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portal hypertensive syndrome

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https://www.readbyqxmd.com/read/29024353/exercise-and-physical-activity-for-patients-with-esld-improving-functional-status-and-sarcopenia-while-on-the-transplant-waitlist
#1
REVIEW
Andrés Duarte-Rojo, Astrid Ruiz-Margáin, Aldo J Montaño-Loza, Ricardo Macías-Rodríguez, Arny Ferrando, W Ray Kim
Sarcopenia and physical deconditioning are frequent complications in patients with cirrhosis and end-stage liver disease (ESLD). They are the end result of impaired dietary intake, chronic inflammation, altered macro- and micronutrient metabolism, and low physical activity. Frailty is the end result of prolonged sarcopenia and physical deconditioning. It severely affects a patient's functional status, and presents in about 1 in 5 patients on the liver transplant (LT) waitlist. Sarcopenia, poor physical fitness/cardiopulmonary endurance, and frailty are all associated with increased mortality in ESLD...
October 11, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28983709/late-complications-of-biliary-atresia-hepatopulmonary-syndrome-and-portopulmonary-hypertension
#2
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...
October 5, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28981166/terlipressin-current-and-emerging-indications-in-chronic-liver-disease
#3
REVIEW
Timothy Papaluca, Paul Gow
Terlipressin is an analogue of vasopressin that has potent vasoactive properties and has been available for use in most countries for nearly two decades. It has both established roles and emerging indications in the management of complications of decompensated chronic liver disease. We explore historic and emerging literature regarding the use of terlipressin for a range of indications including hepatorenal syndrome, portal hypertensive bleeding and disruptions in sodium homeostasis. Novel methods of infusion based terlipressin administration including the beneficial effect in reduction of adverse events are explored, in addition to new indications for the use of terlipressin in decompensated cirrhosis in an outpatient setting...
October 5, 2017: Journal of Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28972485/peculiarities-of-the-clinical-course-of-non-alcoholic-steatohepatitis-against-the-background-of-the-chronic-kidney-disease-of-the-i-iii-stage-with-secondary-arterial-hypertension
#4
O Hukhlina, A Antoniv, I Dudka, T Dudka, O Mandryk
The article addresses the theoretical generalization of the clinical study of non-alcoholic steatohepatitis peculiarities in comorbidity with obesity and chronic kidney disease of the І-ІІІ stage, characterized by higher frequency and intensity of clinical and biochemical syndromes, the manifestation of which is likely to increase the occurrence of secondary arterial hypertension (portal hypertension syndromes, cholestasis, mesenchymal inflammation). Comorbid course of non-alcoholic steatohepatitis with chronic kidney disease is characterized by higher degree of liver steatosis compared to the patients with only non-alcoholic steatohepatitis (p<0...
September 2017: Georgian Medical News
https://www.readbyqxmd.com/read/28954837/clinical-laboratory-and-molecular-findings-and-long-term-follow-up-data-in-96-french-patients-with-pmm2-cdg-phosphomannomutase-2-congenital-disorder-of-glycosylation-and-review-of-the-literature
#5
Manuel Schiff, Céline Roda, Marie-Lorraine Monin, Alina Arion, Magali Barth, Nathalie Bednarek, Maud Bidet, Catherine Bloch, Nathalie Boddaert, Delphine Borgel, Anaïs Brassier, Alexis Brice, Arnaud Bruneel, Roger Buissonnière, Brigitte Chabrol, Marie-Chantal Chevalier, Valérie Cormier-Daire, Claire De Barace, Emmanuel De Maistre, Anne De Saint-Martin, Nathalie Dorison, Valérie Drouin-Garraud, Thierry Dupré, Bernard Echenne, Patrick Edery, François Feillet, Isabelle Fontan, Christine Francannet, François Labarthe, Cyril Gitiaux, Delphine Héron, Marie Hully, Sylvie Lamoureux, Dominique Martin-Coignard, Cyril Mignot, Gilles Morin, Tiffany Pascreau, Olivier Pincemaille, Michel Polak, Agathe Roubertie, Christel Thauvin-Robinet, Annick Toutain, Géraldine Viot, Sandrine Vuillaumier-Barrot, Nathalie Seta, Pascale De Lonlay
BACKGROUND: Phosphomannomutase 2-congenital disorder of glycosylation (PMM2-CDG) is a multisystem inborn error of metabolism. OBJECTIVES: To better characterise the natural history of PMM2-CDG. METHODS: Medical charts of 96 patients with PMM2-CDG (86 families, 41 males, 55 females) were retrospectively reviewed. Data on clinical, laboratory and molecular parameters at diagnosis were analysed. Follow-up data at last examination were reported for 25 patients...
