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portal hypertensive syndrome

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https://www.readbyqxmd.com/read/28091982/death-from-pulmonary-embolism-of-cyanoacrylate-glue-following-gastric-varix-endoscopic-injection
#1
Michael Philip Burke, Chris O'Donnell, Yeliena Baber
We present the case of a 25 year old woman with a complex past medical history including Crigler-Najjar syndrome (Type 1) with a liver transplant in 1993 and subsequent development of cirrhosis with portal hypertension in the transplanted liver. The deceased presented to hospital with hematemesis and investigations showed a large gastric varix. The varix was injected with cyanoacrylate glue. Within 30 min of injection the patient became acutely hypoxic. Urgent chest X-ray demonstrated radio opaque glue within the pulmonary arteries...
January 14, 2017: Forensic Science, Medicine, and Pathology
https://www.readbyqxmd.com/read/28058764/diagnosis-and-treatment-guidelines-for-aberrant-portal-hemodynamics
#2
Fuminori Moriyasu, Yoshihiro Furuichi, Atsushi Tanaka, Hajime Takikawa, Hiroshi Yoshida, Isao Sakaida, Katsutoshi Obara, Makoto Hashizume, Masayoshi Kage, Satoko Ohfuji, Seigo Kitano, Seiji Kawasaki, Shigehiro Kokubu, Shoichi Matsutani, Susumu Eguchi, Susumu Shiomi, Tetsuhito Kojima, Yoshihiko Maehara, Yukio Kuniyoshi
Idiopathic portal hypertension (IPH), causing aberrant portal hemodynamics, is a disease with an as yet unidentified cause and no treatment has been established so far. The Japanese research group on IPH in Japan was set up in 1975 by the Ministry of Health, Labour and Welfare, furthermore extra-hepatic portal obstruction (EHO) and Budd-Chiari syndrome (BCS) have been added to the research subjects and further work has continued. The aims of the research group are to accurately evaluate the current status of the three diseases in Japan, elucidate the disease aetiology and pathogenesis, and develop new treatments...
January 6, 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/28003725/neonatal-cholestasis-as-initial-presentation-of-portosystemic-shunt-a-case-report
#3
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/27989547/graft-inflow-modulation-in-adult-to-adult-living-donor-liver-transplantation-a-systematic-review
#4
REVIEW
Roberto I Troisi, Giammauro Berardi, Federico Tomassini, Mauricio Sainz-Barriga
INTRODUCTION: Small-for-size syndrome (SFSS) has an incidence between 0 and 43% in small-for-size graft (SFSG) adult living donor liver transplantation (LDLT). Portal hypertension following reperfusion and the hyperdynamic splanchnic state are reported as the major triggering factors of SFSS. Intra- and postoperative strategies to prevent or to reduce its onset are still under debate. We analyzed graft inflow modulation (GIM) during adult LDLT considering the indications, efficacy of the available techniques, changes in hemodynamics and outcomes...
December 8, 2016: Transplantation Reviews
https://www.readbyqxmd.com/read/27984174/emergency-tips-in-a-child-pugh-b-patient-when-does-the-window-of-opportunity-open-and-close
#5
REVIEW
Jonel Trebicka
Transjugular intrahepatic portosystemic shunt (TIPS) is used to treat complications of cirrhosis such as variceal bleeding and refractory ascites, but it also bears the risk of liver failure, overt hepatic encephalopathy (HE) and cardiac decompensation. Variceal bleeding may be controlled using endoscopic and medical treatment in patients with compensated cirrhosis; in decompensated patients, however, TIPS improves survival. Therefore, an early TIPS (within 72h or if later, still early after bleeding) might improve the survival of patients by preventing an inflammatory response and bacterial translocation...
October 29, 2016: Journal of Hepatology
https://www.readbyqxmd.com/read/27933201/association-between-metabolic-syndrome-and-liver-histology-among-nafld-patients-without-diabetes
#6
Pushpjeet Kanwar, James E Nelson, Katherine Yates, David E Kleiner, Aynur Unalp-Arida, Kris V Kowdley
BACKGROUND: Metabolic syndrome (MetS) and obesity are associated with non-alcoholic fatty liver disease (NAFLD). The aim of this observational study was to examine the relationship of MetS and a diagnosis of non-alcoholic steatohepatitis (NASH) in patients without diabetes in the NASH Clinical Research Network (CRN). METHODS: Clinical, demographic, histological, laboratory and anthropometric data were collected on 356 adult patients without diabetes with NAFLD. Obesity was defined as body mass index ≥30...
