B M van Deursen, P I Bonta, M D de Kruif
BACKGROUND: Hepatopulmonary syndrome is a severe complication of liver disease, with greatly increased mortality. The syndrome is characterized by increased blood-flow, intrapulmonary vasodilatation and angiogenesis, leading to effects including the formation of shunts. This leads to a decrease in arterial oxygen pressure. Liver transplantation is the only effective treatment. CASE DESCRIPTION: A 74-year-old woman with cirrhosis of the liver attended the pulmonary outpatients' clinic with progressive dyspnoea, which worsened if she sat upright from a lying position (platypnoea)...
2017: Nederlands Tijdschrift Voor Geneeskunde
https://www.readbyqxmd.com/read/28556380/waitlist-outcomes-for-patients-relisted-following-failed-donation-after-cardiac-death-liver-transplant-implications-for-awarding-model-for-end-stage-liver-disease-exception-scores
#19
K P Croome, D D Lee, J H Nguyen, A P Keaveny, C B Taner
Understanding of outcomes for patients relisted for ischemic cholangiopathy following a donation after cardiac death (DCD) liver transplant (LT) will help standardization of a Model for End-Stage Liver Disease exception scheme for retransplantation. Early relisting (E-RL) for DCD graft failure caused by primary nonfunction (PNF) or hepatic artery thrombosis (HAT) was defined as relisting ≤14 days after DCD LT, and late relisting (L-RL) due to biliary complications was defined as relisting 14 days to 3 years after DCD LT...
September 2017: American Journal of Transplantation
https://www.readbyqxmd.com/read/28543822/good-for-the-lung-but-bad-for-the-liver-garlic-induced-hepatotoxicity-following-liver-transplantation
#20
S A Shaikh, S Tischer, E K Choi, R J Fontana
WHAT IS KNOWN AND OBJECTIVE: Limited data exist surrounding the metabolism and safety of garlic supplements. CASE DESCRIPTION: A patient with a history of hepatopulmonary syndrome (HPS) and orthotopic liver transplantation was admitted to our surgery transplant service with severe hypoxaemia. The patient was started on high-dose Garlicin Cardio(®) (Allium sativum) for HPS and soon after had elevated liver function tests. Garlicin Cardio(®) was discontinued and liver enzymes normalized...
May 25, 2017: Journal of Clinical Pharmacy and Therapeutics
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