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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Liver transplantation has been performed in individuals with a pretransplant clinical diagnosis of cirrhosis but with nodular regenerative hyperplasia histologically. The purpose of this report is to investigate the results of liver transplantation in patients proven to have nodular regenerative hyperplasia post-transplant. A retrospective review was undertaken of four patients who underwent liver transplantation with a histologic diagnosis of nodular regenerative hyperplasia. All were felt to be cirrhotic on clinical grounds. Final histology of the explanted liver was confirmed by a single pathologist. Their ages ranged from 39 to 54 years, and three of the four were male. Three had pretransplant needle liver biopsies, two percutaneous and one transjugular. All revealed nonspecific reactive changes. Ultrasound and MRI were interpreted as consistent with cirrhosis in four of four and three of four cases, respectively. Portal vein flow was hepatopedal in three and absent in one. Pretransplant clinical characteristics and frequency were as follows: bleeding varices two, clinical ascites three, encephalopathy three, and impaired hepatic synthetic function two. All four patients underwent successful liver transplantation. There were no episodes of acute rejection. All are alive and well with normal graft function 2 to 4 years post-transplant. We conclude the following. 1) Patients with clinical end-stage liver disease due to underlying nodular regenerative hyperplasia can successfully undergo transplantation. 2) Nodular regenerative hyperplasia can present with signs and symptoms of liver failure, is difficult to diagnose by needle biopsy, and can be difficult to discriminate clinically from cirrhosis. 3) Although each case must be individually evaluated transplantation may be the optimal therapy in patients presenting with complications of liver failure.
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PMID:Results of liver transplantation for nodular regenerative hyperplasia. 1109 22

Nodular regenerative hyperplasia of the liver (NRHL) is an uncommon non-malignant finding typically associated with haematological or auto-immune disease. The main clinical symptom is portal hypertension in the absence of underlying liver cirrhosis. The pathogenesis of NRHL remains unknown. We report a case of NRHL with cholestasis and progression to liver insufficiency without any underlying disease and no association with systemic disease or drug intake. Cholestasis and liver function tests improved significantly during treatment with ursodeoxycholic acid (750 mg per day). Based on this case, it may be concluded that treatment with ursodeoxycholic acid might be beneficial in patients with NRHL and progression to liver insufficiency.
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PMID:Nodular regenerative hyperplasia of the liver: a rare differential diagnosis of cholestasis with response to ursodeoxycholic acid. 1266 46

Computed tomography (CT) and Magnetic Resonance Imaging (MRI) have no significant impact in the evaluation of diffuse liver disease. Cirrhosis and hepatitis are not of specific imaging findings, the image of cirrhosis is depending on degree of disease. Nodular lesions are frequent findings in cirrhotic livers. For differentiation of regenerative nodules, dysplastic nodules and hepatocellular carcinoma CT and MRI are playing their role in localization and characterization of these lesions. Sensitivity and specificity are varying, depending on the technical applications of CT and MRI, and the application of contrast materials. MRI is superior in characterizing the lesions due to the different appearance of the lesions in different sequences. CT is superior as the staging modality. Complications of cirrhosis like ascites, varices of the oesophageal veins are diagnosed. CT and MRI are necessary when interventional or surgical procedures are planned or for therapy surveillance.
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PMID:[Role of computed tomography and magnetic resonance imaging in the diagnosis of hepatitis and liver cirrhosis]. 1590 Aug 26

Nodular regenerative hyperplasia (NRH) is characterized by a non-cirrhotic micronodular transformation of the liver parenchyma. It is based on the obliteration of small portal veins. Macroregenerative nodules (MRN) develop in areas of favourable blood flow in otherwise hypoperfused liver tissue. Hypoperfusion is caused by obliteration of liver veins and/or large portal veins with the subsequent atrophy or extinction of parenchyma. The hyperperfused and sometimes rapidly growing MRN might simulate a malignant tumor in CT and MRT. Morphologically, MRN resemble FNH. In contrast to hepatocellular adenoma, they show a more or less nodular architecture with fibrous septa and ductular structures. NRH and cases of MRN without cirrhosis can indicate an extrahepatic/systemic disease causing altered liver perfusion. MRN in liver cirrhosis must be differentiated from dysplastic nodules and highly differentiated hepatocellular carcinoma by cytological and microarchitectural criteria. Focal nodular hyperplasia (FNH) can imitate liver cirrhosis, steatohepatitis, cholangitis or chronic hepatitis, if biopsy material does not include normal perilesional liver tissue. Telangiectatic FNH might resemble classic hepatocellular adenoma. Neoductular structures and septation argue for this rare subtype of FNH. Neoductular transformation of hypoperfused liver parenchyma might imitate cholangioma or cholangiocarcinoma.
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PMID:[Nodular lesions of liver parenchyma caused by pathological vascularisation/perfusion]. 1677 11

Intrahepatic portal venopathy leads to various entities that are important causes of portal hypertension. Noncirrhotic portal fibrosis (NCPF) occurs in the Indian subcontinent, whereas idiopathic portal hypertension (IPH) occurs in Japan although the pathogenesis and presentation of both are similar. NCPF presents mainly with upper gastrointestinal bleeding; IPH presents with massive splenomegaly. The liver functions are preserved. Wedged hepatic venous pressure is normal, but portal venous pressure is high indicating a presinusoidal block. Patients are best managed with endoscopic therapy or surgery, with better results than in patients with cirrhosis. Nodular regenerative hyperplasia is a histological diagnosis characterized by development of nodules in the liver due to uneven perfusion of the portal venous blood. These patients may develop portal hypertension and if they bleed would require treatment as in NCPF/IPH. Schistosomiasis produces portal hypertension by the development of fibrous tissue around the portal veins as a response to schistosome eggs. Gratifying results have been reported with praziquantel therapy. Rarely sarcoidosis and chronic biliary obstruction may also produce portal venopathy.
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PMID:Intrahepatic portal venopathy and related disorders of the liver. 1881 80

