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portal hypertensive syndrome

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https://www.readbyqxmd.com/read/29472420/budd-chiari-syndrome-in-a-patient-with-ulcerative-colitis
#1
Gopal Krishana Bohra, Vivek Chhabra, Naresh Midha, Binit Sureka
We report a case of 26-year-old man who was admitted on our ward for the evaluation of ascites. He was a known case of inflammatory bowel disease(ulcerative colitis) and was on regular mesalamine therapy. On evaluation, he was having high serum ascites albumin gradient. CT scan of the abdomen revealed features of portal hypertension and non-visualisation of right and middle hepatic veins along with thrombus in inferior vena cava, suggesting a diagnosis of Budd-Chiari syndrome (BCS). There are only few case reports available showing association of BCS with UC...
February 22, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29404365/acute-right-ventricular-failure-after-orthotopic-liver-transplantation
#2
Rohan M Goswami, Rami N Khouzam
The interdependence between the heart and liver in maintaining hemodynamic stability during the perioperative period of either orthotopic heart (OHT) or liver (OLT) transplantation is important. The pre-transplant hemodynamic changes that occur in patients with end-stage liver disease (ESLD) can include decreased systemic vascular resistance, poor ventricular response to stress and increased cardiac output (CO). Concomitant pulmonary disorders are often present in ESLD. Portopulmonary hypertension (PoPHTN) is an important marker for increased mortality in liver transplant patients...
January 2018: Annals of Translational Medicine
https://www.readbyqxmd.com/read/29362613/protective-effects-of-the-third-generation-vasodilatory-%C3%AE-eta-blocker-nebivolol-against-d-galactosamine-induced-hepatorenal-syndrome-in-rats
#3
Ahmed Atwa, Rehab Hegazy, Rania Mohsen, Neamat Yassin, Sanaa Kenawy
BACKGROUND: Renal dysfunction is very common in patients with advanced liver cirrhosis and portal hypertension. The development of renal failure in the absence of clinical, anatomical or pathological causes renal of failure is termed hepatorenal syndrome (HRS). AIM: The present study was constructed to investigate the possible protective effects of nebivolol (Nebi) against D-galactosamine (Gal)-induced HRS in rats. MATERIAL AND METHODS: Rats were treated with Nebi for ten successive days...
December 15, 2017: Open Access Macedonian Journal of Medical Sciences
https://www.readbyqxmd.com/read/29340275/timing-of-transjugular-intrahepatic-portosystemic-shunt-for-budd-chiari-syndrome-an-italian-hepatologist-s-perspective
#4
Andrea Mancuso
Budd-Chiari syndrome (BCS) management flow-chart is derived from experts' opinion and is not evidence-based. Guidelines suggest BCS management should follow a stepwise strategy: medical therapy as first-line treatment, revascularization or transjugular intrahepatic portosystemic shunt (TIPS) if no response to medical therapy, and liver transplant as rescue therapy. Recent evidence suggests that only medical therapy results in a bad long-term outcome. The biggest criticism of guidelines is the indication that BCS should receive further treatment only when hemodynamic consequences of portal hypertension become clinically evident...
December 2017: Journal of Translational Internal Medicine
https://www.readbyqxmd.com/read/29319259/alstr%C3%A3-m-syndrome-with-portal-hypertension
#5
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 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/29318565/a-case-of-severe-stenosis-of-hepatic-veins-and-inferior-vena-cava-with-stomal-variceal-bleeding-induced-by-oxaliplatin-based-chemotherapy
#6
Hayato Yamaguchi, Yoshihiro Furuichi, Yoshitaka Kasai, Hirohito Takeuchi, Yuu Yoshimasu, Katsutoshi Sugimoto, Ikuo Nakamura, Takao Itoi
A 27-year-old woman with colon cancer and liver metastasis was referred to our hospital. Colectomy and colostomy were performed to improve her ileus. Following 13 sessions of oxaliplatin-based chemotherapy (OC) with mFOLFOX6 + bevacizumab, thrombocytopenia and frequent peristomal bleeding occurred. Computed tomography showed severe ascites, splenomegaly, significant collateral veins around the stoma, and severe stenosis of the hepatic veins (HV) and inferior vena cava (IVC). Ultrasound elastography showed high liver (and spleen) stiffness values...
January 9, 2018: Clinical Journal of Gastroenterology
https://www.readbyqxmd.com/read/29316565/transjugular-intrahepatic-portosystemic-shunt-in-patients-with-portal-hypertension-patency-depends-on-coverage-and-interventionalist-s-experience
#7
Matthias Buechter, Paul Manka, Guido Gerken, Ali Canbay, Sandra Blomeyer, Axel Wetter, Jens Altenbernd, Alisan Kahraman, Jens M Theysohn
BACKGROUND AND AIMS: Transjugular intrahepatic portosystemic shunt (TIPS) is the treatment of choice in decompensated portal hypertension. TIPS revision due to thrombosis or stenosis increases morbidity and mortality. Our aim was to investigate patient- and procedure-associated risk factors for TIPS-revision. PATIENTS AND METHODS: We retrospectively evaluated 189 patients who underwent the TIPS procedure. Only patients who required TIPS revision within 1 year (Group I, 34 patients) and patients who did not require re-intervention within the first year (Group II [control group], 54 patients) were included...
