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Hepatopulmonary syndrome

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https://www.readbyqxmd.com/read/29128026/hepatopulmonary-syndrome-and-portopulmonary-hypertension-implications-for-liver-transplantation
#1
REVIEW
Shaz Iqbal, Kerri Akaya Smith, Vandana Khungar
Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PoPH) represent serious pulmonary complications of advanced liver diseases. Orthotopic liver transplantation (OLT) is capable of completely resolving the underlying abnormalities associated with HPS. On the other hand, post-OLT response in patients with PoPH is less predictable, although heavily influenced by pre-OLT mean pulmonary arterial pressure. It remains the case that the opportunity to reverse 2 potentially fatal organ dysfunctions in the liver and the lung make HPS and PoPH more than worthy for further clinical investigations...
December 2017: Clinics in Chest Medicine
https://www.readbyqxmd.com/read/29092970/extracorporeal-membrane-oxygenation-for-post-transplant-hypoxaemia-following-very-severe-hepatopulmonary-syndrome
#2
Lakshmi Kumar, Dinesh Balakrishnan, Rekha Varghese, Sudhindran Surendran
Hepatopulmonary syndrome (HPS) associated with end-stage liver disease has a high morbidity when room air PaO2 is less than 50 mm Hg. Safe levels of oxygenation to facilitate transplantation have not been defined despite advancement in care. Postoperatively, hypoxaemia worsens due to ventilation perfusion mismatch contributed by postoperative pulmonary vasoconstriction and due to decrease in endogenous nitric oxide. A 16-year-old boy with cirrhosis presented with HPS and a PaO2 of 37 mm Hg on room air and underwent living donor liver transplant...
November 1, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29091154/ventilatory-support-and-hospital-stay-after-liver-transplant-in-cirrhotic-patients-with-hepatopulmonary-syndrome
#3
José Leonardo Faustini Pereira, Lucas Homercher Galant, Eduardo Garcia, Luis Henrique Telles da Rosa, Ajácio Bandeira de Mello Brandão, Cláudio Augusto Marroni
OBJECTIVE: To compare mechanical ventilation time, need for non-invasive ventilation, length of intensive care unit stay, and hospital stay after liver transplant in cirrhotic patients with and with no diagnosis of hepatopulmonary syndrome. METHODS: This was a prospective cohort study with a convenience sample of 178 patients (92 with hepatopulmonary syndrome) who were diagnosed as alcoholic or hepatitis C virus cirrhosis. The statistical analysis included Kolmogorov-Smirnov test and Students t test...
July 2017: Einstein
https://www.readbyqxmd.com/read/29023811/placental-growth-factor-inhibition-targets-pulmonary-angiogenesis-and-represents-a-novel-therapy-for-hepatopulmonary-syndrome-in-mice
#4
Sarah Raevens, Anja Geerts, Annelies Paridaens, Sander Lefere, Xavier Verhelst, Anne Hoorens, Jo Van Dorpe, Tania Maes, Ken R Bracke, Christophe Casteleyn, Bart Jonckx, Thomas Horvatits, Valentin Fuhrmann, Hans Van Vlierberghe, Christophe Van Steenkiste, Lindsey Devisscher, Isabelle Colle
Hepatopulmonary syndrome (HPS) is a severe complication of cirrhosis with increased risk of mortality. Pulmonary microvascular alterations are key features of HPS, but underlying mechanisms are incompletely understood, and studies on HPS are limited to rats. Placental growth factor (PlGF), a pro-angiogenic molecule that is selectively involved in pathological angiogenesis, may play an important role in HPS development, however, its role has never been investigated. In this study, we validated an HPS model by common bile duct ligation (CBDL) in mice, investigated the kinetic changes in pulmonary angiogenesis and inflammation during HPS development, and provide evidence for a novel therapeutic strategy by targeting pathological angiogenesis...
October 10, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28987478/clinical-impact-of-intrapulmonary-vascular-dilatation-in-liver-transplant-candidates
#5
Hilary M DuBrock, Michael J Krowka, Kimberly A Forde, Karen Krok, Mamta Patel, Tiffany Sharkoski, Michael Sprys, Grace Lin, Jae K Oh, Carl D Mottram, Paul D Scanlon, Michael B Fallon, Steven M Kawut
BACKGROUND: Intrapulmonary vascular dilatations (IPVD) are frequently detected in patients with liver disease by the delayed appearance of microbubbles on contrast-enhanced echocardiography. IPVD with an elevated alveolar-arterial (A-a) gradient define hepatopulmonary syndrome, however the importance of IPVD in the absence of abnormal gas exchange is unknown. We aimed to determine the clinical impact of IPVD in patients with liver disease. METHODS: We performed a cross-sectional study within the Pulmonary Vascular Complications of Liver Disease (PVCLD2) Study, a multicenter prospective cohort study of patients being evaluated for liver transplantation...
October 5, 2017: Chest
https://www.readbyqxmd.com/read/28983709/late-complications-of-biliary-atresia-hepatopulmonary-syndrome-and-portopulmonary-hypertension
#6
Frederick M Karrer, Bradley J Wallace, Arturo E Estrada
Children with biliary atresia (BA) following Kasai portoenterostomy have a high risk for portal hypertension, however, while variceal and hemorrhagic complications have been more commonly studied, less frequent but no less possibly devastating complications of hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPH) remain less well understood. HPS and PPH both occur in a setting of portal hypertension, however, paradoxically patients with HPS develop pulmonic vasculature dilation leading to shunting and hypoxia, while those with PPH develop an opposite progression of pulmonary vasoconstriction eventually leading to cor pulmonale and decompensation...
