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Noncirrhotic portal hypertension

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https://www.readbyqxmd.com/read/28491877/nodular-regenerative-hyperplasia-a-case-of-rare-prognosis
#1
Mindy Lee, Manhal Izzy, Ashwin Akki, Kathryn Tanaka, Harmit Kalia
Introduction: Nodular regenerative hyperplasia (NRH) is a known etiology of noncirrhotic portal hypertension. Cases of biopsy-proven NRH in human immunodeficiency virus (HIV)-positive patients have been described. While these patients often have normal synthetic liver function, several reports described disease progression to liver failure. Case: We here present a 26-year-old woman with history of congenital HIV on antiretroviral therapy complicated by Pneumocystis carinii pneumonia at age 14. CD4 counts have been >300 with undetectable viral load...
January 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28437324/noncirrhotic-portal-fibrosis-in-pediatric-population
#2
Vikrant Sood, Bikrant B Lal, Rajeev Khanna, Dinesh Rawat, Chhagan Bihari, Seema Alam
OBJECTIVES: Noncirrhotic portal fibrosis (NCPF) has been classically described as a disease of young to middle age with limited literature regarding its occurrence, onset, or clinical presentation in children. We hereby present a series of 19 patients diagnosed and managed as NCPF in pediatric age group. METHODS: A retrospective review of all the patients presenting to the pediatric hepatology department (age <18 years) and diagnosed as NCPF was done and data were evaluated...
May 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28295661/non-cirrhotic-portal-hypertension-in-necrobiotic-xanthogranuloma-associated-with-monoclonal-gammopathy
#3
LETTER
M Vignon, L Placais, M Malphettes, J D Bouaziz, B Asli, P Bedossa, J Rivet, R Szalat, A M Zagdanski, M Rybojad, J P Fermand, M Baron, P E Rautou, B Arnulf
No abstract text is available yet for this article.
March 10, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28223722/to-screen-or-not-to-screen-celiac-antibodies-in-liver-diseases
#4
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/28151736/nodular-regenerative-hyperplasia-of-the-liver-in-patients-with-ibd-treated-with-allopurinol-thiopurine-combination-therapy
#5
Margien L Seinen, Dirk P van Asseldonk, Nanne K de Boer, Gerd Bouma, Carin M van Nieuwkerk, Chris J Mulder, Elisabeth Bloemena, Adriaan A van Bodegraven
BACKGROUND: Thiopurine therapy, particularly thioguanine, has been associated with nodular regenerative hyperplasia (NRH) of the liver. Combination therapy of allopurinol and an adapted low-dose thiopurine leads to a pharmacokinetic profile that has similarities to that of thioguanine. Therefore, allopurinol-thiopurine combination therapy may also be associated with NRH of the liver. We assessed the prevalence of NRH in patients with inflammatory bowel disease (IBD) treated with allopurinol-thiopurine combination therapy by liver biopsy specimen examination...
March 2017: Inflammatory Bowel Diseases
https://www.readbyqxmd.com/read/27920646/successful-treatment-of-small-intestinal-bleeding-in-a-crohn-s-patient-with-noncirrhotic-portal-hypertension-by-transjugular-portosystemic-shunt-placement-and-infliximab-treatment
#6
Benjamin Heimgartner, Heather Dawson, Andrea De Gottardi, Reiner Wiest, Jan Hendrik Niess
Small intestinal bleeding in Crohn's disease patients with noncirrhotic portal hypertension and partial portal and superior mesenteric vein thrombosis is a life-threatening event. Here, a case is reported in which treatment with azathioprine may have resulted in nodular regenerative hyperplasia, portal hypertension and portal vein thrombosis. The 56-year-old patient with Crohn's disease developed nodular regenerative hyperplasia under treatment with azathioprine. He was admitted with severe bleeding. Gastroscopy showed small esophageal varices without bleeding stigmata...
