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syndrome hepatopulmonary

Mamatha Bhat, Bianca M Arendt, Venkat Bhat, Eberhard L Renner, Atul Humar, Johane P Allard
The intestinal microbiome (IM) is altered in patients with cirrhosis, and emerging literature suggests that this impacts on the development of complications. The PubMed database was searched from January 2000 to May 2015 for studies and review articles on the composition, pathophysiologic effects and therapeutic modulation of the IM in cirrhosis. The following combination of relevant text words and MeSH terms were used, namely intestinal microbiome, microbiota, or dysbiosis, and cirrhosis, encephalopathy, spontaneous bacterial peritonitis, hepatorenal syndrome, variceal bleeding, hepatopulmonary syndrome, portopulmonary hypertension and hepatocellular carcinoma...
September 28, 2016: World Journal of Hepatology
Yoshiaki Takahashi, Toshiharu Matsuura, Yusuke Yanagi, Koichiro Yoshimaru, Tomoaki Taguchi
BACKGROUND/PURPOSE: There is currently no unified view regarding whether liver transplantation or splenectomy should be performed for hypersplenism before liver transplantation in biliary atresia (BA) patients. We herein describe the efficacy of splenectomy before liver transplantation. METHODS: Splenectomy was performed in ten patients with hypersplenism associated with BA. We retrospectively reviewed their perioperative and postoperative courses, the number of leukocytes and thrombocytes, and the MELD score...
September 26, 2016: Journal of Pediatric Surgery
Shikha S Sundaram, Cara L Mack, Amy G Feldman, Ronald J Sokol
Biliary Atresia is a progressive, fibro-obliterative disorder of the intra and extrahepatic bile ducts in infancy. The majority of affected children will eventually develop end-stage liver disease and require liver transplantation. Indications for liver transplant in biliary atresia include failed Kasai portoenterostomy, significant and recalcitrant malnutrition, recurrent cholangitis, and the progressive manifestations of portal hypertension. Extra-hepatic complications of this disease, such as hepatopulmonary syndrome and portopulmonary hypertension, are also indications for liver transplantation...
September 21, 2016: Liver Transplantation
Haruka Sato, Masanobu Miura, Nobuhiro Yaoita, Saori Yamamoto, Shunsuke Tatebe, Tatsuo Aoki, Kimio Satoh, Hideki Ota, Kei Takase, Koichiro Sugimura, Hiroaki Shimokawa
Cardiopulmonary abnormalities are often present in patients with liver diseases. We herein report a case of congenital portosystemic shunts complicated by hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PoPH). A 57-year-old woman complained of dyspnea and was subsequently diagnosed with HPS and PoPH caused by congenital portosystemic shunts. Although shunt closure by transcatheter embolization was successfully performed, her dyspnea worsened and pulmonary artery pressure and pulmonary vascular resistance elevated...
2016: Internal Medicine
Hai-Yong Chen, Yao-Min Chen, Jian Wu, Fu-Chun Yang, Zhen Lv, Xiao-Feng Xu, Shu-Sen Zheng, Sang-Sang Liao, Yi-Hui Luo
OBJECTIVE: This case-control study is designed to explore the relationship between microRNA-196a2 (miR-196a2) rs11614913 C > T polymorphism and the risk of hepatopulmonary syndrome (HPS) in liver cirrhosis METHODS: From January 2013 to January 2015, 163 liver cirrhosis patients with HPS (case group), 264 liver cirrhosis patients without HPS (control group) and 195 healthy people (normal group) were selected. DNA extraction Kit was used to extract plasma DNA from peripheral blood...
August 10, 2016: Hepatology Research: the Official Journal of the Japan Society of Hepatology
Parisá Khodayar-Pardo, Andrés Peña Aldea, Ana Ramírez González, Adela Meseguer Carrascosa, Cristina Calabuig Bayo
Extrahepatic portal vein obstruction, although rare in children, is a significant cause of portal hypertension (PHT) leading to life-threatening gastrointestinal bleeding in the pediatric age group. PHT may also lead to other complications such as hyperesplenism, cholangyopathy, ascites, and even hepatopulmonary syndrome and portopulmonary hypertension that may require organ transplantation. Herein we report the case of an asymptomatic 11-month-old infant wherein a hepatomegaly and cavernous transformation of the portal vein was detected by liver ultrasound...
