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portal hypertension syndrome

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https://www.readbyqxmd.com/read/28223722/to-screen-or-not-to-screen-celiac-antibodies-in-liver-diseases
#1
REVIEW
Janaína Luz Narciso-Schiavon, Leonardo Lucca Schiavon
Celiac disease (CD) is a systemic immune-mediated disorder triggered by dietary gluten in genetically predisposed individuals. The typical symptoms are anemia, diarrhea, fatigue, weight loss, and abdominal pain. CD has been reported in patients with primary sclerosing cholangitis, primary biliary cholangitis, autoimmune hepatitis, aminotransferase elevations, nonalcoholic fatty liver disease, hepatitis B, hepatitis C, portal hypertension and liver cirrhosis. We evaluate recommendations for active screening for CD in patients with liver diseases, and the effect of a gluten-free diet in these different settings...
February 7, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28166812/efficacy-of-high-intensity-focused-ultrasound-assisted-hepatic-resection-hifu-ar-on-blood-loss-reduction-in-patients-with-liver-metastases-requiring-hepatectomy-study-protocol-for-a-randomized-controlled-trial
#2
Aurélien Dupré, David Pérol, Ellen Blanc, Patrice Peyrat, Valéria Basso, Yao Chen, Jérémy Vincenot, Anthony Kocot, David Melodelima, Michel Rivoire
BACKGROUND: Liver resection is the only potentially curative treatment for colorectal liver metastases (LM). It is considered a safe procedure, but is often associated with blood loss during liver transection. Blood transfusions are frequently needed, but they are associated with increased morbidity and risk of recurrence. Many surgical devices have been developed to decrease blood loss. However, none of them has proven superior to the standard crushing technique. We developed a new, powerful intra-operative high-intensity focused ultrasound (HIFU) transducer which destroys tissue by coagulative necrosis...
February 6, 2017: Trials
https://www.readbyqxmd.com/read/28160419/adams-oliver-syndrome-review-of-the-literature-refining-the-diagnostic-phenotype
#3
Susan Hassed, Shibo Li, John Mulvihill, Christopher Aston, Susan Palmer
The Adams-Oliver syndrome (AOS) is defined as aplasia cutis congenita (ACC) with transverse terminal limb defects (TTLD). Frequencies of associated anomalies are not well characterized. Six causative genes have been identified: ARHGAP31, DOCK6, EOGT, RBPJ, NOTCH1, and DLL4. We review 385 previously described individuals (139 non-familial and 246 familial probands and family members) and add clinical data on 13 previously unreported individuals with AOS. In addition to ACC and TTLD, the most commonly associated anomalies included a wide variety of central nervous system (CNS) anomalies and congenital heart defects each seen in 23%...
February 4, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28153558/middle-east-respiratory-syndrome-coronavirus-mers-cov-outbreak-in-south-korea-2015-epidemiology-characteristics-and-public-health-implications
#4
K H Kim, T E Tandi, J W Choi, J M Moon, M S Kim
BACKGROUND: Since the first case of Middle East respiratory syndrome coronavirus (MERS-CoV) in South Korea was reported on 20(th) May 2015, there have been 186 confirmed cases, 38 deaths and 16,752 suspected cases. Previously published research on South Korea's MERS outbreak was limited to the early stages, when few data were available. Now that the outbreak has ended, albeit unofficially, a more comprehensive review is appropriate. METHODS: Data were obtained through the MERS portal by the Ministry for Health and Welfare (MOHW) and Korea Centres for Disease Control and Prevention, press releases by MOHW, and reports by the MERS Policy Committee of the Korean Medical Association...
February 2017: Journal of Hospital Infection
https://www.readbyqxmd.com/read/28150309/portal-systemic-shunt-between-the-hepatic-portal-vein-and-right-renal-vein-in-a-patient-with-multifocal-hepatocellular-carcinoma-case-report
#5
Mateja Sabol Pušić, Ivan Budimir, Zdravko Dorosulić, Branko Ostrički, Marko Nikolić, Gordana Lovrenčić Prpić, Katherina B Sreter
Portal hypertension is a clinical syndrome characterized by the development of collateral circulation and portosystemic shunts, as well as ascites and hepatic encephalopathy. We present the case of a large portosystemic shunt between the hepatic portal vein and aneurysmal right renal vein in a cirrhotic 64-year-old man with thrombosis of the portal vein and hepatocellular carcinoma. This is a very rare clinical manifestation which, to our knowledge, has been described only once previously in the literature...
February 2, 2017: Journal of Clinical Ultrasound: JCU
https://www.readbyqxmd.com/read/28115278/the-role-of-thromboelastography-teg-in-pediatric-patients-with-sinusoidal-obstructive-syndrome-receiving-defibrotide
#6
Joanna L Gendreau, Christine Knoll, Roberta H Adams, Leon L Su
Sinusoidal obstructive syndrome (SOS) is a potentially fatal form of hepatic injury following hematopoietic stem cell transplant (HSCT). Patients can develop liver dysfunction, portal hypertension, ascites, coagulopathies, and multi-system organ failure. The mortality rate of severe SOS has been reported as high as 98% by Day 100 post-transplant. Defibrotide, which is now approved for the treatment of SOS, has significantly decreased mortality. Defibrotide is a polynucleotide with pro-fibrinolytic, anti-ischemic, and anti-inflammatory activity...
January 20, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28115008/hereditary-hemorrhagic-telangiectasia-liver-disease-and-elevated-serum-testosterone-osler-weber-rendu-syndrome-a-case-report
#7
R Dissanayake, K P K Y M D S Wickramarathne, S N Seneviratne, S N Perera, M U J Fernando, V P Wickramasinghe
BACKGROUND: A Sri Lankan girl with hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) is described. CASE PRESENTATION: She presented with recurrent spontaneous epistaxis, pulmonary arterio venous malformation and oral telangiectasia. A diagnosis of Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) was made based on the presence of three Curacao criteria (out of four). Evaluations of her jaundice revealed chronic parenchymal liver disease with multiple nodules in the liver with early portal hypertension...
January 23, 2017: BMC Research Notes
https://www.readbyqxmd.com/read/28091982/death-from-pulmonary-embolism-of-cyanoacrylate-glue-following-gastric-varix-endoscopic-injection
#8
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...
March 2017: Forensic Science, Medicine, and Pathology
https://www.readbyqxmd.com/read/28058764/diagnosis-and-treatment-guidelines-for-aberrant-portal-hemodynamics
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
REVIEW
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
#20
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
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