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https://www.readbyqxmd.com/read/28414743/the-et-1-mediated-carbonylation-and-degradation-of-anxa1-induce-inflammatory-phenotype-and-proliferation-of-pulmonary-artery-smooth-muscle-cells-in-hps
#1
Jing He, Bin Yi, Yang Chen, Qing Huang, Huan Wang, Kaizhi Lu, Weiling Fu
Hepatopulmonary syndrome (HPS) is a serious complication of advanced liver disease, which markedly increases mortality. Pulmonary vascular remodelling (PVR) induced by circulating mediators plays an important role in the pathogenesis of HPS, while the underlying mechanism remains undefined. In the present study, we reported that endothelin-1 (ET-1) is up-regulated and annexin A1(ANXA1) is down-regulated in HPS rat, and ET-1 decreases the ANXA1 expression in a dose-dependent manner in rat pulmonary arterial smooth muscle cells (PASMCs)...
2017: PloS One
https://www.readbyqxmd.com/read/28302500/development-and-evaluation-of-an-online-resource-for-hepatopulmonary-syndrome-patients-and-caregivers-www-hpscare-com
#2
Justina Marianayagam, Katherine Griffin, Jenna Sykes, Samir Gupta
No abstract text is available yet for this article.
March 14, 2017: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28301945/the-utility-of-the-macro-aggregated-albumin-lung-perfusion-scan-in-the-diagnosis-and-prognosis-of-hepatopulmonary-syndrome-in-cirrhotic-patients-candidates-for-liver-transplantation
#3
Israel Grilo Bensusan, Juan Manuel Pascasio, Juan Luis Tirado, Francisco-Jesús López-Pardo, Francisco Ortega-Ruiz, José Manuel Sousa, María José Rodríguez-Puras, María Teresa Ferrer, Miguel Ángel Gómez-Bravo, Antonio Grilo Reina
BACKGROUND: The macro-aggregated albumin lung perfusion scan (99mTc-MAA) is a diagnostic method for hepatopulmonary syndrome (HPS). GOAL: To determine the sensitivity of 99mTc-MAA in diagnosing HPS, to establish the utility of 99mTc-MAA in determining the influence of HPS on hypoxemia in patients with concomitant pulmonary disease and to determine the correlation between 99mTc-MAA values and other respiratory parameters. METHODS: Data from 115 cirrhotic patients who were eligible for liver transplantation (LT) were prospectively analyzed...
March 17, 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/28243044/hepatopulmonary-syndrome-is-associated-with-the-presence-of-hepatocellular-carcinoma-in-patients-with-decompensated-cirrhosis
#4
Stergios Soulaidopoulos, Ioannis Goulis, George Giannakoulas, Theofilos Panagiotidis, Petros Doumtsis, Areti Karasmani, Theodora Oikonomou, Theodora Tzoumari, Haralampos Karvounis, Εvangelos Cholongitas
BACKGROUND: Hepatopulmonary syndrome (HPS) is a relatively common complication in patients with decompensated cirrhosis. Our aim was to evaluate the prevalence of HPS, its clinical impact, and the possible association between HPS and characteristics of patients with decompensated cirrhosis. METHODS: Patients with stable decompensated cirrhosis admitted to our department and assessed for HPS were included. For each patient, several clinical, laboratory and echocardiographic parameters as well as renal function were recorded...
2017: Annals of Gastroenterology: Quarterly Publication of the Hellenic Society of Gastroenterology
https://www.readbyqxmd.com/read/28222032/unexplained-cyanosis-caused-by-hepatopulmonary-syndrome-in-a-girl-with-apeced-syndrome
#5
Fatih Celmeli, Abdullah Kocabas, Ishak A Isik, Mesut Parlak, Kai Kisand, Serdar Ceylaner, Doga Turkkahraman
Autoimmune polyendocrinopathy, candidiasis and ectodermal dystrophy (APECED) is a rare but devastating primary immunodeficiency disease caused by loss-of-function mutations in autoimmune regulator (AIRE) gene on chromosome 21q22.3. The clinical spectrum of the disease is characterized by a wide heterogeneity because of autoimmune reactions toward different endocrine and non-endocrine organs. Here, we report a 17-year-old Turkish girl diagnosed with APECED at 9 years in whom a novel homozygote mutation in AIRE gene p...
