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portal hypertension syndrome

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https://www.readbyqxmd.com/read/28643816/-budd-chiari-syndrome-and-ulcerative-colitis-in-an-adolescent
#1
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
#2
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
#3
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 14, 2017: Steroids
https://www.readbyqxmd.com/read/28614198/the-almost-normal-liver-biopsy-presentation-clinical-associations-and-outcome
#4
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...
June 13, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28611533/functional-budd-chiari-syndrome-associated-with-severe-polycystic-liver-disease
#5
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
#6
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
#7
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
#8
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 due to 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 more accurately estimate true GFR...
June 7, 2017: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28591307/schistosomiasis-and-hepatopulmonary-syndrome-the-role-of-concomitant-liver-cirrhosis
#9
Liana Gonçalves-Macedo, Edmundo Pessoa Lopes, Ana Lucia Coutinho Domingues, Brivaldo Markman, Vitor Gomes Mota, Carlos Feitosa Luna
BACKGROUND: Hepatopulmonary syndrome (HPS) is defined as an oxygenation defect induced by intrapulmonary vasodilation in patients with liver disease or portal hypertension. It is investigated in patients with liver cirrhosis and less frequently in those with portal hypertension without liver cirrhosis, as may occur in hepatosplenic schistosomiasis (HSS). OBJECTIVES: To investigate the prevalence of HPS in patients with HSS, and to determine whether the occurrence of HPS is influenced by concomitant cirrhosis...
July 2017: Memórias do Instituto Oswaldo Cruz
https://www.readbyqxmd.com/read/28589849/portal-vein-thrombosis-and-nephrotic-syndrome-after-liver-transplant
#10
Jérôme Dumortier, Antoine Sicard, Olivier Guillaud, Pierre-Jean Valette, Jean-Yves Scoazec, Olivier Boillot
Despite systemic thromboembolic complications being frequent, portal vein thrombosis is a rare complication of nephrotic syndrome. We report here a liver transplant recipient who presented a late extensive portal vein thrombosis related to nephrotic syndrome. During regular follow-up after liver transplant, the patient presented with diabetes, arterial hypertension, hypercholesterolemia, and progressive renal dysfunction. In addition, urine analysis showed isolated proteinuria, and the diagnosis of nephrotic syndrome was made 36 months after liver transplant...
June 5, 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28583409/portopulmonary-hypertension-and-hepatorenal-syndrome-two-faces-of-the-same-coin
#11
REVIEW
Enrico Maria Zardi, Domenico Maria Zardi, Chiara Giorgi, Diana Chin, Aldo Dobrina
Portopulmonary hypertension and hepatorenal syndrome are both severe local hypertensive complications of liver cirrhosis and portal hypertension. Both are characterized by vasoconstrictive manifestations regarding pulmonary and renal vascular network, respectively. This review addresses the mechanisms underlying the development of vasoconstriction that leads to local vascular hypertension in the lung and in the kidney with the result of organ dysfunction. Potential therapeutic options are available for the management of these two syndromes as a bridge for liver transplantation; clinical efficacy depends in part on the time and rapidity of intervention and in part on how serious the chain of events is that has triggered the entire vasoconstrictive process...
June 2, 2017: European Journal of Internal Medicine
https://www.readbyqxmd.com/read/28539032/elevated-gastric-antrum-erosions-in-portal-hypertension-patients-peptic-disease-or-mucosal-congestion
#12
Fernanda Cordeiro de Azevedo Conejo, Mabel Tatty Medeiros Fracassi, Maurício Saab Assef, Maurício Alves Ribeiro, Luiz Arnaldo Szutan, Fabio Gonçalves Ferreira
Background/Aims: Portal hypertension (PH) is a syndrome characterized by chronic increase in the pressure gradient between the portal vein and inferior vena cava. Previous studies have suggested an increased frequency of antral elevated erosive gastritis in patients with PH, as well as an etiologic association; however, there has not been any histological evidence of this hypothesis to date. Our aim was to evaluate the histological features found in elevated antral erosions in patients with portal hypertension...
May 25, 2017: Korean Journal of Gastroenterology, Taehan Sohwagi Hakhoe Chi
https://www.readbyqxmd.com/read/28533908/ascites-refractory-ascites-and-hyponatremia-in-cirrhosis
#13
Brett Fortune, Andres Cardenas
Ascites is the most common complication related to cirrhosis and is associated with increased morbidity and mortality. Ascites is a consequence of the loss of compensatory mechanisms to maintain the overall effective arterial blood volume due to worsening splanchnic arterial vasodilation as a result of clinically significant portal hypertension. In order to maintain effective arterial blood volume, vasoconstrictor and antinatriuretic pathways are activated, which increase overall sodium and fluid retention...
May 2017: Gastroenterology Report
https://www.readbyqxmd.com/read/28503308/portal-hypertension-as-the-initial-manifestation-of-poems-syndrome-a-case-report
#14
Lina Wu, Yue Li, Fang Yao, Chongmei Lu, Jian Li, Weixun Zhou, Jiaming Qian
BACKGROUND: Portal hypertension has a broad differential diagnosis. POEMS syndrome is an uncommon cause of it. POEMS syndrome is a rare disease involving multiple organs. In differential diagnosis of portal hypertension, POEMS syndrome should be considered especially when other symptoms such as numbness, organomegaly, endocrine alteration and skin changes also present, as it is highlighted by our case. CASE PRESENTATION: We report a 46-year-old Chinese male, a teacher, presenting with portal hypertension...