Nodular regenerative hyperplasia of the liver, characterised by regenerative nodules distributed throughout the liver in the absence of fibrosis, is a rare but important complication of systemic lupus erythematosus. The main consequence of nodular regenerative hyperplasia of the liver is non-cirrhotic portal hypertension. This condition is probably underdiagnosed, as many of these patients may remain asymptomatic. Furthermore, nodular regenerative hyperplasia of the liver may be misdiagnosed as cirrhosis. We describe three female patients with nodular regenerative hyperplasia of the liver associated with systemic lupus erythematosus. All three patients have clinical manifestations of portal hypertension, and all were initially misdiagnosed as having cryptogenic cirrhosis.
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PMID:Nodular regenerative hyperplasia of the liver associated with systemic lupus erythematosus: three cases. 1934 41

Primary damage to hepatic vessels is rare. (i) Hepatic arterial disorders, related mostly to iatrogenic injury and occasionally to systemic diseases, lead to ischemic cholangiopathy. (ii) Hepatic vein or inferior vena cava thrombosis, causing primary Budd-Chiari syndrome, is related typically to a combination of underlying prothrombotic conditions, particularly myeloproliferative neoplasms, factor V Leiden, and oral contraceptive use. The outcome of Budd-Chiari syndrome has markedly improved with anticoagulation therapy and, when needed, angioplasty, stenting, TIPS, or liver transplantation. (iii) Extrahepatic portal vein thrombosis is related to local causes (advanced cirrhosis, surgery, malignant or inflammatory conditions), or general prothrombotic conditions (mostly myeloproliferative neoplasms or factor II gene mutation), often in combination. Anticoagulation at the early stage prevents thrombus extension and, in 40% of the cases, allows for recanalization. At the late stage, gastrointestinal bleeding related to portal hypertension can be prevented in the same way as in cirrhosis. (iv) Sinusoidal obstruction syndrome (or venoocclusive disease), caused by agents toxic to bone marrow progenitors and to sinusoidal endothelial cells, induces portal hypertension and liver dysfunction. Decreasing the intensity of myeloablative regimens reduces the incidence of sinusoidal toxicity. (v) Obstruction of intrahepatic portal veins (obliterative portal venopathy) can be associated with autoimmune diseases, prothrombotic conditions, or HIV infection. The disease can eventually be complicated with end-stage liver disease. Extrahepatic portal vein obstruction is common. Anticoagulation should be considered. (vi) Nodular regenerative hyperplasia is induced by the uneven perfusion due to obstructed sinusoids, or portal or hepatic venules. It causes pure portal hypertension.
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PMID:Management of hepatic vascular diseases. 2446 42

The incidence of hepatocellular carcinoma (HCC) appears to be increasing across the globe. Well-established protocols for screening are available, and the most common underlying liver problem associated with the development of HCC is cirrhosis. However, with few exceptions, patients without cirrhosis are generally not screened for HCC. Nodular regenerative hyperplasia (NRH) is not associated with the development of significant fibrosis or impaired liver synthetic function. The major clinical impact of NRH appears to be in the development of portal hypertension. Patients with NRH are also not recommended to undergo routine screening for the development of HCC. This report describes a case of a 44-year-old woman with NRH found to have de novo HCC. Emerging evidence suggest a possible pathogenetic relationship between NRH and HCC. The case described here and our review of the published work suggests that additional studies regarding the epidemiological association between NRH and HCC may change the current notion that NRH is not a premalignant lesion, and further studies assessing the utility of routine screening of NRH patients for HCC should be considered.
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PMID:Patients with nodular regenerative hyperplasia should be considered for hepatocellular carcinoma screening. 2360 91

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.
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PMID:Portal Hypertension, Nodular Regenerative Hyperplasia of the Liver, and Obstructive Portal Venopathy due to Metastatic Breast Cancer. 2398 49

Conventional ultrasonogram of the abdomen being noninvasive, inexpensive and ubiquitously available is the first imaging modality that raises suspicion of HCC in a patient with chronic liver disease with or without cirrhosis. The lesions in liver particularly nodule are being recognized with increased frequency with the wide spread use of ultrasonogram as the initial investigation and computerized tomography and magnetic resonance imaging subsequently. Any nodule in a cirrhotic liver should be considered as hepatocellular carcinoma until otherwise proved. This approach certainly is helpful in diagnosing HCC at its earliest possible stage to offer meaningful curative measures be it transplant, resection or ablative therapy. After a nodule is detected on ultrasonogram the next imaging modality can be a contrast enhanced study (dynamic CT scan or an MRI) to see if are present or not. Two vital clues for diagnosis of HCC by contrast enhanced imaging are presence of arterial hypervascularity and washout which are considered as "classical imaging features". This sequence of events of arterial uptake followed by washout is highly specific for diagnosis of HCC by imaging. If the features are typical showing classical imaging features (i.e hypervascular in the arterial phase with washout in portal venous or delayed phase) the lesion should be treated as HCC biopsy is not necessary. Nodular lesions showing an atypical imaging pattern, such as iso- or hypovascular in the arterial phase or arterial hypervascularity alone without portal venous washout, should undergo further examinations with another contrast enhanced imaging. Biopsy is advisable for those lesions which do not show classical features on the imaging.
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PMID:Nodule in Liver: Investigations, Differential Diagnosis and Follow-up. 2575 12


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