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portal hypertensive syndrome

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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
#1
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
#2
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
#3
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.
August 4, 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
#4
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
#5
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
#6
Solbee Han, Tae Yeon Jeon, Sook Min Hwang, So-Young Yoo, Yon Ho Choe, Suk-Koo Lee, Ji Hye Kim
OBJECTIVES: 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)...
July 27, 2017: British Journal of Radiology
https://www.readbyqxmd.com/read/28744150/radiofrequency-ablation-using-barrx-%C3%A2-for-the-endoscopic-treatment-of-gastric-antral-vascular-ectasia-a-series-of-three-cases-and-a-review-of-the-literature-on-treatment-options
#7
Anish Patel, Sunil Patel, Prasanna C Wickremesinghe, Deepak Vadada
Gastric antral vascular ectasia (GAVE), also known as "watermelon stomach", is an uncommon condition, which can cause gastrointestinal bleeding due to rupture of blood vessels that line the stomach. The pathogenesis of GAVE remains unclear; however it is thought that hemodynamic changes, mechanical stress, and autoimmune factors all have a part to play. A range of conditions are also commonly associated with the syndrome, such as portal hypertensive gastropathy, liver cirrhosis, and autoimmune disorders. Less commonly, chronic renal failure, cardiac diseases, and bone marrow transplantation have coexisted with GAVE...
2017: Clinical and Experimental Gastroenterology
https://www.readbyqxmd.com/read/28721347/peliosis-hepatis-complicating-pregnancy-a-rare-entity
#8
Muhammad Osama Butt, Nasir Hassan Luck, Syed Mujahid Hassan, Zaigham Abbas, Muhammed Mubarak
Peliosis hepatis (PH) is a rare, benign condition of the liver characterized by the presence of blood-filled lacunar spaces in the parenchyma. It usually has a chronic presentation and is a rare cause of portal hypertension reported in adult patients. Its etiology is diverse and ranges from infectious agents to tumors to toxic substances and anabolic steroids; however, the cause remains unclear in 25-50% of patients. Similarly, the symptomatology and imaging findings are diverse. Biopsy is the definitive test to diagnose the condition...
June 2017: Journal of Translational Internal Medicine
https://www.readbyqxmd.com/read/28715896/use-of-abdominal-ultrasound-in-the-diagnosis-of-hepatic-sinusoidal-obstruction-syndrome-sos-in-a-patient-receiving-a-hematopoietic-cell-transplant
#9
Marta Abadía Barnó, Eva Marín Serrano, Gloria Ruiz Fernández
The sinusoidal obstruction syndrome (SOS) is an uncommon form of portal hypertension that occurs in hematopoietic-cell transplant recipients who receive intense conditioning treatments. The diagnosis is clinical and it is usually delayed, because in these patients there are many causes that can damage the liver and can delay its detection. We present the case of a 47-year-old man, whose diagnosis was made with clinical and analytical data supported by ultrasound signs that showed hepatic congestion. After treatment with defibrotide the patient improved clinically and analytically...
July 18, 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/28685257/budd-chiari-syndrome-hepatic-venous-outflow-tract-obstruction
#10
REVIEW
Dominique-Charles Valla
BACKGROUND: Budd-Chiari syndrome (BCS) is a rare disease characterized by hepatic venous outflow tract obstruction (HVOTO). METHODS: Recent literature has been analyzed for this narrative review. RESULTS: Primary BCS/HVOTO is a result of thrombosis. The same patient often has multiple risk factors for venous thrombosis and most have at least one. Presentation and etiology may differ between Western and certain Eastern countries. Myeloproliferative neoplasms are present in 40% of patients and are usually associated with the V617F-JAK2 mutation in myeloid cells, in particular peripheral blood granulocytes...
July 6, 2017: Hepatology International
https://www.readbyqxmd.com/read/28680603/isolated-congenital-hepatic-fibrosis-associated-with-tmem67-mutations-report-of-a-new-genotype-phenotype-relationship
#11
Ida Vogel, Peter Ott, Dorte Lildballe, Stephen Hamilton-Dutoit, Hendrik Vilstrup, Henning Grønbæk
We report an otherwise healthy 32-year-old man with portal hypertension, variceal bleeding, and congenital hepatic fibrosis with ductal plate malformation. Genetic screening identified two TMEM67 mutations. Biallelic TMEM67 mutations are known to cause Joubert/Meckel syndrome or nephronopthisis with hepatic fibrosis, but have never been found in isolated hepatic fibrosis.
