Spectrum of Idiopathic Noncirrhotic Portal Hypertension

Dhiman, Radha K.

doi:10.1016/s0973-6883(11)60122-9

Cited by 5 publications

(4 citation statements)

References 12 publications

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“…It can be surmised that the widely reported reduction of incidence of NCIPH in India in recent years, 80 is to some extent related to the reduction seen in the incidence of chronic enteropathies such as tropical sprue. In a Caucasian population, Eapen et al reported a significantly worse prognosis of NCIPH in those with untreated adult coeliac disease.…”

Section: Treatmentmentioning

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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Section: Treatmentmentioning

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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“…Incidence of NCPF is decreasing with NCPF now being responsible for only 5.6% of cases of portal hypertension compared to 23.3% in the 1980s. 50 On histology NCPF is diagnosed based on demonstration of obliterative portal venopathy involving obliteration of small branches of portal veins, predominantly mediumsized and preterminal veins (0.2-3 mm in diameter). 51 Development of ascites, jaundice, and hepatic encephalopathy is uncommon in NCPF and the prognosis is usually good.…”

Section: Correct Answers: 2 Andmentioning

confidence: 99%

Hepatobiliary Quiz 11 (2014)

Agrawal

Dhiman

2014

Journal of Clinical and Experimental Hepatology

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“…However, its incidence has considerably decreased over time and accounts for only 5.6% of all cases of portal hypertension now. 66 The histological hallmark of NCPF termed obliterative portal venopathy includes sclerosis and obliteration of small branches of portal veins, predominantly medium-sized and preterminal veins (0.2-3 mm in diameter). 67 Patients with NCPF commonly present with one or more well-tolerated episodes of gastrointestinal hemorrhage, a long-standing mass in the left upper quadrant (splenomegaly), or consequences of hypersplenism.…”

Section: Correct Answers: 3 Andmentioning

confidence: 99%