Nodular regenerative hyperplasia of the liver: a cause of noncirrhotic portal hypertension

1984 ◽  
Vol 51 (3) ◽  
pp. 559-564 ◽  
Author(s):  
W. R. Hart ◽  
R. J. Tuthill
Hepatology ◽  
1998 ◽  
Vol 28 (2) ◽  
pp. 385-390 ◽  
Author(s):  
Michael F. McEntee ◽  
Kathy N. Wright ◽  
Ian Wanless ◽  
Robert DeNovo ◽  
John F. Schneider ◽  
...  

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Andrew T. Turk ◽  
Matthias J. Szabolcs ◽  
Jay H. Lefkowitch

Nodular regenerative hyperplasia (NRH) of the liver is associated with noncirrhotic portal hypertension, rheumatologic and hematologic disorders, administration of certain drugs, and other underlying conditions. This report describes a 64-year-old man with clinically presumed cirrhosis who presented to our institution with coffee-ground emesis, esophageal varices, ascites, and encephalopathy. Eleven years earlier he had been treated for breast cancer with mastectomy and chemo-radiotherapy. He died suddenly, and the autopsy showed no evidence of cirrhosis but instead demonstrated NRH with extensive emboli of recurrent breast carcinoma within the portal vein and its intrahepatic branches. Neoplastic occlusion of the portal vein as a cause of presinusoidal noncirrhotic portal hypertension has not previously been reported for metastatic breast carcinoma. This case highlights the importance of obstructive portal venopathy in the pathogenesis of NRH as well as the diagnostic difficulties that may be encountered in determining the cause of portal hypertension.


2012 ◽  
Vol 2012 ◽  
pp. 1-5 ◽  
Author(s):  
Lisa M. Louwers ◽  
Jared Bortman ◽  
Alan Koffron ◽  
Veslav Stecevic ◽  
Steven Cohn ◽  
...  

Nodular regenerative hyperplasia (NRH) is an uncommon condition, but an important cause of noncirrhotic intrahepatic portal hypertension (NCIPH), characterized by micronodules of regenerative hepatocytes throughout the liver without intervening fibrous septae. Herein, we present a case of a thirty-seven-year-old female with systemic lupus erythematosus (SLE) who was discovered to have significant esophageal varices on endoscopy for dyspepsia. Her labs revealed a slight elevation in the alkaline phosphatase and mild thrombocytopenia. Abdominal MRI revealed seven focal hepatic masses, splenomegaly, no ascites, and a patent portal vein. Ultrasound-guided core biopsy was reported as focal nodular hyperplasia. However, her varices persisted despite treatment with beta-blockers and four additional upper endoscopies with banding. She was subsequently referred for a surgical opinion. At that time, given her history of SLE, azathioprine use, and portal hypertension, suspicion for NRH was raised. Given her normal synthetic function and lack of parenchymal liver disease, the patient was offered surgical shunting. During shunt surgery, a liver wedge biopsy was also performed and this confirmed NRH. An upper endoscopy six weeks after shunting verified complete resolution of varices. Currently, fifteen months after surgery duplex ultrasonography demonstrates shunt patency and the patient is without recurrence of her portal hypertension.


2017 ◽  
Vol 5 (1) ◽  
pp. 232470961769074
Author(s):  
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. She was referred to our Hepatology service for evaluation of splenomegaly, elevated liver tests, and thrombocytopenia. On initial presentation, she reported easy bruising and gingival bleeding, and abdominal imaging showed evidence of portal hypertension without associated cirrhosis. Upper endoscopy was significant for large esophageal varices without bleeding stigmata. Liver biopsy showed minimal fibrosis around the portal areas without significant inflammation. The lobules showed focal zones of thin hepatocyte plates on reticulin stain with adjacent areas showing mild regenerative changes. The diagnosis of NRH was made and patient was placed on propranolol for variceal bleeding prophylaxis. Two years later, the patient presented with bleeding gastric varices warranting transjugular intrahepatic portosystemic shunt. Postprocedure course was complicated by mild encephalopathy. Subsequent magnetic resonance imaging showed a 1.7 × 1.3 cm lesion suggestive of hepatocellular carcinoma (HCC). The patient was deemed to be a candidate for liver transplantation, and she is now delisted due to ongoing pregnancy. Conclusion: This report describes the first case of HCC in an HIV patient with NRH. The possible association of NRH with HCC warrants further investigation.