December 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28972388/hepatopulmonary-syndrome-which-blood-gas-analysis-criteria-and-position-should-we-use-for-diagnosis
#7
Israel Grilo, Juan Manuel Pascasio, Francisco-Jesús López-Pardo, Francisco Ortega-Ruiz, Juan Luis Tirado, José Manuel Sousa, María José Rodríguez-Puras, María Teresa Ferrer, Miguel Ángel Gómez-Bravo, Antonio Grilo
INTRODUCTION: Different blood gas criteria have been used in the diagnosis of hepatopulmonary syndrome (HPS). PATIENTS AND METHODS: Arterial blood gases were prospectively evaluated in 194 cirrhotic candidates for liver transplantation (LT) in the supine and seated position. Three blood gas criteria were analyzed: classic (partial pressure of oxygen [PaO2] < 70 mmHg and/or alveolar-arterial gradient of oxygen [A-a PO2] ≥ 20 mmHg), modern (A-a PO2 ≥ 15 mmHg or ≥ 20 mmHg in patients over 64) and the A-a PO2 ≥ threshold value adjusted for age...
October 3, 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/28969989/the-effects-of-pioglitazone-in-cirrhotic-rats-with-hepatopulmonary-syndrome
#8
Tsung-Yi Cheng, Wen-Shin Lee, Hui-Chun Huang, Fa-Yauh Lee, Ching-Chih Chang, Han-Chieh Lin, Shou-Dong Lee
BACKGROUND: Hepatopulmonary syndrome (HPS) is characterized by oxygen desaturation and increased intrapulmonary shunting formation in cirrhosis. Due to an unclarified mechanism, there is still no effective therapy except liver transplantation. Recent studies revealed that pulmonary angiogenesis may participate in pathogenesis, in which nitric oxide (NO) and vascular endothelial growth factor (VEGF) play roles. Pioglitazone, a peroxisome proliferator-activated receptor gamma agonist, exerts anti-angiogenesis effect...
November 2017: Journal of the Chinese Medical Association: JCMA
https://www.readbyqxmd.com/read/28961571/screening-for-hepatopulmonary-syndrome-in-cirrhotic-patients-using-technetium-99m-macroaggregated-albumin-perfusion-lung-scan-tc-maa-diagnostic-approach-and-clinical-correlations
#9
Maria Fragaki, Dimitra Sifaki-Pistolla, Dimitrios N Samonakis, Mairi Koulentaki, Sofia Koukouraki, Maria Stathaki, Elias Kouroumalis
BACKGROUND AND AIMS: The aims of this study were to prospectively screen cirrhotic patients with arterial blood gas test and albumin perfusion scan, identify those fulfilling the classic hepatopulmonary syndrome (HPS) criteria, correlate with clinical parameters, and evaluate the survival of patients with HPS compared with those without HPS in a genetically homogenous Cretan cirrhotic population. MATERIALS AND METHODS: Data on consecutive 102 patients within 1 year were collected and analyzed...
September 28, 2017: Journal of Clinical Gastroenterology
https://www.readbyqxmd.com/read/28954708/pioglitazone-decrease-intrapulmonary-shunt-in-biliary-cirrhotic-rats-with-hepatopulmonary-syndrome
#10
EDITORIAL
Hung-Cheng Tsai, Ying-Ying Yang
No abstract text is available yet for this article.
November 2017: Journal of the Chinese Medical Association: JCMA
https://www.readbyqxmd.com/read/28948051/persistent-unexplained-dyspnea-a-case-of-hepatopulmonary-syndrome
#11
Alfonso Campanile, Alessandro Colombo, Maurizio Del Pinto, Claudio Cavallini
Regarding a patient with dyspnea, the history and physical examination often lead to the correct diagnosis. In some circumstances, when more than one underlying disease is present, the diagnostic process can be more challenging. We describe an unusual case of dyspnea and persistent hypoxemia related to a hepatopulmonary syndrome in a 53-year-old patient with known heart failure and chronic liver disease. Initially managed with intravenous diuretic therapy, due to signs of lung and peripheral congestion, our patient did not improve as expected; therefore we performed more advanced studies with a chest-abdomen CT scan and a right heart catheterization...
2017: Case Reports in Cardiology
https://www.readbyqxmd.com/read/28884036/severe-hepatopulmonary-syndrome-in-a-child-with-caroli-syndrome
#12
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
#13
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
#14
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
#15
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
#16
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...
October 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
#17
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...
November 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28759737/differential-diagnosis-of-hepatopulmonary-syndrome-hps-portopulmonary-hypertension-pph-and-hereditary-hemorrhagic-telangiectasia-hht
#18
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
#19
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...
October 1, 2017: Experimental Cell Research
https://www.readbyqxmd.com/read/28705726/dual-organ-beneficial-effects-of-metformin-in-cirrhotic-rats-with-hepatopulmonary-syndrome
#20
EDITORIAL
Chih-Wei Liu, Ying-Ying Yang
No abstract text is available yet for this article.
August 2017: Journal of the Chinese Medical Association: JCMA
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