May 2016: Case Reports in Gastroenterology
S T Lauren, S O Simons, A I J Arens
A 42-year-old man with a medical history of liver cirrhosis and portal hypertension was admitted to the hospital because of slowly progressive shortness of breath and hypoxemia. The diagnosis hepatopulmonary syndrome was confirmed by a pulmonary perfusion scan with 99m Tc-albumin. The scan showed a right-left shunt, because the 99m Tc-albumin transited the lungs and appeared in the brain, the thyroid gland, the kidneys and the spleen. The patient received a liver transplantation, which is considered the only definitive treatment...
2016: Nederlands Tijdschrift Voor Geneeskunde
H Y Ma, X Li
Hepatopulmonary syndrome is a disease with abnormal gas exchange caused by liver diseases, with the feature of abnormal oxygenation caused by intrapulmonary vasodilation. This article introduces the pathogenesis, natural course, clinical manifestations, diagnostic methods, and therapeutic measures of this disease and discusses potential therapeutic measures besides liver transplantation.
May 20, 2016: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
Salim R Surani, Yamely Mendez, Humayun Anjum, Joseph Varon
Severe chronic liver disease (CLD) may result from portal hypertension, hepatocellular failure or the combination of both. Some of these patients may develop pulmonary complications independent from any pulmonary pathology that they may have. Among them the hepatopulmonary syndrome (HPS), portopulmonary hypertension (PPH) and hepatic hydrothorax (HH) are described in detail in this literature review. HPS is encountered in approximately 15% to 30% of the patients and its presence is associated with increase in mortality and also requires liver transplantation in many cases...
July 14, 2016: World Journal of Gastroenterology: WJG
Israel Grilo-Bensusan, Juan Manuel Pascasio-Acevedo
Hepatopulmonary syndrome (HPS) is characterized by abnormalities in blood oxygenation caused by the presence of intrapulmonary vascular dilations (IPVD) in the context of liver disease, generally at a cirrhotic stage. Knowledge about the subject is still only partial. The majority of the information about the etiopathogenesis of HPS has been obtained through experiments on animals. Reported prevalence in patients who are candidates for a liver transplantation (LT) varies between 4% and 32%, with a predominance of mild or moderate cases...
July 7, 2016: World Journal of Gastroenterology: WJG
S Sreekantam, I Nicklaus-Wollenteit, J Orr, K Sharif, S Vijay, P J McKiernan, S Santra
Late-onset LAL deficiency, previously referred to as cholesteryl ester storage disorder, is a rare lysosomal storage disorder characterized by accumulation of cholesteryl esters. It has a heterogeneous clinical phenotype including abdominal pain, poor growth, hyperlipidemia with vascular complications and hepatosplenomegaly. End-stage liver disease may occur, but there are few reports of successful LT. There are also concerns that systemic manifestations of the disease might persist post-LT. We report a case with excellent outcome eight yr following LT...
September 2016: Pediatric Transplantation
Yingming Amy Chen, Vikramaditya Prabhudesai, Helene Castel, Samir Gupta
BACKGROUND: Hepatopulmonary syndrome (HPS) is defined by liver dysfunction, intrapulmonary vascular dilatations, and impaired oxygenation. The gold standard for detection of intrapulmonary vascular dilatations in HPS is contrast echocardiography. However, two small studies have suggested that patients with HPS have larger segmental pulmonary arterial diameters than both normal subjects and normoxemic subjects with cirrhosis, when measured by CT. We sought to compare CT imaging-based pulmonary vasodilatation in patients with HPS, patients with liver dysfunction without HPS, and matching controls on CT imaging...
2016: PloS One
X Li, Y Chen, L Wang, G Shang, C Zhang, Z Zhao, H Zhang, A Liu
Quercetin shows protective effects against hepatopulmonary syndrome (HPS), as demonstrated in a rat model. However, whether these effects involve pulmonary vascular angiogenesis in HPS remains unclear. Therefore, this study aimed to assess the effect of quercetin on pulmonary vascular angiogenesis and explore the underlying mechanisms. Male Sprague-Dawley rats weighing 200-250 g underwent sham operation or common bile duct ligation (CBDL). Two weeks after surgery, HIF-1α and NFκB levels were assessed in rat lung tissue by immunohistochemistry and western blot...
