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portal hypertensive syndrome

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
Stefano Fiorucci, Angela Zampella, Giuseppe Cirino, Mariarosaria Bucci, Eleonora Distrutti
Bile acids are end product of cholesterol metabolism generated in the liver and released in the intestine. In addition to their role in nutrient absorption, bile acids are increasingly recognized as regulatory signals which 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 mediate the vasodilatory effects of bile acids in the systemic and splanchnic circulation...
October 7, 2016: American Journal of Physiology. Heart and Circulatory Physiology
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
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
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...
October 10, 2016: International Journal for Parasitology
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
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
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
Shikha S Sundaram, Cara L Mack, Amy G Feldman, Ronald J Sokol
Biliary Atresia is a progressive, fibro-obliterative disorder of the intra and extrahepatic bile ducts in infancy. The majority of affected children will eventually develop end-stage liver disease and require liver transplantation. Indications for liver transplant in biliary atresia include failed Kasai portoenterostomy, significant and recalcitrant malnutrition, recurrent cholangitis, and the progressive manifestations of portal hypertension. Extra-hepatic complications of this disease, such as hepatopulmonary syndrome and portopulmonary hypertension, are also indications for liver transplantation...
September 21, 2016: Liver Transplantation
Jiří Ehrmann, Květa Aiglová, Michal Konečný, Vlastimil Procházka, Drahomíra Vrzalová
UNLABELLED: There are 40 000-60 000 patients with cirrhosis in the Czech Republic. 2 000 die of this disease yearly. This group of patients needs a complex treatment and it is mostly an internist cooperating with other specialists. The most important for an ambulant internist is to diagnose the disease as soon as possible and start with treatment of chronic liver disease that could lead to a cirrhosis. It means especially chronic viral hepatitis, alcoholic or non-alcoholic steatosis/steatohepatitis, auto-immune liver damage and metabolic disease...
2016: Vnitr̆ní Lékar̆ství
Chao Zhang, Yongquan Gu, Ce Bian, Guangchang Zhu, Tao Luo, Chao Yan, Zhonggao Wang
Budd-Chiari syndrome (BCS) is a rare hepatic disease caused by occlusion of the hepatic venous outflow at any level from the small hepatic veins to the atrio-cava junction. BCS could have serious consequences if not treated promptly. The appropriate therapeutic strategy can be offered to change the natural course of the disease. The present case reports a young man with BCS who was successfully received the hybrid treatment combined with endovascular intervention and mesocaval shunt by step. The 11-year follow-up showed that the patient was free of clinical symptoms, CT and ultrasonography confirmed the patency of the stent and shunt...
August 20, 2016: Annals of Vascular Surgery
Mériam Koob, Danièle Pariente, Dalila Habes, Béatrice Ducot, Catherine Adamsbaum, Stéphanie Franchi-Abella
OBJECTIVES: To describe and evaluate an additional sonographic sign in the diagnosis of biliary atresia (BA), the microcyst of the porta hepatis, in comparison with previously described signs. METHODS: Ultrasound performed in 321 infants (mean age 55 days) with cholestasis were retrospectively analyzed. BA was surgically confirmed in 193 patients and excluded in 128. US evaluated gallbladder type (1: normal; 2: consistent with BA; 3: suspicious), triangular cord sign (TCS), microcyst and macrocyst, polysplenia syndrome, portal hypertension, and bile duct dilatation...
August 23, 2016: European Radiology
Joseph Frankl, Charles Hennemeyer, Michael S Flores, Archita P Desai
Chronic Budd-Chiari syndrome can present with cirrhosis and signs and symptoms similar to those of other chronic liver diseases. We present a case of Budd-Chiari syndrome discovered during attempted transjugular intrahepatic portosystemic shunting in a patient with decompensated cirrhosis believed to be secondary to hepatitis C. Although the patient had hepatocellular carcinoma, the Budd-Chiari syndrome was a primary disease due to hepatic venous webs. Angioplasty was performed in this case, which resolved the patient's symptoms related to portal hypertension...