March 1, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28196822/hepatopulmonary-fusion-a-rare-and-potentially-lethal-association-with-right-congenital-diaphragmatic-hernia
#6
REVIEW
Vishesh Jain, Devendra Kumar Yadav, Devasenathipathy Kandasamy, Devendra Kumar Gupta
Hepatopulmonary fusion is an extremely rare accompaniment of right congenital diaphragmatic hernia. It is associated with abnormal systemic arterial supply and venous drainage of the right lung along with congenital heart disease. Children with this condition have a comparatively poor prognosis. We report a case of right congenital diaphragmatic hernia with hepatopulmonary fusion along with review of the literature with stress on diagnosis and management.
February 14, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28078819/live-donor-liver-transplantation-for-a-child-presented-with-severe-hepatopulmonary-syndrome-and-nodular-liver-lesions-due-to-abernethy-malformation
#7
Cahit Yilmaz, Zafer Onen, Rasim Farajov, Onur S Duygu, Latife Doganay, Alper Yuksel, Mehmet Alper, Murat Zeytunlu, Cigdem Arikan, Murat Kilic
A 15-year-old boy first presented with severe lung lesions and hypoxia and he was considered as a lung transplant candidate. Upon evaluation, hepatopulmonary syndrome, multiple nodular liver lesions, and Abernethy type 1b malformation were diagnosed. The patient underwent successful right lobe live donor liver transplantation, and all of the symptoms disappeared soon after the transplant. He is currently alive and well with excellent liver and lung functions 4 years after surgery.
January 12, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28042141/prolonged-unexplained-hypoxemia-as-initial-presentation-of-cirrhosis-a-case-report
#8
Anand Puttappa, Kumaraswamy Sheshadri, Aurelie Fabre, Georgina Imberger, John Boylan, Silke Ryan, Masood Iqbal, Niamh Conlon
BACKGROUND Hepatopulmonary syndrome (HPS) is a pulmonary complication of advanced liver disease with dyspnea as the predominant presenting symptom. The diagnosis of HPS can often be missed due to its nonspecific presentation and the presence of other comorbidities. CASE REPORT We present an interesting case of an obese 43-year-old man who presented with progressive, unexplained hypoxemia and shortness of breath in the absence of any symptoms or signs of chronic liver disease. After extensive cardiopulmonary investigations, he was diagnosed with severe HPS as a result of non-alcoholic steatohepatitis (NASH) leading to cirrhosis...
January 2, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28039901/living-donor-liver-transplantation-in-a-pediatric-patient-with-preexisting-yolk-sac-tumor
#9
Taiichi Wakiya, Yoshikazu Toyoki, Keinosuke Ishido, Daisuke Kudo, Norihisa Kimura, Shinji Tsutsumi, Tadashi Odagiri, Akiko Suto, Chiaki Uchida, Kenichi Hakamada
There is ongoing discussion regarding the indications and timing of LT for patients with a preexisting extrahepatic malignancy. We herein report a pediatric case that underwent LDLT after therapy for YST. The patient, a 13-year-old female with biliary atresia, had undergone portoenterostomy at 2 months of age. She developed a left ovarian tumor with a high serum alpha-fetoprotein concentration at 10 years of age. She underwent left oophorectomy and was diagnosed with ovarian YST (Stage I). After surgery, hepatopulmonary syndrome progressed gradually...
December 30, 2016: Pediatric Transplantation
https://www.readbyqxmd.com/read/28009313/character-and-frequency-of-the-variations-of-co-and-polymorbid-syntropic-extrahepatic-lesions-and-their-dependence-on-the-hepatopulmonary-syndrome-severity-degree-in-cirrhotic-patients
#10
O Abrahamovych, M Abrahamovych, S Tolopko, O Fayura, M Ferko
In this study, we comprehensively examined 93 patients with liver cirrhosis, selected in a randomized manner, with the preliminary stratification by the presence of hepatopulmonary syndrome with the aim to study the nature and frequency of extrahepatic syntropic lesions of organs and systems and their dependence on the severity of lung injury that is necessary for the appointment of the most effective individualized comprehensive treatment. The results of our calculations showed that with the increasing of the hepatopulmonary syndromeseverity degree, the nature and the frequency of the syntropic co- and polymorbid functional and organic extrahepatic lesions of the organs and body systems was significantly (p<0...