September 2016: Case Reports in Gastroenterology
https://www.readbyqxmd.com/read/27843428/a-rare-case-of-gastric-variceal-hemorrhage-secondary-to-infiltrative-b-cell-lymphoma
#7
Adrienne Lenhart, Juan Fernandez-Castillo, Keith Mullins, Reena Salgia
Portal hypertension commonly arises in the setting of advanced liver cirrhosis and is the consequence of increased resistance within the portal vasculature. Less commonly, left-sided noncirrhotic portal hypertension can develop in a patient secondary to isolated obstruction of the splenic vein. We present a rare case of left-sided portal hypertension and isolated gastric varices in a patient with large B-cell lymphoma, who was treated with splenic artery embolization. The patient is a 73-year-old male with no previous history of liver disease, who presented with coffee ground emesis and melena...
September 2016: Case Reports in Gastroenterology
https://www.readbyqxmd.com/read/27807573/portal-hypertension-and-ascites-due-to-an-arterioportal-fistula-sequela-of-a-remote-traumatic-liver-laceration
#8
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/27755254/risk-factors-associated-with-complications-in-cirrhotic-patients-undergoing-endoscopic-retrograde-cholangio-pancreatography
#9
Ricardo U Macías-Rodríguez, Astrid Ruiz-Margáin, Jose L Rodriguez-Garcia, Sergio Zepeda-Gómez, Aldo Torre
OBJECTIVE: Endoscopic retrograde cholangio-pancreatography (ERCP) is useful for the management of biliary tract diseases; in patients with cirrhosis, portal hypertension may increase the risk for complications from ERCP. We evaluated the outcome and risk factors related to ERCP in patients with cirrhosis and portal hypertension. PATIENTS AND METHODS: In this case-control study, 37 patients (71 procedures) with cirrhosis and portal hypertension (group 1) and 37 controls (group 2) undergoing ERCP were included...
February 2017: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/27695164/safety-and-efficacy-of-2-octyl-cyanoacrylate-in-the-management-of-patients-with-gastric-and-duodenal-varices-who-are-not-candidates-for-transjugular-intrahepatic-portosystemic-shunts
#10
Luis Lizardo-Sanchez, James Burdick, James F Trotter
Gastric variceal bleeding is associated with significant morbidity and mortality in patients with portal hypertension and cirrhosis. Options are limited for patients who are not candidates for transjugular intrahepatic portosystemic shunts (TIPS). Cyanoacrylate injections have been reported to be efficacious in previous case series. The aim of this retrospective study was to report our single-center experience with the safety and efficacy of 2-octyl-cyanoacrylate in patients who were not TIPS candidates. Electronic medical records were reviewed for 16 patients who underwent a total of 18 esophagogastroduodenoscopies for acute gastric or duodenal variceal bleeding and secondary prophylaxis of gastric varices; 14 patients had cirrhosis with an average Model for End-Stage Liver Disease score of 16, and 2 patients had noncirrhotic portal hypertension...
October 2016: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/27663417/portal-hypertension-in-children-high-risk-varices-primary-prophylaxis-and-consequences-of-bleeding
#11
Mathieu Duché, Béatrice Ducot, Oanez Ackermann, Florent Guérin, Emmanuel Jacquemin, Olivier Bernard
BACKGROUND & AIMS: Primary prophylaxis of bleeding is debated for children with portal hypertension because of the limited number of studies on its safety and efficacy, the lack of a known endoscopic pattern carrying a high-risk of bleeding for all causes, and the assumption that the mortality of a first bleed is low. We report our experience with these issues. METHODS: From 1989 to 2014, we managed 1300 children with portal hypertension. Endoscopic features were recorded; high-risk varices were defined as: grade 3 esophageal varices, grade 2 varices with red wale markings, or gastric varices...
February 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/27638992/mesenteric-vein-thrombosis-can-be-safely-treated-with-anticoagulation-but-is-associated-with-significant-sequelae-of-portal-hypertension
#12
Thomas S Maldonado, Sheila N Blumberg, Sharvil U Sheth, Gabriel Perreault, Mikel Sadek, Todd Berland, Mark A Adelman, Caron B Rockman
BACKGROUND: Mesenteric venous thrombosis (MVT) is a relatively uncommon but potentially lethal condition associated with bowel ischemia and infarction. The natural history and long-term outcomes are poorly understood and under-reported. METHODS: A single-institution retrospective review of noncirrhotic patients diagnosed with MVT from 1999 to 2015 was performed using International Classification of Diseases, Ninth Revision and radiology codes. Patients were excluded if no radiographic imaging was available for review...