July 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28676445/metformin-reduces-intrahepatic-fibrosis-and-intrapulmonary-shunts-in-biliary-cirrhotic-rats
#12
Mu-Tzu Ko, Hui-Chun Huang, Wen-Shin Lee, Chiao-Lin Chuang, I-Fang Hsin, Shao-Jung Hsu, Fa-Yauh Lee, Ching-Chih Chang, Shou-Dong Lee
BACKGROUND: Liver fibrosis causes portal hypertension which dilates collateral vasculature and enhances extra-hepatic angiogenesis including intrapulmonary shunts, which subsequently complicates with hepatopulmonary syndrome. Metformin is an anti-diabetic agent which possesses anti-inflammation and anti-angiogenesis properties. This study evaluated the effect of metformin treatment on liver and lung in a non-diabetic rat model with biliary cirrhosis induced via common bile duct ligation (CBDL)...
July 1, 2017: Journal of the Chinese Medical Association: JCMA
https://www.readbyqxmd.com/read/28643816/-budd-chiari-syndrome-and-ulcerative-colitis-in-an-adolescent
#13
Federico Marchetti, Martina Mainetti, Michela Giovannini, Cristina Morelli
In patients with inflammatory bowel disease (IBD) there is an increased incidence of thromboembolic events. We report a case of a female, age 14, with a 6 months history of diarrhea with occasional presence of red blood and with a very distended abdomen with evident ascites. The diagnosis was Budd-Chiari syndrome (BCS) in ulcerative colitis (UC). Therapy with subcutaneous low molecular weight heparin, methylprednisolone, mesalazine was started. Clinical and radiological features quickly improved. No thrombophilia abnormality nor other risk factor for thrombosis were detected...
May 2017: Recenti Progressi in Medicina
https://www.readbyqxmd.com/read/28638930/-different-adipose-tissue-depots-and-metabolic-syndrome-in-human
#14
Ran Wang, Xiao-Nan Li
Obesity is characterized by abnormal and excessive adipose tissue accumulated in the body. Compared with peripheral obesity (the accumulation of subcutaneous adipose tissue), abdominal obesity (the accumulation of visceral adipose tissue) is associated with increased risk of the metabolic syndrome, such as diabetes, hypertension, atherosclerosis, and dyslipidemia. Adipose tissue is a highly heterogeneous endocrine organ. Adipose tissue depots differ significantly in anatomy, cell biology, glucose and lipid metabolism as well as in endocrine regulation...
June 25, 2017: Sheng Li Xue Bao: [Acta Physiologica Sinica]
https://www.readbyqxmd.com/read/28624548/glucocorticoids-and-gut-bacteria-the-galf-hypothesis-in-the-metagenomic-era
#15
REVIEW
David J Morris, Jason M Ridlon
A new concept is emerging in biomedical sciences: the gut microbiota is a virtual 'organ' with endocrine function. Here, we explore the literature pertaining to the role of gut microbial metabolism of endogenous adrenocorticosteroids as a contributing factor in the etiology of essential hypertension. A body of literature demonstrates that bacterial products of glucocorticoid metabolism are absorbed into the portal circulation, and pass through the kidney before excretion into urine. Apparent mineralocorticoid excess (AME) syndrome patients were found to have congenital mutations resulting in non-functional renal 11β-hydroxysteroid dehydrogenase-2 (11β-HSD2) and severe hypertension often lethal in childhood...
June 15, 2017: Steroids
https://www.readbyqxmd.com/read/28614198/the-almost-normal-liver-biopsy-presentation-clinical-associations-and-outcome
#16
Thomas W Czeczok, John S Van Arnam, Laura D Wood, Michael S Torbenson, Taofic Mounajjed
Liver biopsies obtained for abnormal liver enzymes or unexplained ascites occasionally appear histologically almost normal. The differential diagnosis for these cases is challenging because literature addressing this topic is lacking. We aimed to establish a differential diagnosis and determine clinical associations and outcomes for almost-normal liver biopsies. Ninety-seven histologically almost-normal liver biopsies were collected from 2 institutions. All cases lacked significant inflammation, fatty change, biliary tract disease, vascular disease, nodular regenerative hyperplasia, iron overload, inherited metabolic or storage disorder, viral hepatitis, or fibrosis...