November 2016: Georgian Medical News
https://www.readbyqxmd.com/read/28009008/down-regulation-of-common-nf%C3%AE%C2%BAb-inos-pathway-by-chronic-thalidomide-treatment-improves-hepatopulmonary-syndrome-and-muscle-wasting-in-rats-with-biliary-cirrhosis
#11
Tzu-Hao Li, Pei-Chang Lee, Kuei-Chuan Lee, Yun-Cheng Hsieh, Chang-Youh Tsai, Ying-Ying Yang, Shiang-Fen Huang, Tung-Hu Tsai, Shie-Liang Hsieh, Ming-Chih Hou, Han-Chieh Lin, Shou-Dong Lee
Thalidomide can modulate the TNFα-NFκB and iNOS pathway, which involve in the pathogenesis of hepatopulmonary syndrome (HPS) and muscle wasting in cirrhosis. In bile duct ligated-cirrhotic rats, the increased circulating CD16(+) (inflammatory) monocytes and its intracellular TNFα, NFκB, monocyte chemotactic protein (MCP-1) and iNOS levels were associated with increased circulating MCP-1/soluable intercellular cell adehesion molecule-1 (sICAM-1), pulmonary TNFα/NOx, up-regulated M1 polarization, exacerbated angiogenesis and hypoxemia (increased AaPO2) in bronchoalveolar lavage (BAL) fluid and pulmonary homogenates...
December 23, 2016: Scientific Reports
https://www.readbyqxmd.com/read/28008407/severe-hepatopulmonary-syndrome-in-an-adolescent-patient-with-non-cirrhotic-portal-fibrosis
#12
Vikrant Sood, S Rajesh, Bikrant Bihari Lal, Dinesh Rawat, Seema Alam
No abstract text is available yet for this article.
August 2016: ACG Case Reports Journal
https://www.readbyqxmd.com/read/28003725/neonatal-cholestasis-as-initial-presentation-of-portosystemic-shunt-a-case-report
#13
Güzide Doğan, Fatih Düzgün, Serdar Tarhan, Yeliz Çağan Appak, Erhun Kasırga
Congenital intrahepatic portosystemic shunts are rare in children. Portosystemic venous malformations are characterized by extreme clinical variability. We report a full-term 33-day-old male infant presenting with neonatal jaundice. On physical examination, he had generalized icterus and the liver was palpable 3.5 cm below the right costal margin. He had no other symptoms. Laboratory tests showed AST 632 U/L, ALT 198 U/L, total bilirubin 12.1 mg/dL, conjugated bilirubin 10.2 mg/dL, alkaline phosphatase 753 U/L, GGT 47 U/L and glucose 67 U/L...
December 2016: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/27981304/the-impact-of-hypoxaemia-on-the-outcome-in-liver-cirrhosis
#14
Andrei Mihai Voiosu, Theodor Alexandru Voiosu, Bianca Smarandache, Aurelia Rădoi, Radu Bogdan Mateescu, Cristian Răsvan Băicuş, Mihail Radu Voiosu, Mihai Mircea Diculescu
BACKGROUND AND AIMS: Prognostic factors for poor evolution are critical in the setting of limited access to liver transplantation for patients with cirrhosis. We aimed to investigate the impact of hypoxaemia on the outcome in cirrhosis and the evolution of arterial oxygen tension during long-term follow-up in these patients. METHODS: Consecutive cirrhotic patients were prospectively enroled and followed-up in our tertiary referral center. Clinical features, biological tests, arterial blood gases, NT-proBNP levels, pulse oximetry measurements, 12-lead ECG, and transthoracic contrast echocardiography were documented on enrolment...
December 2016: Journal of Gastrointestinal and Liver Diseases: JGLD
https://www.readbyqxmd.com/read/27951601/serum-bile-acids-in-patients-with-hepatopulmonary-syndrome
#15
Thomas Horvatits, Andreas Drolz, Karoline Rutter, Kevin Roedl, Günter Fauler, Christian Müller, Stefan Kluge, Michael Trauner, Peter Schenk, Valentin Fuhrmann
Background Hepatopulmonary syndrome (HPS) occurs in 20 - 30 % of patients with cirrhosis and is associated with increased mortality. Cholestasis and accumulation of bile acids (BAs) play a major role in chronic liver disease. Aim We aimed to evaluate the clinical role of serum BAs in patients with HPS. Methods Seventy-four patients with cirrhosis were included in this prospective study. Marker for cholestasis as total and individual serum BAs, bilirubin, alkaline phosphatase (AP), and gamma-glutamyl transpeptidase (GGT) were analyzed in patients screened for HPS...