January 9, 2018: Digestive Diseases
https://www.readbyqxmd.com/read/29290508/the-emergency-medicine-evaluation-and-management-of-the-patient-with-cirrhosis
#8
REVIEW
Brit Long, Alex Koyfman
BACKGROUND: Cirrhosis is a significant cause of death in the U.S. and has a variety of causes, most commonly Hepatitis C and alcohol. Liver fibrosis and nodule formation result in significant complications due to portal system hypertension. There are several deadly complications emergency physicians must consider. OBJECTIVE OF THE REVIEW: Provide an evidence-based update for the resuscitation of decompensating cirrhotic patients and an overview of cirrhosis complications...
December 23, 2017: American Journal of Emergency Medicine
https://www.readbyqxmd.com/read/29229261/preliminary-experience-in-combined-somatic-and-cerebral-oximetry-monitoring-in-liver-transplantation
#9
Tina Hu, Yves Collin, Réal Lapointe, François Martin Carrier, Luc Massicotte, Annik Fortier, Jean Lambert, Franck Vandenbroucke-Menu, André Y Denault
OBJECTIVE: The use of cerebral near-infrared spectroscopy (NIRS) has become widespread in cardiac surgery after research demonstrated an association between perioperative cerebral desaturations and postoperative complications. Somatic NIRS desaturation also is associated with an increased risk of postoperative complications and mortality. The objective of this study was to explore the trends of both somatic and cerebral NIRS during liver transplantation. DESIGN: A prospective, single-site, observational case series...
July 20, 2017: Journal of Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/29211374/-splanchnic-vein-thrombosis
#10
Bobby Gouin, Helia Robert-Ebadi, Alessandro Casini, Yan Beauverd, Pierre Fontana, Marc Righini, Laure Elkrief
Splanchnic vein thromboses include thrombosis of the portal venous system (including the portal, mesenteric and splenic vein) and hepatic vein thrombosis (also called Budd-Chiari syndrome). They are rare manifestations of venous thromboembolism. These thromboses are frequently associated with local or systemic factors. The therapeutic approach is often complex due to heterogeneity of patients and limited available data in the literature. The cornerstone of treatment is anticoagulation. However, the bleeding risk, related to portal hypertension, should be accurately assessed to individualize the treatment...
December 6, 2017: Revue Médicale Suisse
https://www.readbyqxmd.com/read/29209118/chronic-liver-disease-is-universal-in-children-with-biliary-atresia-living-with-native-liver
#11
Way Seah Lee, Sik Yong Ong, Hee Wei Foo, Shin Yee Wong, Chen Xi Kong, Ru Bin Seah, Ruey Terng Ng
AIM: To examine the medical status of children with biliary atresia (BA) surviving with native livers. METHODS: In this cross-sectional review, data collected included complications of chronic liver disease (CLD) (cholangitis in the preceding 12 mo, portal hypertension, variceal bleeding, fractures, hepatopulmonary syndrome, portopulmonary hypertension) and laboratory indices (white cell and platelet counts, total bilirubin, albumin, international normalized ratio, alanine aminotransferase, aspartate aminotransferase, γ-glutamyl transpeptidase)...
November 21, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/29176474/hepatic-parenchymal-injury-in-crigler-najjar-type-i
#12
Ellen Mitchell, Sarangarajan Ranganathan, Patrick McKiernan, Robert H Squires, Kevin Strauss, Kyle Soltys, George Mazariegos, James E Squires
BACKGROUND: Crigler-Najjar syndrome type I (CNI) arises from biallelic variants of UGT1A1 that abrogate UGT1A1 activity resulting in unconjugated hyperbilirubinemia. Historically, liver parenchyma in CNI was considered structurally and histologically normal. Recent review of CNI liver explants revealed fibrosis. Our aim was to investigate the association between hepatic histology and disease phenotype in CNI. METHODS: We extracted data from the medical record at the time of liver transplant from 22 patients with CNI at the Children's Hospital of Pittsburgh, and reviewed explant histology...
November 22, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29124669/shunt-occlusion-for-portosystemic-shunt-syndrome-related-refractory-hepatic-encephalopathy-a-single-center-experience-in-21-patients-from-kerala
#13
Cyriac Abby Philips, Lijesh Kumar, Philip Augustine
INTRODUCTION: Large spontaneous portosystemic shunts (SPSS) are seen in a subset of patients with liver disease and medically refractory recurrent/persistent hepatic encephalopathy (MRHE). Shunt occlusion has been shown to improve clinical outcomes. METHODS: We retrospectively analyzed patient characteristics, SPSS attributes, procedural features, baseline clinical and investigational parameters, neurological outcomes, adverse effects (procedure and portal hypertension related), and risk factors predicting outcomes in liver disease patients undergoing shunt occlusion procedure for MRHE...
