Nodular regenerative hyperplasia of the liver associated with primary pulmonary hypertension

Yutani, Chikao; Imakita, Masami; Ishibashi‐Ueda, Hatsue; Okubo, Shumpei; Naito, Makoto; Kunieda, Takeyoshi

doi:10.1016/s0046-8177(88)80180-1

Cited by 32 publications

(7 citation statements)

References 17 publications

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“…[14][15][16][17][18][19][20][21] In our series, the incidence of PPH was 3%. This percentage is similar to that reported in the study by Hadengue et al, 5 but it is higher than a previous estimate.…”

Section: Discussionmentioning

confidence: 94%

Portopulmonary hypertension: distinctive hemodynamic and clinical manifestations

Yang

Lin

Lee

et al. 2001

Journal of Gastroenterology

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Portopulmonary hypertension is now recognized as one of the pulmonary complications of chronic liver disease. However, previous studies reported that the incidence ranged from 0.25% to 2%, excluding fortuitous coincidence. In this study, we aimed to determine the variant hemodynamic and clinical features of portopulmonary hypertension in an area with a high prevalence of viral cirrhosis. After reviewing the hemodynamic data of 322 patients with portal hypertension admitted to the Taipei Veterans General Hospital between 1987 and 1999, we found 10 with portopulmonary hypertension. The overall incidence was, therefore, 3.1% in all patients with portal hypertension. Most of the patients with portopulmonary hypertension experienced exertional dyspnea. The survival times ranged from 2 to 86 months. In our series, most of the patients who died, died of complications related to cirrhosis and portal hypertension, but not of complications related to pulmonary hypertension. This study suggested that portopulmonary hypertension was not a frequent complication in cirrhotic patients and was not associated with an adverse outcome.

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Section: Discussionmentioning

confidence: 94%

Portopulmonary hypertension: distinctive hemodynamic and clinical manifestations

Yang

Lin

Lee

et al. 2001

Journal of Gastroenterology

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“…Laboratory testing for diagnosis of other known causes of liver disease is unyielding. Both, hepato-pulmonary syndrome 21 and porto-pulmonary hypertension, 34,68 may arise as complications of NCIPH.…”

Section: Clinical Examinationmentioning

confidence: 99%

Idiopathic Non-Cirrhotic Intrahepatic Portal Hypertension (NCIPH)—Newer Insights into Pathogenesis and Emerging Newer Treatment Options

Goel

Elias

Eapen

et al. 2014

Journal of Clinical and Experimental Hepatology

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Chronic microangiopathy of portal venules results in idiopathic non-cirrhotic intrahepatic portal hypertension (NCIPH). Recent data suggest a role for vasoactive factors of portal venous origin in the pathogenesis of this 'pure' vasculopathy of the liver. Enteropathies (often silent), are an important 'driver' of this disease. NCIPH is under-recognized and often mis-labeled as cryptogenic cirrhosis. Liver biopsy is needed to prove the diagnosis of NCIPH. In these patients, with advancing disease and increased porto-systemic shunting, the portal venous vasoactive factors bypass the liver filter and contribute to the development of pulmonary vascular endothelial disorders-porto-pulmonary hypertension and hepato-pulmonary syndrome as well as mesangiocapillary glomerulonephritis. Prognosis in NCIPH patients is determined by presence, recognition and management of associated disorders. With better understanding of the pathogenesis of NCIPH, newer treatment options are being explored. Imbalance in ADAMTS 13 (a disintegrin and metalloprotease with thrombospondin type 1 motif, member 13): vWF (von-Willebrand factor) ratio is documented in NCIPH patients and may have a pathogenic role. Therapeutic interventions to correct this imbalance may prove to be important in the management of NCIPH. ( J CLIN EXP HEPATOL 2014;4:247-256) T he term "portal hypertension" is used to reflect a clinical condition secondary to increased pressure in the hepatic portal circulation. Clinically, it usually presents with symptoms or signs of gastroesophageal varices, ascites, encephalopathy, splenomegaly and/or hypersplenism. Portal hypertension can be classified in terms of the anatomic location of the causal resistance to portal blood flow as pre-hepatic (e.g. portal vein thrombosis), post-hepatic (e.g. Budd-Chiari syndrome) and intrahepatic. Intrahepatic portal hypertension can be further sub-classified as pre-sinusoidal (e.g. congenital hepatic fibrosis) or sinusoidal (sinusoidal obstruction syndrome/ veno-occlusive disease). This review focuses on idiopathic non-cirrhotic intrahepatic portal hypertension (NCIPH), a cause of pre-sinusoidal intrahepatic portal hypertension.NCIPH occurs as a consequence of increased resistance to flow within intrahepatic portal vein radicles. Based on vascular corrosion cast and morphometric studies, the site of narrowing or occlusion was localized to the 3rd/4th order portal vein radicles. Boyer 1 and Wanless 2 proposed that the pruning of portal vein radicles is a consequence of thrombosis, but this view is not universally accepted. Sarin et al 3 have shown that there are two independent pressure gradients, one between intra-splenic and intrahepatic pressure and another between intrahepatic and wedged hepatic venous pressure indicating the likelihood of both presinusoidal and peri-sinusoidal resistance to blood flow. Intra-variceal pressure is representative of portal pressure. 3 NOMENCLATUREVarious terminologies have been used in an attempt to define and characterize NCIPH-idiopathic portal hypertensio...

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“…This condition is associated with various diseases such as Budd-Chiari syndrome, myeloproliferative syndromes, lymphoproliferative syndromes, and collagen vascular disorders, and with drugs such as immunosuppressive or antineoplastic medications (43). The pathogenesis of the nodules has not yet been determined; however, increased resistance to sinusoid hepatic blood flow (with resultant portal hypertension) and a decrease in portal venous inflow or hepatic venous outflow (as seen in right-sided heart failure, passive hepatic congestion, and pulmonary hypertension) have been cited as possible factors (44,45). In Budd-Chiari syndrome, progressive thrombotic occlusion of the hepatic vein branches and chronic hepatic sinusoidal congestion lead to prolonged exposure of hepatocytes to blood-borne hepatopoietins, thus stimulating nodular hepatocellular regeneration (46).…”

Section: Nodular Regenerative Hyperplasiamentioning

confidence: 99%

MR Imaging of Hypervascular Liver Masses: A Review of Current Techniques

Silva

Evans

McCullough³

et al. 2009

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Major technologic advances in magnetic resonance (MR) imaging, including the advent of novel pulse sequences (eg, diffusion-weighted and steady-state free precession sequences) and the use of hepatocyte-specific contrast agents, have led to better image quality and shorter acquisition times, resulting in dramatic improvements in the noninvasive detection and characterization of hepatic lesions, particularly hypervascular neoplasms. However, as the role of MR imaging in clinical evaluation of the liver continues to evolve, keeping abreast of new developments can be daunting as well as confusing. A systematic approach that makes use of a simple decision algorithm can help differentiate hypervascular hepatic lesions on the basis of their distinguishing MR imaging characteristics and related clinical information.

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Nodular regenerative hyperplasia of the liver associated with primary pulmonary hypertension

Cited by 32 publications

References 17 publications

Portopulmonary hypertension: distinctive hemodynamic and clinical manifestations

Portopulmonary hypertension: distinctive hemodynamic and clinical manifestations

Idiopathic Non-Cirrhotic Intrahepatic Portal Hypertension (NCIPH)—Newer Insights into Pathogenesis and Emerging Newer Treatment Options

MR Imaging of Hypervascular Liver Masses: A Review of Current Techniques

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