2016: Case Reports in Hepatology
Parisá Khodayar-Pardo, Andrés Peña Aldea, Ana Ramírez González, Adela Meseguer Carrascosa, Cristina Calabuig Bayo
Extrahepatic portal vein obstruction, although rare in children, is a significant cause of portal hypertension (PHT) leading to life-threatening gastrointestinal bleeding in the pediatric age group. PHT may also lead to other complications such as hyperesplenism, cholangyopathy, ascites, and even hepatopulmonary syndrome and portopulmonary hypertension that may require organ transplantation. Herein we report the case of an asymptomatic 11-month-old infant wherein a hepatomegaly and cavernous transformation of the portal vein was detected by liver ultrasound...
May 2016: Case Reports in Gastroenterology
S T Lauren, S O Simons, A I J Arens
A 42-year-old man with a medical history of liver cirrhosis and portal hypertension was admitted to the hospital because of slowly progressive shortness of breath and hypoxemia. The diagnosis hepatopulmonary syndrome was confirmed by a pulmonary perfusion scan with 99m Tc-albumin. The scan showed a right-left shunt, because the 99m Tc-albumin transited the lungs and appeared in the brain, the thyroid gland, the kidneys and the spleen. The patient received a liver transplantation, which is considered the only definitive treatment...
2016: Nederlands Tijdschrift Voor Geneeskunde
Salim R Surani, Yamely Mendez, Humayun Anjum, Joseph Varon
Severe chronic liver disease (CLD) may result from portal hypertension, hepatocellular failure or the combination of both. Some of these patients may develop pulmonary complications independent from any pulmonary pathology that they may have. Among them the hepatopulmonary syndrome (HPS), portopulmonary hypertension (PPH) and hepatic hydrothorax (HH) are described in detail in this literature review. HPS is encountered in approximately 15% to 30% of the patients and its presence is associated with increase in mortality and also requires liver transplantation in many cases...
July 14, 2016: World Journal of Gastroenterology: WJG
Anahita Dua, Lisa McElroy, Abby Wochinski, Johnny C Hong, David C Cronin
Successful liver transplantation typically results in an immediate decrease in intrahepatic resistance accompanied by an initial increased hepatopedal portal flow. Within a short period of time, the portal hypertension resolves and the variceal shunts involute. However, in situations in which intrahepatic vascular resistance to venous flow remains elevated, significant hepatofugal portal flow may continue through persistent mesenteric shunts. This situation, portal steal, can result in decreased perfusion of the liver graft leading to graft dysfunction, failure, and potentially recipient death...
June 2016: WMJ: Official Publication of the State Medical Society of Wisconsin
Jin Hee Jeong, In Sung Park, Dong Hoon Kim, Seong Chun Kim, Changwoo Kang, Soo Hoon Lee, Tae Yun Kim, Sang Bong Lee
Hepatic encephalopathy (HE) is a complication associated with worst prognosis in decompensated liver cirrhosis (LC) patients. Previous studies have identified prognostic factors for HE, and recent studies reported an association between systemic inflammatory response syndrome (SIRS) and liver disease. This study aimed to identify prognostic factors for 30-day mortality in alcoholic LC patients with HE who visited the emergency department (ED).This was a retrospective study of alcoholic LC patients with HE from January 1, 2010, to April 30, 2015...
June 2016: Medicine (Baltimore)
Seiichi Kawabata, Seisuke Sakamoto, Masaki Honda, Shintaro Hayashida, Hidekazu Yamamoto, Yoshiki Mikami, Yukihiro Inomata
BACKGROUND: Liver involvement in Turner syndrome (TS) patients has been more clearly clarified in recent years. Most of the clinical manifestations in TS are asymptomatic and can be detected as liver test abnormalities; however, a few cases may present with end-stage liver disease and thus require liver transplantation (LT). To the best of our knowledge, only three cases undergoing LT for liver involvements in TS have been previously reported. CASE PRESENTATION: A 30-year-old female successfully underwent living donor LT for liver dysfunction related to TS syndrome...
December 2016: Surgical Case Reports
Gianni Testino, Silvia Leone, Valentino Patussi, Emanuele Scafato, Paolo Borro
Hepatocellular carcinoma (HCC) ranks third among the causes of cancer deaths globally. The most frequent causes are the hepatitis C virus (HCV), a combination of alcohol/HCV and metabolic syndrome (MS). The introduction of new pharmaceutical drugs that inhibit protease will bring a relative increase in the number of cases of HCC that are linked to the consumption of alcohol and MS. The latest development in the diagnostic sector is the total recognition of the contrast-enhanced ultrasound diagnostic algorithm...
December 2016: Minerva Medica
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