Nodular regenerative hyperplasia: The main liver disease in patients with primary hypogammaglobulinemia and hepatic abnormalities

Malamut, Georgia; Ziol, Marianne; Suarez, Félipe; Beaugrand, Michel; Viallard, J.F.; Lascaux, Anne Sophie; Verkarre, Virginie; Béchade, Dominique; Poynard, Thierry; Hermine, Olivier; Cellier, Christophe

doi:10.1016/j.jhep.2007.08.011

Cited by 163 publications

(131 citation statements)

References 28 publications

(39 reference statements)

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“…35,36 Earlier studies emphasized the importance of common variable immunodeficiency (CVID). 37,38 Treatment with intravenous immunoglobulin replacement has largely resolved the issue, further supporting the notion that enteropathies associated with CVID were somehow promoting the vasculopathy of terminal portal venous radicles. Such a sequence points strongly to a gastrointestinal source for the initiating factors in NCIPH.…”

Section: Associated Disorders Gut and Immune Disordersmentioning

confidence: 89%

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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Section: Associated Disorders Gut and Immune Disordersmentioning

confidence: 89%

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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“…Hepatitis with no evidence of viral infection was reported in 43% of patients in two unrelated cohorts (16,17), and raised blood alkaline phosphatase levels were often observed. In most cases, liver biopsy showed mild to moderate periportal lymphocyte infiltration and cholestasis.…”

Section: Cvid and Inflammationmentioning

confidence: 99%

“…Gastrointestinal tract involvement is present in up to 50% of CVID patients (16). Some cases of transient or persistent diarrhea or malabsorption are due to bacterial, parasitic or viral infection (5).…”

Section: Cvid and Inflammationmentioning

confidence: 99%

Common variable immunodeficiency: Crossroads between infections, inflammation and autoimmunity

Baldovino

Montin

Martino

et al. 2013

Autoimmunity Reviews

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“…Up to 10% of CVID patients may suffer from liver dysfunctions (abnormal Review liver function tests, predominantly increased alkaline phosphatase and nodular regenerative hyperplasia) caused by infections (e.g., HBV and HCV), autoimmunity (e.g., autoimmune hepatitis, primary biliary cirrhosis) and granulomatous diseases [84,160,167]. Previous studies suggested that nodular regenerative hyperplasia has different etiology, including circulating immune complexes or light chain deposits in the sinusoidal walls [168]. Unexplained liver disease with portal hypertension occurs in 5-10% of the patients.…”

Section: Management Of Gi Complications and Enteropathymentioning

confidence: 99%

A review on guidelines for management and treatment of common variable immunodeficiency

Abolhassani

Sagvand

Shokuhfar

et al. 2013

Expert Review of Clinical Immunology

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Nodular regenerative hyperplasia: The main liver disease in patients with primary hypogammaglobulinemia and hepatic abnormalities

Cited by 163 publications

References 28 publications

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

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

Common variable immunodeficiency: Crossroads between infections, inflammation and autoimmunity

A review on guidelines for management and treatment of common variable immunodeficiency

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