October 2016: Journal of Vascular Surgery. Venous and Lymphatic Disorders
https://www.readbyqxmd.com/read/27555800/idiopathic-noncirrhotic-portal-hypertension-current-perspectives
#13
REVIEW
Oliviero Riggio, Stefania Gioia, Ilaria Pentassuglio, Valeria Nicoletti, Michele Valente, Giulia d'Amati
The term idiopathic noncirrhotic portal hypertension (INCPH) has been recently proposed to replace terms, such as hepatoportal sclerosis, idiopathic portal hypertension, incomplete septal cirrhosis, and nodular regenerative hyperplasia, used to describe patients with a hepatic presinusoidal cause of portal hypertension of unknown etiology, characterized by features of portal hypertension (esophageal varices, nonmalignant ascites, porto-venous collaterals), splenomegaly, patent portal, and hepatic veins and no clinical and histological signs of cirrhosis...
2016: Hepatic Medicine: Evidence and Research
https://www.readbyqxmd.com/read/27225799/arsenicosis-possibly-from-contaminated-groundwater-associated-with-noncirrhotic-intrahepatic-portal-hypertension
#14
Ashish Goel, Pamela Christudoss, Renu George, Banumathi Ramakrishna, G Jayakumar Amirtharaj, Shyamkumar N Keshava, Anup Ramachandran, K A Balasubramanian, Ian Mackie, Jude J Fleming, Elwyn Elias, Chundamannil E Eapen
BACKGROUND AND AIMS: Idiopathic noncirrhotic intrahepatic portal hypertension (NCIPH), a chronic microangiopathy of the liver caused by arsenicosis from use of contaminated groundwater, was reported from Asia. This study aimed to see, if in the twenty-first century, arsenicosis was present in NCIPH patients at our hospital and, if present, to look for groundwater contamination by arsenic in their residential locality. METHODS: Twenty-seven liver biopsy proven NCIPH patients, 25 portal hypertensive controls with hepatitis B or C related cirrhosis and 25 healthy controls, matched for residential locality, were studied...
May 2016: Indian Journal of Gastroenterology: Official Journal of the Indian Society of Gastroenterology
https://www.readbyqxmd.com/read/27218209/the-role-of-ascitic-fluid-viscosity-in-differentiating-the-nature-of-ascites-and-in-the-prediction-of-renal-impairment-and-duration-of-icu-stay
#15
Amr S Hanafy
BACKGROUND AND AIM: Serum-ascites albumin gradient (SAAG) has been used in the classification of ascites for the last 20 years but it has some drawbacks. This study searches for possible correlations between ascitic fluid viscosity and the etiology of ascites, renal impairment, and length of ICU stay. MATERIALS AND METHODS: The study was conducted in Zagazig University Hospital, Egypt. It included 240 patients with ascites due to various causes. The patients were divided into two groups: the cirrhotic ascites group, which included 120 patients, and the noncirrhotic ascites group, which included 120 patients...
September 2016: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/27194018/portosystemic-shunt-surgery-in-patients-with-idiopathic-noncirrhotic-portal-hypertension
#16
Servet Karagul, Mehmet Ali Yagci, Ali Tardu, Ismail Ertugrul, Serdar Kirmizi, Fatih Sumer, Burak Isik, Cuneyt Kayaalp, Sezai Yilmaz
BACKGROUND Idiopathic noncirrhotic portal hypertension (INCPH) is a rare disease characterized by increased portal venous pressure in the absence of cirrhosis and other causes of liver diseases. The aim of the present study was to present our results in using portosystemic shunt surgery in patients with INCPH. MATERIAL AND METHODS Patients who had been referred to our Liver Transplantation Institute for liver transplantation and who had undergone surgery from January 2010 to December 2015 were retrospectively analyzed...