2016: BMJ Open Gastroenterology
https://www.readbyqxmd.com/read/27920483/outcomes-of-partial-splenic-embolization-in-patients-with-massive-splenomegaly-due-to-idiopathic-portal-hypertension
#7
Omer Ozturk, Gonca Eldem, Bora Peynircioglu, Taylan Kav, Aysegul Görmez, Barbaros Erhan Cil, Ferhun Balkancı, Cenk Sokmensuer, Yusuf Bayraktar
AIM: To determine the outcomes of partial splenic embolization (PSE) for massive splenomegaly due to idiopathic portal hypertension (IPH). METHODS: In this prospective study, we evaluated the characteristics and prognosis of consecutive patients with IPH who underwent PSE for all indications at a single medical center between June 2009 and January 2015. The inclusion criteria were: presence of hypersplenism, massive splenomegaly, and resultant pancytopenia. The exclusion criteria were: presence of other diseases causing portal hypertension...
November 21, 2016: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/27871159/-acute-kidney-injury-in-cirrhotic-patients-with-portal-hypertension
#8
REVIEW
So Mi Kim, Il Han Song
Acute kidney injury (AKI) is one of the most common manifestations encountered in clinical practice. It is associated with high morbidity and mortality in cirrhotic pre- and post-transplantation patients. Hepatorenal syndrome (HRS), a special form of AKI in cirrhotic patients, was recognized as a consequence of renal vasoconstriction from systemic/renal hemodynamic alterations developed in advanced cirrhosis with portal hypertension. Recently, multiple factors-such as infection/inflammation, underlying glomerulonephritis, bile cast, or increased abdominal pressure-have been considered to contribute to renal dysfunction in cirrhotic patients, which were presumed to induce HRS...
November 25, 2016: Korean Journal of Gastroenterology, Taehan Sohwagi Hakhoe Chi
https://www.readbyqxmd.com/read/27864873/hereditary-haemorrhagic-telangiectasia-to-transplant-or-not-to-transplant-is-there-a-right-time-for-liver-transplantation
#9
Yannick D Muller, Roland Oppliger, Romain Breguet, Philippe Meyer, Laura Rubbia-Brandt, Pierre-Auguste Petignat, Thomas Harr, Eric Dayer, Jörg D Seebach
BACKGROUND & AIMS: Hereditary haemorrhagic telangiectasia is characterized by arterio-venous malformations (AVM). It frequently involves the liver without clinical symptoms, but may lead to biliary ischaemia, portal hypertension, or fatal high-output heart failure. The indication of liver transplantation is controversial. METHODS: Herein, we report the case of a 65-year-old female patient with a 'double Osler syndrome' consisting of hereditary haemorrhagic telangiectasia (HHT) and type I hereditary angioedema diagnosed at the age of 25 and 22 years respectively...
December 2016: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/27853493/implantability-complications-and-follow-up-after-transjugular-intrahepatic-portosystemic-stent-shunt-creation-with-the-6f-self-expanding-sinus-superflex-visual-stent
#10
Daniel Spira, Jakub Wiskirchen, Ulrich Lauer, Dominik Ketelsen, Konstantin Nikolaou, Benjamin Wiesinger
BACKGROUND: The transjugular intrahepatic portosystemic stent-shunt (TIPSS) builds a shortcut between the portal vein and a liver vein, and represents a sophisticated alternative to open surgery in the management of portal hypertension or its complications. OBJECTIVES: To describe clinical experiences with a low-profile nitinol stent system in TIPSS creation, and to assess primary and long-term success. PATIENTS AND METHODS: Twenty-six patients (5 females, 21 males; mean age 54...
