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Noncirrhotic portal hypertension

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https://www.readbyqxmd.com/read/29515345/adult-presentation-of-noncirrhotic-portal-hypertension-and-ascites-following-treatment-for-wilms-tumor-in-childhood
#1
Linda Kievit, Pia Kræmer, Stephen Hamilton-Dutoit, Henning Grønbæk
A 37-year-old male, who at the age of 8 years had been treated for right-sided Wilms' tumor with nephrectomy, radiotherapy, and chemotherapy, presented with noncirrhotic portal hypertension (NCPH), grade 2 esophageal varices, and ascites. A CT scan demonstrated hypoplasia of liver segments 2 and 3. A liver biopsy showed portal tract fibrosis without cirrhosis, with histological features of NCPH. Liver vein catheterization showed a normal portal pressure gradient of 5 mm Hg while spleen to hepatic vein pressure was 29 mm Hg...
January 2018: Case Reports in Gastroenterology
https://www.readbyqxmd.com/read/29465431/noncirrhotic-portal-hypertension
#2
Ben L Da, Christopher Koh, Theo Heller
PURPOSE OF REVIEW: Noncirrhotic portal hypertension represents a heterogeneous group of liver disorders that is characterized by portal hypertension in the absence of cirrhosis. The purpose of this review is to serve as a guide on how to approach a patient with noncirrhotic portal hypertension with a focus on recent developments. RECENT FINDINGS: Recent studies pertaining to noncirrhotic portal hypertension have investigated aetiological causes, mechanisms of disease, noninvasive diagnostic modalities, clinical characteristics in the paediatric population and novel treatment targets...
May 2018: Current Opinion in Gastroenterology
https://www.readbyqxmd.com/read/29438119/liver-disease-in-patients-with-cystic-fibrosis
#3
Natasha Kamal, Pallavi Surana, Christopher Koh
PURPOSE OF REVIEW: The aim of this study was to provide an overview of the current understanding of the pathophysiology, diagnosis and management of cystic fibrosis-liver disease (CFLD). RECENT FINDINGS: CFLD has a variety of manifestations. Previously, it was thought that patients progressed from mild cholestatic disease to cirrhosis to decompensated cirrhosis with portal hypertension. Newer evidence suggests that some patients may develop cirrhosis while others develop noncirrhotic portal hypertension...
May 2018: Current Opinion in Gastroenterology
https://www.readbyqxmd.com/read/29324586/ten-year-experience-of-transjugular-intrahepatic-portosystemic-shunt-for-noncirrhotic-portal-hypertension
#4
David Regnault, Louis d'Alteroche, Charlotte Nicolas, Fanny Dujardin, Jean Ayoub, Jean Marc Perarnau
BACKGROUND: Transjugular intrahepatic portosystemic shunt (TIPS) is considered to be well suited for the treatment of noncirrhotic portal hypertension (NCPHT) because of a usually severe portal hypertension (PHT) and a mild liver failure, but very less data are available. PATIENTS AND METHODS: Records of patients referred for TIPS between 2004 and 2015 for NCPHT were reviewed. No patient should have clinical or biological or histological features of cirrhosis. RESULTS: Twenty-five patients with a wide variety of histological lesions (sinusoidal dilatations, granulomatosis, regenerative nodular hyperplasia, obliterative portal venopathy, or subnormal liver) and a wide variety of associated diseases (thrombophilia, sarcoidosis, common variable immunodeficiency, scleroderma, Castleman's disease, early primitive biliary cirrhosis, congenital liver fibrosis, chemotherapy, purinethol intake, and congenital varices) were included...
May 2018: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/29315156/bleeding-risk-of-variceal-band-ligation-in-extrahepatic-portal-vein-obstruction-is-not-increased-by-oral-anticoagulation
#5
Maeva Guillaume, Camille Christol, Aurélie Plessier, Michèle Corbic, Jean-Marie Péron, Agnès Sommet, Pierre-Emmanuel Rautou, Yann Consigny, Jean-Pierre Vinel, Charles-Dominique Valla, Christophe Bureau
BACKGROUND AND OBJECTIVES: Noncirrhotic nontumoral extrahepatic portal vein obstruction (EHPVO) is the second leading cause of portal hypertension (PHT) and is mainly related to prothrombotic disorders. Patients with EHPVO often require prolonged oral anticoagulation therapy (OAT) together with variceal band ligation (VBL) to prevent thrombosis recurrence and PHT-related bleeding, respectively. The benefit-risk balance of VBL in this context remains unknown. We aimed to assess upper gastrointestinal bleeding (UGB) risk and variceal eradication efficacy in EHPVO patients undergoing a VBL program without stopping OAT...