September 27, 2017: Journal of Medical Genetics
https://www.readbyqxmd.com/read/28948051/persistent-unexplained-dyspnea-a-case-of-hepatopulmonary-syndrome
#6
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/28932502/ultrasound-guided-direct-intrahepatic-portosystemic-shunt-in-patients-with-budd-chiari-syndrome-short-and-long-term-results
#7
Adam Hatzidakis, Nikolaos Galanakis, Elias Kehagias, Dimitrios Samonakis, Mairi Koulentaki, Erminia Matrella, Dimitrios Tsetis
BACKGROUND AND AIMS: Budd-Chiari syndrome (BCS) is treated with anticoagulation therapy, transjugular intrahepatic portosystemic shunt (TIPS), angioplasty, and liver transplantation. TIPS is not always technically feasible, due to the complete hepatic venous thrombosis. Direct intrahepatic portosystemic shunt (DIPS) is an alternative method for decompression of portal hypertension. This is a retrospective, single-center study aiming to evaluate the outcome of ultrasound (US)-guided DIPS in patients with BCS...
June 2017: Interventional Medicine & Applied Science
https://www.readbyqxmd.com/read/28921803/the-pathophysiology-of-arterial-vasodilatation-and-hyperdynamic-circulation-in-cirrhosis
#8
Søren Møller, Flemming Bendtsen
Patients with cirrhosis and portal hypertension often develop complications from a variety of organ systems leading to a multiple organ failure. The combination of liver failure and portal hypertension result in a hyperdynamic circulatory state partly owing to simultaneous splanchnic and peripheral arterial vasodilatation. Increases in circulatory vasodilators are believed to be due to portosystemic shunting and bacterial translocation leading to redistribution of the blood volume with central hypovolemia. Portal hypertension per se and increased splanchnic blood flow are mainly responsible for the development and perpetuation of the hyperdynamic circulation and the associated changes in cardiovascular function with development of cirrhotic cardiomyopathy, autonomic dysfunction, and renal dysfunction as part of a cardiorenal syndrome...
September 16, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28901345/-acute-alcoholic-hepatitis
#9
Gianni Testino, Emanuele Scafato, Valentino Patussi
Chronic alcohol related liver disease is characterized by a cascade of events defined as follows: steatosis, steatohepatitis/steatofibrosi, cirrhosis and hepatocellular carcinoma. On one of these histologic patterns may overlap acute alcoholic hepatitis (AAE) (mild, moderate, severe). Severe AAE can cause a severe clinical picture: jaundice with a duration of less than three months, jaundice in the first decompensation event, serum bilirubin higher than 5 mg/dL, ratio AST/ALT >2:1, AST less than 500 IU/L ALT <300 IU/L, neutrophil leukocytosis and increased GGT...
September 2017: Recenti Progressi in Medicina
https://www.readbyqxmd.com/read/28884036/severe-hepatopulmonary-syndrome-in-a-child-with-caroli-syndrome
#10
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/28853802/-hepatopulmonary-syndrome-and-portopulmonary-hypertension
#11
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/28836723/rifaximin-treatment-is-associated-with-reduced-risk-of-cirrhotic-complications-and-prolonged-overall-survival-in-patients-experiencing-hepatic-encephalopathy
#12
S H Kang, Y B Lee, J-H Lee, J Y Nam, Y Chang, H Cho, J-J Yoo, Y Y Cho, E J Cho, S J Yu, M Y Kim, Y J Kim, S K Baik, J-H Yoon
BACKGROUND: Rifaximin might decrease the risk of portal hypertension-related complications by controlling small intestinal bacterial overgrowth. AIM: To evaluate whether rifaximin was associated with the risk of death and cirrhotic complications. METHODS: We conducted a retrospective study that included 1042 patients experiencing hepatic encephalopathy (HE): 421 patients without hepatocellular carcinoma (HCC; the non-HCC cohort) and 621 patients with HCC (the HCC cohort)...
November 2017: Alimentary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/28836115/management-of-ascites-and-hepatorenal-syndrome
#13
REVIEW
Salvatore Piano, Marta Tonon, Paolo Angeli
Ascites represents the most common decompensating event in patients with liver cirrhosis. The appearance of ascites is strongly related to portal hypertension, which leads to splanchnic arterial vasodilation, reduction of the effective circulating volume, activation of endogenous vasoconstrictor systems, and avid sodium and water retention in the kidneys. Bacterial translocation further worsens hemodynamic alterations of patients with cirrhosis and ascites. The first-line treatment of uncomplicated ascites is a moderate sodium-restricted diet combined with diuretic treatment...
August 23, 2017: Hepatology International
https://www.readbyqxmd.com/read/28834866/liver-cirrhosis-caused-by-chronic-budd-chiari-syndrome
#14
Mengjie Lin, Feng Zhang, Yi Wang, Bin Zhang, Wei Zhang, Xiaoping Zou, Ming Zhang, Yuzheng Zhuge
Chronic Budd-Chiari syndrome (BCS) is a rare cause of liver cirrhosis (LC) and tends to be misdiagnosed in clinical practice. In order to characterize LC caused by chronic BCS, we conducted this retrospective observational study. Medical records of all patients who were initially diagnosed as chronic BCS with LC when discharged from our department from January, 2011 to October, 2016 were reviewed. Cirrhotic patients with known causes and cases lacked key data were excluded. Data of remaining patients was collected and analyzed...