May 19, 2016: Annals of Transplantation: Quarterly of the Polish Transplantation Society
https://www.readbyqxmd.com/read/27167116/noncirrhotic-portal-hypertension-in-perinatally-hiv-infected-adolescents-treated-with-didanosine-containing-antiretroviral-regimens-in-childhood
#17
Henriette J Scherpbier, Valeska Terpstra, Dasja Pajkrt, Thanyawee Puthakanit, Jintanat Ananworanich, Caroline Foster, Marius van den Bergh Weerman, Eline E Deurloo, Marc van der Valk, Taco W Kuijpers, Bart G Koot
BACKGROUND: Noncirrhotic portal hypertension (NCPH) has been reported in HIV-infected adults. Antiretroviral drugs, as well as genetic and thrombophilic predisposition, have been suggested as possible etiologic factors. METHODS: Clinical data were collected from 6 HIV-infected patients attending the Infectious Diseases Departments at respectively Emma Children's Hospital Academic Medical Centre in Amsterdam, The Thai Red Cross AIDS Research Centre, Bangkok, Imperial College Healthcare NHS Trust, London who were diagnosed with NCPH...
August 2016: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/26990687/role-of-the-transjugular-intrahepatic-portosystemic-shunt-in-the-management-of-severe-complications-of-portal-hypertension-in-idiopathic-noncirrhotic-portal-hypertension
#18
Julien Bissonnette, Juan Carlos Garcia-Pagán, Agustín Albillos, Fanny Turon, Carlos Ferreira, Luis Tellez, Jean-Charles Nault, Nicolas Carbonell, Jean-Paul Cervoni, Mohamed Abdel Rehim, Annie Sibert, Louis Bouchard, Pierre Perreault, Jonel Trebicka, Félix Trottier-Tellier, Pierre-Emmanuel Rautou, Dominique-Charles Valla, Aurélie Plessier
UNLABELLED: Idiopathic noncirrhotic portal hypertension is a heterogeneous group of diseases characterized by portal hypertension in the absence of cirrhosis. The efficacy and safety of transjugular intrahepatic portosystemic shunt (TIPS) in this population are unknown. The charts of patients with idiopathic noncirrhotic portal hypertension undergoing TIPS in seven centers between 2000 and 2014 were retrospectively reviewed. Forty-one patients were included. Indications for TIPS were recurrent variceal bleeding (n = 25) and refractory ascites (n = 16)...
July 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/26904707/splenic-marginal-zone-lymphoma-in-the-setting-of-noncirrhotic-portal-hypertension
#19
Saman Ratnayake, Ali Ammar, Rodd Rezvani, Greti Petersen
We present a case of a 65-year-old Hispanic man with a history of disseminated cutaneous coccidioidomycosis who presented to the emergency room for progressively worsening abdominal pain associated with shortness of breath. The patient was found to have pleural effusion and moderate ascites on physical examination. Abdominal ultrasound and computed tomography scan were consistent with moderate ascites and portal hypertension but negative for both liver cirrhosis and for venous or arterial thrombosis. Cytology of ascitic fluid was suggestive of portal hypertension and was negative for infection...
October 2015: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/26895906/laparoscopic-hepatectomy-in-cirrhotics-safe-if-you-adjust-technique
#20
David J Worhunsky, Monica M Dua, Thuy B Tran, Bernard Siu, George A Poultsides, Jeffrey A Norton, Brendan C Visser
BACKGROUND: Minimally invasive liver surgery is a growing field, and a small number of recent reports have suggested that laparoscopic liver resection (LLR) is feasible even in patients with cirrhosis. However, parenchymal transection of the cirrhotic liver is challenging due to fibrosis and portal hypertension. There is a paucity of data regarding the technical modifications necessary to safely transect the diseased parenchyma. METHODS: Patients undergoing LLR by a single surgeon between 2008 and 2015 were reviewed...
October 2016: Surgical Endoscopy
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