April 2017: Zeitschrift Für Gastroenterologie
https://www.readbyqxmd.com/read/27879294/cxcr2-is-involved-in-pulmonary-intravascular-macrophage-accumulation-and-angiogenesis-in-a-rat-model-of-hepatopulmonary-syndrome
#16
Xujiong Li, Yunxia Chen, Yongli Chang, Shufen Li, Zhongfu Zhao, Huiying Zhang
Hepatopulmonary syndrome (HPS) is a lung complication in various liver diseases, with high incidence, poor prognosis and no effective non-surgical treatments in patients with hepatocirrhosis. Therefore, assessing HPS pathogenesis to explore proper therapy strategies is clinically relevant. In the present study, male Sprague-Dawley rats underwent sham operation or common bile duct ligation (CBDL). Two weeks post-surgery, the following groups were set up for 2 weeks of treatment: sham + normal saline, CBDL + CXCR2 antagonist SB225002, CBDL + tumour necrosis factor α (TNF-α) antagonist PTX and CBDL + normal saline groups...
January 1, 2017: Clinical Science (1979-)
https://www.readbyqxmd.com/read/27851335/1700-va-ecmo-for-a-pediatric-patient-with-worsening-hepatopulmonary-syndrome-following-liver-transplant
#17
Laura Schroeder, Gregory Yurasek, Khalid Khan, David Steinhorn
No abstract text is available yet for this article.
December 2016: Critical Care Medicine
https://www.readbyqxmd.com/read/27833324/hepatocellular-carcinoma-with-intra-atrial-tumor-extension-identified-on-99m-tc-labeled-macroaggregated-albumin-single-photon-emission-computed-tomography-computed-tomography
#18
Piyush Chandra, Sneha Shah, Nilendu Purandare, Archi Agrawal, Venkatesh Rangarajan
(99)mTc macroaggregated albumin (MAA) scintigraphy is always performed before administration of (90)Y--microspheres for the treatment of liver tumors for hepatopulmonary shunt calculation. Tumor thrombus visualization in the hepatic vasculature is an infrequent finding on the (99)m Tc--MAA single photon emission computed tomography. We present a rare case of a hepatocellular carcinoma extending as a tumor thrombus through hepatic vein, inferior vena cava into the right atrium.
October 2016: Indian Journal of Nuclear Medicine: IJNM: the Official Journal of the Society of Nuclear Medicine, India
https://www.readbyqxmd.com/read/27816135/anesthesia-for-patients-with-concomitant-hepatic-and-pulmonary-dysfunction
#19
REVIEW
Geraldine C Diaz, Michael F O'Connor, John F Renz
Hepatic function and pulmonary function are interrelated with failure of one organ system affecting the other. With improved therapies, patients with concomitant hepatic and pulmonary failure increasingly enjoy a good quality of life and life expectancy. Therefore, the prevalence of such patients is increasing with more presenting for both emergent and elective surgical procedures. Hypoxemia requires a thorough evaluation in patients with end-stage liver disease. The most common etiologies respond to appropriate therapy...
December 2016: Anesthesiology Clinics
https://www.readbyqxmd.com/read/27777895/hypoxic-hepatitis-a-review-and-clinical-update
#20
REVIEW
Najeff Waseem, Po-Hung Chen
Hypoxic hepatitis (HH), also known as ischemic hepatitis or shock liver, is characterized by a massive, rapid rise in serum aminotransferases resulting from reduced oxygen delivery to the liver. The most common predisposing condition is cardiac failure, followed by circulatory failure as occurs in septic shock and respiratory failure. HH does, however, occur in the absence of a documented hypotensive event or shock state in 50% of patients. In intensive care units, the incidence of HH is near 2.5%, but has been reported as high as 10% in some studies...
September 28, 2016: Journal of Clinical and Translational Hepatology
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