July 2016: Iranian Journal of Radiology: a Quarterly Journal Published By the Iranian Radiological Society
https://www.readbyqxmd.com/read/27807573/portal-hypertension-and-ascites-due-to-an-arterioportal-fistula-sequela-of-a-remote-traumatic-liver-laceration
#11
Benjamin M Hulkower, Sabah Butty, Marwan Ghabril
Arterioportal fistulas (APFs) are a group of vascular disorders, in which systemic arteries communicate with the portal circulation, presenting as a congenital syndrome or more commonly acquired from iatrogenic instrumentation or abdominal trauma. We report the case of a 58-year-old man who developed ascites without underlying risk factors for portal hypertension, which was attributed to an APF found on imaging, manifesting 43 years after sustaining a liver laceration. After angiographic embolization of the APF, the patient's ascites resolved completely...
August 2016: ACG Case Reports Journal
https://www.readbyqxmd.com/read/27784438/-research-advances-in-diagnosis-and-treatment-of-post-transjugular-intrahepatic-portosystemic-shunt-hepatic-encephalopathy
#12
J F Yang, B Q Zhang
Transjugular intrahepatic portosystemic shunt (TIPS) has become an important minimally invasive interventional technique for the treatment of complications of cirrhotic portal hypertension, and currently, it is often used in cirrhotic patients with esophagogastric variceal bleeding (EVB), intractable ascites, hepatic hydrothorax, and Budd-Chiari syndrome. On one hand, TIPS can effectively reduce portal vein pressure and the risk of EVB and intractable ascites; on the other hand, it may reduce the blood flow in liver perfusion, aggravate liver impairment, and cause porto-systemic encephalopathy...
July 20, 2016: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/27766814/alstr%C3%A3-m-syndrome-with-portal-hypertension
#13
Ameya S Joshi, Ashok R Mohite, Premlata K Varthakavi, Pravin M Rathi
Alstrom syndrome is an autosomal recessive multisystem disorder caused by mutation in ALMS1 (2p13). Very few cases of same are reported so far of same. We report a case of Alstrom syndrome (AS) who presented with type II diabetes mellitus and portal hypertension. Unilateral anorchia with hypergonadotropic hypogonadism is another unique feature of our case.
October 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27765751/decoding-the-vasoregulatory-activities-of-bile-acid-activated-receptors-in-systemic-and-portal-circulation-role-of-gaseous-mediators
#14
REVIEW
Stefano Fiorucci, Angela Zampella, Giuseppe Cirino, Mariarosaria Bucci, Eleonora Distrutti
Bile acids are end products of cholesterol metabolism generated in the liver and released in the intestine. Primary and secondary bile acids are the result of the symbiotic relation between the host and intestinal microbiota. In addition to their role in nutrient absorption, bile acids are increasingly recognized as regulatory signals that exert their function beyond the intestine by activating a network of membrane and nuclear receptors. The best characterized of these bile acid-activated receptors, GPBAR1 (also known as TGR5) and the farnesosid-X-receptor (FXR), have also been detected in the vascular system and their activation mediates the vasodilatory effects of bile acids in the systemic and splanchnic circulation...
January 1, 2017: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/27757214/positive-impact-of-eculizumab-therapy-on-surgery-for-budd-chiari-syndrome-in-a-patient-with-paroxysmal-nocturnal-hemoglobinuria-and-a-long-term-history-of-thrombosis
#15
Silvia De-la-Iglesia, Hugo Luzardo, Angelina Lemes, Melissa Torres, Maria Teresa Gómez-Casares, Naylen Cruz, Teresa Molero
Paroxysmal nocturnal hemoglobinuria (PNH) is associated with severe end-organ damage and a high risk of thrombosis. Budd-Chiari syndrome, which develops after thrombotic occlusion of major hepatic blood vessels, is relatively common in PNH and has been associated with increased mortality. We report the case of a 46-year-old male with PNH who presented with Budd-Chiari syndrome associated with portal cavernoma, portal hypertension and hypersplenism. In September 2010, the patient suffered gastrointestinal bleeding, hematuria, and elevated plasma lactate dehydrogenase; he started eculizumab therapy with a good response...