August 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28815029/nodular-regenerative-hyperplasia-rarely-leads-to-liver-transplantation-a-20-year-cohort-study-in-all-dutch-liver-transplant-units
#15
Berrie Meijer, Melek Simsek, Hans Blokzijl, Robert A de Man, Minneke J Coenraad, Gerard Dijkstra, Carin Mj van Nieuwkerk, Chris Jj Mulder, Nanne Kh de Boer
BACKGROUND: Nodular regenerative hyperplasia is an uncommon liver condition associated with several autoimmune disorders and drugs. The clinical symptoms of nodular regenerative hyperplasia vary from asymptomatic to severe complications of portal hypertension (nodular regenerative hyperplasia-syndrome). OBJECTIVE: The purpose of this study was to identify the prognosis and optimal management, as well as the role of liver transplantation, in nodular regenerative hyperplasia...
August 2017: United European Gastroenterology Journal
https://www.readbyqxmd.com/read/28796076/factors-affecting-mortality-and-resource-use-for-hospitalized-patients-with-cirrhosis-a-population-based-study
#16
Phunchai Charatcharoenwitthaya, Ngamphol Soonthornworasiri, Khemajira Karaketklang, Kittiyod Poovorawan, Wirichada Pan-Ngum, Watcharasak Chotiyaputta, Tawesak Tanwandee, Kamthorn Phaosawasdi
Hospitalizations for advanced liver disease are costly and associated with significant mortality. This population-based study aimed to evaluate factors associated with in-hospital mortality and resource use for the management of hospitalized patients with cirrhosis.Mortality records and resource utilization for 52,027 patients hospitalized with cirrhosis and/or complications of portal hypertension (ascites, hepatic encephalopathy, variceal bleeding, spontaneous bacterial peritonitis, or hepatorenal syndrome) were extracted from a nationally representative sample of Thai inpatients covered by Universal Coverage Scheme during 2009 to 2013...
August 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28776386/post-transfusion-hyperhemolysis-syndrome-following-gastrointestinal-bleeding-secondary-to-prehepatic-portal-hypertension
#17
María de Los Ángeles Mejías Manzano, Álvaro Giráldez Gallego, Yolanda María Sánchez Torrijos
Supportive transfusion represents a basic tool in the management of gastrointestinal (GI) bleeding. However, the use of hemoderivatives is not exempt from risks such as development of hemolysis. We report a case of post-transfusion hyperhemolysis syndrome in a female patient with prehepatic portal hypertension.
September 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/28763855/-pathophysiological-basis-of-portal-hypertension-and-the-new-concept-of-acute-kidney-injury-in-patients-with-liver-cirrhosis
#18
J B Wang
Recently, the International Club of Ascites (ICA) has developed a new expert consensus on the diagnosis and treatment of acute kidney injury (AKI) in patients with liver cirrhosis, which reflects the new concept of AKI management in patients with liver cirrhosis. This consensus emphasizes the increase in the absolute value of serum creatinine (SCr) and establishes a new staging system for AKI, which may help to evaluate disease progression and recovery. In addition, the new management concept also emphasizes that when AKI progresses to stage 2/3 or still progresses after comprehensive treatment, a diagnosis can be made and vasoconstrictors and albumin can be used as long as the patient meets the other diagnostic criteria for hepatorenal syndrome, regardless of SCr level...
June 20, 2017: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/28758559/small-for-size-syndrome-bridging-the-gap-between-liver-transplantation-and-graft-recovery
#19
Akila Rajakumar, Ilankumaran Kaliamoorthy, Mohamed Rela, M Susan Mandell
In living donor liver transplantation, optimal graft size is estimated from values like graft volume/standard liver volume and graft/recipient body weight ratio but the final functional hepatic mass is influenced by other donor and recipient factors. Grafts with insufficient functional hepatic mass can produce a life-threatening condition with rapidly progressive liver failure called small-for-size syndrome (SFSS). Diagnosis of SFSS requires careful surveillance for signs of inadequate hepatocellular function, residual portal hypertension, and systemic inflammation that suggest rapidly progressive liver failure...
September 2017: Seminars in Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/28749707/imaging-findings-of-alagille-syndrome-in-young-infants-differentiation-from-biliary-atresia
#20
Solbee Han, Tae Yeon Jeon, Sook Min Hwang, So-Young Yoo, Yon Ho Choe, Suk-Koo Lee, Ji Hye Kim
OBJECTIVE: To compare the imaging findings using ultrasonography, MR cholangiopancreatography (MRCP), and intraoperative cholangiography (IOC) between Alagille syndrome (AGS) and biliary atresia (BA) in young infants with cholestatic jaundice. METHODS: The institutional review board approved this retrospective study. Ultrasonography (n = 55), MRCP (n = 33), and IOC (n = 22) studies were performed in 55 infants (all younger than 3 months) with AGS (n = 7) and BA (n = 48)...
August 2017: British Journal of Radiology
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