May 2018: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/29234547/testicular-cancer-presenting-as-gastric-variceal-hemorrhage
#6
Carlos Eduardo Salazar-Mejía, David Hernández-Barajas, Edio Llerena-Hernández, José Luis González-Vela, María Inés Contreras-Salcido, Adriana González-Gutiérrez, Omar David Borjas-Almaguer, Luis Alberto Pérez-Arredondo, Blanca Otilia Wimer-Castillo
Testicular cancer is the most common solid malignancy affecting males between the ages of 15 and 35. The symptomatology caused by this tumor varies according to the site of metastasis. We present the case of a 26-year-old male who arrived to the emergency department with hematemesis. He had no previous medical history. On arrival, we noted enlargement of the left scrotal sac. There was also a mass in the left scrotum which provoked displacement of the penis and right testis. The serum alpha-fetoprotein level was 17,090 ng/mL, lactate dehydrogenase was 1480 U/L, and human chorionic gonadotropin was 287...
2017: Case Reports in Gastrointestinal Medicine
https://www.readbyqxmd.com/read/28733177/transjugular-local-thrombolysis-with-without-tips-in-patients-with-acute-non-cirrhotic-non-malignant-portal-vein-thrombosis
#7
Christoph Klinger, Bettina Riecken, Arthur Schmidt, Andrea De Gottardi, Benjamin Meier, Jaime Bosch, Karel Caca
BACKGROUND & AIMS: Therapeutic anticoagulation is the standard treatment in patients with acute non-cirrhotic portal vein thrombosis (PVT). In critically ill patients, anticoagulation only may not suffice to achive rapid and stable recanalization. This study evaluates efficacy and safety of transjugular interventional therapy in acute non-cirrhotic PVT. METHODS: This retrospective study includes 17 consecutive patients with acute noncirrhotic, non-malignant PVT...
December 2017: Digestive and Liver Disease
https://www.readbyqxmd.com/read/28597108/prevalence-of-histological-features-of-idiopathic-noncirrhotic-portal-hypertension-in-general-population-a-retrospective-study-of-incidental-liver-biopsies
#8
Chunlai Zuo, Vaibhav Chumbalkar, Peter F Ells, Daniel J Bonville, Hwajeong Lee
BACKGROUND: Idiopathic noncirrhotic portal hypertension (INCPH) is associated with histologic changes secondary to obliterative portal venopathy without cirrhosis. We studied the prevalence of individual histological features of INCPH in liver biopsies obtained incidentally during unrelated elective procedures and in elective liver biopsies with the diagnosis of fatty liver disease. METHODS: A total of 53 incidental liver biopsies obtained intraoperatively during unrelated elective procedures and an additional 28 elective biopsies with the diagnosis of fatty liver disease without portal hypertension and cirrhosis were studied...
September 2017: Hepatology International
https://www.readbyqxmd.com/read/28588619/portal-hypertensive-biliopathy-presents-with-massive-bleeding-during-ercp-after-balloon-sphincteroplasty-in-a-noncirrhotic-saudi-sickler-patient
#9
Ahmad M Al-Akwaa, Mohammed Elsadig, Ahmed E Al-Fayaa, Mohja D Al-Shehri
Portal hypertensive biliopathy (PHB) is described as abnormalities of the walls of the biliary tree secondary to portal hypertension. Gastrointestinal bleeding caused by PHB is rare. PHB as a cause of serious bleeding after sphincteroplasty during ERCP is extremely rare. Here, we report a case of PHB in a young Saudi male with cell sickle anemia who developed massive hemorrhage during ERCP after balloon dilation of the ampulla of Vater. We further discussed the diagnosis and management. To the best of our knowledge, no such case has been reported...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28491877/nodular-regenerative-hyperplasia-a-case-of-rare-prognosis
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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