September 28, 2016: Hematology Reports
https://www.readbyqxmd.com/read/27747278/efficacy-of-argon-plasma-coagulation-in-the-management-of-portal-hypertensive-gastropathy
#16
Amr Shaaban Hanafy, Amr Talaat El Hawary
Objectives: Evaluation of the outcome and experience in 2 years of management of portal hypertensive gastropathy (PHG) by argon plasma coagulation (APC) in a cohort of Egyptian cirrhotic patients. Methods: This study was conducted over a 2-year period from January 2011 to February 2013. Upper gastrointestinal endoscopy was performed to evaluate the degree and site of PHG. APC was applied to areas with mucosal vascular lesions. Results: In total, 200 cirrhotic patients were enrolled; 12 patients were excluded due to death (n = 6) caused by hepatic encephalopathy (n = 3), hepatorenal syndrome (n = 2), or chronic lymphatic leukemia (n = 1), or did not complete the treatment sessions (n = 6), so 188 patients completed the study...
October 2016: Endoscopy International Open
https://www.readbyqxmd.com/read/27729270/serum-osteopontin-is-a-biomarker-of-severe-fibrosis-and-portal-hypertension-in-human-and-murine-schistosomiasis-mansoni
#17
Thiago Almeida Pereira, Wing-Kin Syn, Fausto E L Pereira, José Roberto Lambertucci, William Evan Secor, Anna Mae Diehl
Schistosomiasis is a major cause of fibrosis and portal hypertension. The reason 4-10% of infected subjects develops hepatosplenic schistosomiasis remains unclear. Chronically infected male CBA/J mice reproduce the dichotomic forms of human schistosomiasis. Most mice (80%) develop moderate splenomegaly syndrome (similar to hepatointestinal disease in humans) and 20% present severe hypersplenomegaly syndrome (analogous to human hepatosplenic disease). We demonstrated that the profibrogenic molecule osteopontin discriminates between mice with severe and mild disease and could be a novel morbidity biomarker in murine and human schistosomiasis...
December 2016: International Journal for Parasitology
https://www.readbyqxmd.com/read/27693870/primary-bacterial-peritonitis-in-a-previously-healthy-adolescent-female-a-case-report
#18
Evangelos Blevrakis, Dimitrios Anyfantakis, Emmanouil Blevrakis, Ioannis Vlachakis
INTRODUCTION: Acute pneumococcal peritonitis represents an unusual entity characterized by infection in the abdominal cavity despite the absence of an obvious causative source. Patients with portal hypertension such as cirrhotics and those with nefrotic syndrome are more frequently encountered in the every day practice. PRESENTATION OF CASE: A 14 years old female was referred to our department by his general practitioner with a 24h history of right lower abdominal pain and fever...
September 21, 2016: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/27693837/massive-upper-gastrointestinal-bleeding-due-to-splenoportal-axis-thrombosis-in-a-patient-with-a-tested-jak2-mutation-a-case-report-and-review-literature
#19
Isabel Macías
Portal hypertension is a clinical syndrome defined as a portal venous pressure that exceeds 10mmHg. Cirrhosis is the most common cause of portal hypertension and thrombosis of the splenoportal axis not associated with liver cirrhosis is the second cause of portal hypertension in the Western world. The primary myeloproliferative disorders are the main cause of portal venous thrombosis and somatic mutation of Janus Kinase 2 gene (JAK2 V617F) can be found in approximately 90% of polycythemia vera, 50% of essential thrombocyrosis and 50% primary myelofibrosis...
August 13, 2016: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/27660674/therapeutic-alternatives-for-the-treatment-of-type-1-hepatorenal-syndrome-a-delphi-technique-based-consensus
#20
Juan P Arab, Juan C Claro, Juan P Arancibia, Jorge Contreras, Fernando Gómez, Cristian Muñoz, Leyla Nazal, Eric Roessler, Rodrigo Wolff, Marco Arrese, Carlos Benítez
AIM: To propose several alternatives treatment of type 1 hepatorenal syndrome (HRS-1) what is the most severe expression of circulatory dysfunction on patients with portal hypertension. METHODS: A group of eleven gastroenterologists and nephrologists performed a structured analysis of available literature. Each expert was designated to review and answer a question. They generated draft statements for evaluation by all the experts. Additional input was obtained from medical community...
September 8, 2016: World Journal of Hepatology
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