July 4, 2016: Brazilian Journal of Medical and Biological Research, Revista Brasileira de Pesquisas Médicas e Biológicas
Zulkifli Amin, Hilman Zulkifli Amin, Nadim Marchian Tedyanto
Hepatopulmonary syndrome (HPS) is a pulmonary complication of liver disease characterized by arterial hypoxemia. Mechanisms related to this event are diffusion-perfusion flaw, ventilation-perfusion (V/Q) mismatch, and direct arteriovenous shunts. Diagnosis of HPS is based on the presence of liver disease or portal hypertension, increased alveolar-arterial (A-a) PO2, and intrapulmonary vascular dilatations (IPVD). Lung transplantation (LT) remains the most effective therapy for HPS. In spite of its poor prognosis, we could improve the quality of life and survival rate of patients...
April 2016: Romanian Journal of Internal Medicine, Revue Roumaine de Médecine Interne
Vijaya S Ramalingam, Sikandar Ansari, Micah Fisher
Cirrhosis, the twelfth leading cause of death, accounts for 1.1% of all deaths in the United States. Although there are multiple pulmonary complications associated with liver disease, the most important complications that cause significant morbidity and mortality are hepatopulmonary syndrome, hepatic hydrothorax, and portopulmonary hypertension. Patients with cirrhosis who complain of dyspnea should be evaluated for these complications. This article reviews these complications.
July 2016: Critical Care Clinics
Michael J Krowka, Michael B Fallon, Steven M Kawut, Valentin Fuhrmann, Julie K Heimbach, Michael A E Ramsay, Olivier Sitbon, Ronald J Sokol
Two distinct pulmonary vascular disorders, hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH) may occur as a consequence of hepatic parenchymal or vascular abnormalities. HPS and POPH have major clinical implications for liver transplantation. A European Respiratory Society Task Force on Pulmonary-Hepatic Disorders convened in 2002 to standardize the diagnosis and guide management of these disorders. These International Liver Transplant Society diagnostic and management guidelines are based on that task force consensus and should continue to evolve as clinical experience dictates...
July 2016: Transplantation
Gaetano Bertino, Graziella Privitera, Francesco Purrello, Shirin Demma, Emanuele Crisafulli, Luisa Spadaro, Nikolaos Koukias, Emmanuel A Tsochatzis
Liver cirrhosis is a major cause of morbidity and mortality worldwide, mainly due to complications of portal hypertension. In this article, we review the current understanding on the pathophysiology, the diagnostic criteria and the available therapeutic options for patients with emerging hepatic syndromes in cirrhosis, namely the hepatorenal, hepato-adrenal and hepatopulmonary syndrome. The hepatorenal syndrome is a well-recognized complication of advanced cirrhosis and is usually associated with an accelerated course to death unless liver transplantation is performed...
October 2016: Internal and Emergency Medicine
Cinta Calvo-Morón, Francisco Javier García-Gómez, Pablo Antonio de la Riva-Pérez, Juan Castro-Montaño
No abstract text is available yet for this article.
August 2016: Digestive and Liver Disease
Ali Mokhtarifar, Hamidreza Azimi, Abbas Esmaeelzadeh, Mahmood Ebrahimi, Seyed Ahmad Eghbali
No abstract text is available yet for this article.
February 2016: Hepatitis Monthly
P B Sriram, R Sindhuja, M Natarajan, P S Arul Rajamurugan, B Palanikumar
We report the case of a 52-year-old female, a known case of Chronic Liver Disease with portal hypertension. She presented with dyspnoea, platypnoea, melena, cyanosis, clubbing and orthodeoxia. She had oesophageal varices and splenomegaly indicating portal hypertension. Her arterial blood gas revealed hypoxaemia and orthodeoxia. From this clinical background and investigation, a diagnosis of hepatopulmonary syndrome was made. Patient was managed conservatively as she was not willing for liver transplantation...
March 2016: Journal of Clinical and Diagnostic Research: JCDR
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