2017: BMC Hematology
https://www.readbyqxmd.com/read/28494529/nash-therapy-omega-3-supplementation-vitamin-e-insulin-sensitizers-and-statin-drugs
#15
Stephen Caldwell
Non-alcoholic steatohepatitis (NASH) is the more aggressive form of non-alcoholic fatty liver disease (NAFLD). NASH can progress to hepatic fibrosis, cirrhosis, portal hypertension and primary liver cancer. Therapy is evolving with a substantial number of trials of promising new agents now in progress. In this article however, we will examine data for several older forms of therapy which have been fairly extensively studied over the years: Polyunsaturated Fatty Acid (PUFA) supplements, vitamin E, insulin sensitizing agents with a focus on pioglitazone and statin agents...
May 10, 2017: Clinical and Molecular Hepatology
https://www.readbyqxmd.com/read/28489830/-clinical-and-laboratory-characteristics-of-patients-with-pulmonary-hypertension-and-pulmonary-vascular-complications-hospitalized-at-the-instituto-nacional-de-salud-del-ni%C3%A3-o
#16
Alexis Jose Ormeño Julca, Carlos Melchor Alvarez Murillo, Pedro Miguel Amoretti Alvino, Angel Aladino Florian Florian, Rosa Aurora Castro Johanson, Maria Danisa Celi Perez, Olga Rocío Huamán Prado
The hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHN) are distinct pulmonary vascular complications of portal hypertension (PHT) and are associated with increased morbidity and mortality. OBJECTIVES: To describe the clinical and laboratory characteristics of patients with pulmonary hypertension and pulmonary vascular complications hospitalized at the Instituto Nacional de Salud del Niño. MATERIALS AND METHODS: We included patients with HTP hospitalized from January 2012 to June 2013 and that during its evolution progressed with SHP or HTPP...
January 2017: Revista de Gastroenterología del Perú: órgano Oficial de la Sociedad de Gastroenterología del Perú
https://www.readbyqxmd.com/read/28468020/novel-approaches-to-reducing-the-risk-of-variceal-hemorrhage
#17
Frederik Nevens
BACKGROUND: Complications of advanced liver disease occur at the moment of clinical significant portal hypertension. Nitric oxide (NO) dysfunction and fibrosis play an important role in the pathophysiology of PH, but other mechanisms are also involved. Non-selective beta blockers (NSBB) stay the cornerstone in the primary and secondary prevention of variceal bleeding, but their safety in advanced cirrhosis has been recently debated and new drugs are under investigation. Transjugular intrahepatic portosystemic shunt and balloon tamponade are the standard therapy in case of refractory variceal bleeding, but both interventions have drawbacks...
2017: Digestive Diseases
https://www.readbyqxmd.com/read/28465872/a-rare-cause-of-portal-hypertension-behcet-s-disease-and-nodular-regenerative-hyperplasia-of-the-liver
#18
Cyriac Philips, Rajaguru Paramaguru, Lijesh Kumar, Padmanabha Shenoy, Philip Augustine
Nodular regenerative hyperplasia (NRH) is a rare liver condition in which widespread benign transformation of the hepatic parenchyma into small regenerative nodules occur, leading to development of non-cirrhotic portal hypertension. Conditions associated with NRH include rheumatologic, hematological, autoimmune, infectious, neoplastic, or drug-related etiology. Accurate diagnosis is made on liver biopsy, showing diffuse micronodular transformation in the absence of fibrosis. Here, we present the second case in world literature of a middle-aged man presenting with recent onset sleep reversal and memory loss with darkening of skin for four years associated with systemic manifestations, in whom porto-systemic shunt syndrome due to NRH secondary to Behcet's disease was eventually diagnosed...
March 29, 2017: Curēus
https://www.readbyqxmd.com/read/28433104/wilson-disease-symptomatic-liver-therapy
#19
Jan Pfeiffenberger, Karl-Heinz Weiss, Wolfgang Stremmel
Wilson disease leads to symptomatic impairment of liver function or liver cirrhosis. Strict adherence to decoppering agents is essential in these patients. Secondary prevention of additional hepatic damage by avoidance of other toxic substances (e.g., alcohol, drugs) and sufficient calorie intake is recommended. Routine examinations in cirrhotic patients include screening for signs of portal hypertension (esophagus varices), development of ascites, and hepatic encephalopathy. Where varices are present, primary or secondary preventive interventions may include treatment with nonselective beta-blockers or variceal ligation, similar to the approach in patients with liver cirrhosis due to other etiologies...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28402911/hassab-s-operation-for-joubert-syndrome-with-congenital-hepatic-fibrosis-a-case-report
#20
Koji Miyazawa, Yasuyuki Hara, Kenji Shimizu, Wataru Nakanishi, Kazuaki Tokodai, Chikashi Nakanishi, Shigehito Miyagi, Naoki Kawagishi, Noriaki Ohuchi
INTRODUCTION: Joubert syndrome is characterized by psychomotor developmental delay, hypotonia, oculomotor abnormalities, occasional retinal dystrophy and cystic kidneys, and frequent and often, striking breathing abnormalities, especially in the neonatal period, with panting tachypnea followed by apnea. We report a case of Joubert syndrome with hepatic fibrosis, portal hypertension, and pancytopenia treated by Hassab's operation. PRESENTATION OF CASE: Our patient was a 27-year-old woman with a history of tachypnea, muscle hypotonia, and psychomotor retardation shortly after birth and a diagnosis of Joubert syndrome at 2 years of age...
2017: International Journal of Surgery Case Reports
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