September 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28611533/functional-budd-chiari-syndrome-associated-with-severe-polycystic-liver-disease
#17
Precil Diego Miranda de Menezes Neves, Bruno Eduardo Pedroso Balbo, Elieser Hitoshi Watanabe, Vinicius Rocha-Santos, Wellington Andraus, Luiz Augusto Carneiro D'Albuquerque, Luiz Fernando Onuchic
A 50-year-old woman with end-stage renal disease secondary to autosomal dominant polycystic kidney disease was referred to a quaternary care center due to significantly increased abdominal girth. Her physical examination revealed tense ascites and abdominal collateral veins. A 10-L paracentesis improved abdominal discomfort and disclosed a transudate, suggestive of portal hypertension. A computed tomographic scan revealed massive hepatomegaly caused by multiple cysts of variable sizes, distributed throughout all hepatic segments...
2017: Clinical Medicine Insights. Gastroenterology
https://www.readbyqxmd.com/read/28611257/isolated-intrapulmonary-vascular-dilatations-and-the-risk-of-developing-hepatopulmonary-syndrome-in-liver-transplant-candidates
#18
Manuel Mendizabal, David Goldberg, Federico Piñero, Diego Arufe, María José de la Fuente, Pablo Testa, Matías Coronel, Sergio Baratta, Luis Podestá, Michael Fallon, Marcelo Silva
BACKGROUND: The natural history of intrapulmonary vascular dilations (IPVD) and their impact on patient outcomes in the setting of portal hypertension has only been described in small series. AIMS: To assess the development of hepatopulmonary síndrome (HPS) in patients with isolated IPVD and to evaluate outcomes of IPVD and HPS among patients evaluated for liver transplantation (LT). MATERIAL AND METHODS: Data from a prospective cohort of patients evaluated for LT with standardized screening for HPS were analyzed...
August 1, 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/28610817/vascular-diseases-of-the-liver-clinical-guidelines-from-the-catalan-society-of-digestology-and-the-spanish-association-for-the-study-of-the-liver
#19
Marta Martín-Llahí, Agustín Albillos, Rafael Bañares, Annalisa Berzigotti, M Ángeles García-Criado, Joan Genescà, Virginia Hernández-Gea, Elba Llop-Herrera, Helena Masnou-Ridaura, José Mateo, Carmen A Navascués, Ángela Puente, Marta Romero-Gutiérrez, Macarena Simón-Talero, Luis Téllez, Fanny Turon, Cándido Villanueva, Roberto Zarrabeitia, Juan Carlos García-Pagán
Despite their relatively low prevalence, vascular diseases of the liver represent a significant health problem in the field of liver disease. A common characteristic shared by many such diseases is their propensity to cause portal hypertension together with increased morbidity and mortality. These diseases are often diagnosed in young patients and their delayed diagnosis and/or inappropriate treatment can greatly reduce life expectancy. This article reviews the current body of evidence concerning Budd-Chiari syndrome, non-cirrhotic portal vein thrombosis, idiopathic portal hypertension, sinusoidal obstruction syndrome, hepatic vascular malformations in hereditary haemorrhagic telangiectasia, cirrhotic portal vein thrombosis and other rarer vascular diseases including arterioportal fistulas...
June 11, 2017: Gastroenterología y Hepatología
https://www.readbyqxmd.com/read/28602971/new-developments-in-hepatorenal-syndrome
#20
Ayse L Mindikoglu, Stephen C Pappas
Hepatorenal syndrome (HRS) continues to be one of the major complications of decompensated cirrhosis, leading to death in the absence of liver transplantation. Challenges in precisely evaluating renal function in the patient with cirrhosis remain because of the limitations of serum creatinine (Cr) alone in estimating glomerular filtration rate (GFR); current GFR estimating models appear to underestimate renal function. Newer models incorporating renal biomarkers, such as the Cr-Cystatin C GFR Equation for Cirrhosis appear to estimate true GFR more accurately...
June 7, 2017: Clinical Gastroenterology and Hepatology
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