September 2017: Indian Journal of Gastroenterology: Official Journal of the Indian Society of Gastroenterology
https://www.readbyqxmd.com/read/29112083/characteristics-of-liver-disease-in-100-individuals-with-joubert-syndrome-prospectively-evaluated-at-a-single-center
#14
Anna Strongin, Theo Heller, Dan Doherty, Ian A Glass, Melissa A Parisi, Joy Bryant, Peter Choyke, Baris Turkbey, Kailash Daryanani, Deniz Yildirimli, Meghana Vemulapalli, Jim C Mullikin, May C Malicdan, Thierry Vilboux, William A Gahl, Meral Gunay-Aygun
BACKGROUND & AIMS: Joubert Syndrome (JS) is a rare, inherited, ciliopathy defined by cerebellar and brainstem malformations and is variably associated with liver, kidney, and ocular dysfunction. This study characterizes the hepatic findings in JS and identifies factors associated with probable portal hypertension. METHODS: 100 individuals with JS were prospectively evaluated at the National Institutes of Health Clinical Center. Laboratory tests, imaging, and DNA sequencing were performed...
November 3, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29076289/orthotopic-liver-transplantation-for-sensenbrenner-syndrome
#15
Ruth Linda Ackah, Dor Yoeli, Michael Kueht, Nhu Thao Nguyen Galván, Ronald Timothy Cotton, Abbas Rana, Christine Ann O'Mahony, John Alan Goss
Sensenbrenner syndrome, or cranioectodermal dysplasia, is a rare heterogeneic autosomal recessive disorder, affecting ~1 of 1 000 000 live births. The syndrome usually manifests within the first year of life and can present with progressive liver and renal involvement. For all Sensenbrenner patients, renal and liver diseases are the main contributors of morbidity and mortality. In this report, we present the case of a 7-year-old boy with congenital liver disease progressing to liver failure secondary to Sensenbrenner syndrome...
October 26, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/29075990/biology-of-portal-hypertension
#16
REVIEW
Matthew McConnell, Yasuko Iwakiri
Portal hypertension develops as a result of increased intrahepatic vascular resistance often caused by chronic liver disease that leads to structural distortion by fibrosis, microvascular thrombosis, dysfunction of liver sinusoidal endothelial cells (LSECs), and hepatic stellate cell (HSC) activation. While the basic mechanisms of LSEC and HSC dysregulation have been extensively studied, the role of microvascular thrombosis and platelet function in the pathogenesis of portal hypertension remains to be clearly characterized...
October 26, 2017: Hepatology International
https://www.readbyqxmd.com/read/29064029/the-portal-hypertension-syndrome-etiology-classification-relevance-and-animal-models
#17
REVIEW
Jaime Bosch, Yasuko Iwakiri
BACKGROUND: Portal hypertension is a key complication of portal hypertension, which is responsible for the development of varices, ascites, bleeding, and hepatic encephalopathy, which, in turn, cause a high mortality and requirement for liver transplantation. AIM: This review deals with the present day state-of-the-art preventative treatments of portal hypertension in cirrhosis according to disease stage. Two main disease stages are considered, compensated and decompensated cirrhosis, the first having good prognosis and being mostly asymptomatic, and the second being heralded by the appearance of bleeding or non-bleeding complications of portal hypertension...
October 24, 2017: Hepatology International
https://www.readbyqxmd.com/read/29062909/congenital-hepatic-fibrosis-in-a-9-year-old-female-patient-a-case-report
#18
Kamil Janowski, Maria Goliszek, Joanna Cielecka-Kuszyk, Irena Jankowska, Joanna Pawłowska
Congenital hepatic fibrosis (CHF) is a rare, autosomal recessive disorder, clinically characterized by hepatic fibrosis and portal hypertension. CHF results from ductal plate malformation (DPM) of the intrahepatic bile ducts. Four clinical forms can be observed: portal hypertensive, cholangitic, mixed and latent. CHF is one of the "fibropolycystic diseases" which also include several conditions with a variety of intrahepatic bile duct dilatation and associated periportal fibrosis such as Caroli disease, autosomal recessive and dominant polycystic kidney disease (ARPKD or ADPKD), Ivemark, Jeune, Joubert, Bardet-Biedl, Meckel-Gruber and Arima syndromes...
September 2017: Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/29024353/exercise-and-physical-activity-for-patients-with-end-stage-liver-disease-improving-functional-status-and-sarcopenia-while-on-the-transplant-waiting-list
#19
Andrés Duarte-Rojo, Astrid Ruiz-Margáin, Aldo J Montaño-Loza, Ricardo U 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 macronutrient 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 approximately 1 in 5 patients on the liver transplantation waiting list. Sarcopenia, poor physical fitness/cardiopulmonary endurance (CPE), and frailty are all associated with increased mortality in ESLD...
January 2018: Liver Transplantation
https://www.readbyqxmd.com/read/28983709/late-complications-of-biliary-atresia-hepatopulmonary-syndrome-and-portopulmonary-hypertension
#20
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...
December 2017: Pediatric Surgery International
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