2019 ◽  
Vol 6 (12) ◽  
pp. e00257 ◽  
Author(s):  
Cindy Piao ◽  
Abhinav Koul ◽  
Dorina Gui ◽  
Ling-xin Chen ◽  
Souvik Sarkar

2021 ◽  
Vol 9 ◽  
pp. 232470962110446
Author(s):  
Prarthana Jain ◽  
Sagar Patel ◽  
Heather N. Simpson ◽  
Richard M. Silver ◽  
David N. Lewin ◽  
...  

Nodular regenerative hyperplasia (NRH) is a rare disease that is characterized by benign transformation of the hepatic parenchyma into small nodules with little to no fibrosis. Nodular regenerative hyperplasia is a cause of noncirrhotic portal hypertension. Symptoms can range from asymptomatic disease to more serious complications of portal hypertension such as esophageal varices and ascites. Nodular regenerative hyperplasia has been described in association with a variety of different rheumatologic, hematologic, and oncologic diseases, as well as in immune deficiency states and with exposures to certain toxins. Diagnosis is made by histology, and the treatment involves addressing the underlying disease. The first description of this rare disease was actually described in a patient with rheumatoid arthritis, neutropenia, and splenomegaly (Felty’s Syndrome). We describe 2 cases of NRH associated with underlying rheumatic disorders, in one of which NRH was actually the presenting feature of the patient’s underlying autoimmune condition. Subsequently, we provide a brief review of the literature of NRH in autoimmune disease with respect to epidemiology, cause, clinical manifestations, diagnosis, and treatment.


2018 ◽  
Vol 2018 ◽  
pp. 1-4 ◽  
Author(s):  
Gary A. Abrams ◽  
Robert Chapman ◽  
Samuel R. W. Horton

We report on a rare case of refractory hepatic hydrothorax in an individual with Scleroderma/CREST syndrome and noncirrhotic portal hypertension. Portal pressure measurements revealed a normal transjugular hepatic venous portal pressure gradient, mild pulmonary hypertension, and an unremarkable liver biopsy except for mild sinusoidal dilation. Pulmonary hypertension, cardiac diastolic dysfunction, and chronic kidney disease were determined to be the causes of his refractory pleural effusions and ascites. Over the year, he underwent 50 thoracenteses and 20 paracenteses averaging 10–12 liters/week. Repeat pulmonary evaluation determined his pulmonary pressures to be normal and a secondary review of the “unremarkable” liver biopsy noted mild venous outflow obstruction and possibly Nodular Regenerative Hyperplasia (NRH). Repeat portal pressures indirectly and directly confirmed the existence of presinusoidal portal hypertension that has been associated with NRH. A transjugular intrahepatic portal systemic shunt (TIPS) was placed and he has not required thoracentesis or paracentesis over the past 18 months.


2018 ◽  
Vol 1 (1) ◽  
pp. 14-16
Author(s):  
Soonthorn Chonprasertsuk

The noncirrhotic portal hypertension is an uncommon cause of bleeding esophagealvarices. This condition must be suspected in patients with preserved liver function. We reporta 25-year old man with SLE disease who presented with hematemesis. He had no historyor risk factors for an underlying liver condition. A huge splenomegaly was detectedby physical examination. The EGD found three large varices with red wale sign, whereas liverfunction tests were unremarkable. The noncirrhotic portal hypertension was diagnosedand confirmed by liver histopathology. Figure 1 แสดงผลการส่องกล้องทางเดินอาหารส่วนบนพบ F3 varices with red wale sign


2012 ◽  
Vol 36 (9) ◽  
pp. 875-885 ◽  
Author(s):  
J. N. L. Schouten ◽  
M. E. Van der Ende ◽  
T. Koëter ◽  
H. H. M. Rossing ◽  
M. Komuta ◽  
...  

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