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Invasive Hemodynamic Characterization of the Portal-hypertensive Syndrome in Cirrhotic Rats
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Noncirrhotic portal hypertension.

Harshal Rajekar1, Rakesh K Vasishta2, Yogesh K Chawla3

  • 1Department of General Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh - 160012, India.

Journal of Clinical and Experimental Hepatology
|March 11, 2015
PubMed
Summary

Noncirrhotic portal hypertension (NCPH) presents better prognoses than cirrhosis and is common in Asia. Understanding NCPH vascular lesions is key for managing portal hypertension complications.

Keywords:
ADPKD, autosomal-dominant polycystic kidney diseaseARPKD, autosomal-recessive polycystic kidney diseaseBCS, Budd-Chiari syndromeBudd-Chiari syndromeCHF, congenital hepatic fibrosisCTGF, connective tissue growth factorDSRS, distal splenorenal ShuntEHPVO, extrahepatic portal vein obstructionERCP, endoscopic retrograde cholangio pancreatographyEST, endoscopic sclerotherapyEVL, endoscopic variceal ligationFHF, fulminant hepatic failureGI, GastrointestinalGVHD, graft versus cells host diseaseHLA, human lymphocyte antigenHVPG, hepatic vanous pressure gradientIPH, idiopathic portal hypertensionIVC, inferior vena cavaMRCP, magnetic resonance cholangio pancreatographyNCPF, noncirrhotic portal hypertensionNCPH, noncirrhotic portal hypertensionNRH, nodular regenerative hyperplasiaPVT, portal vein thrombosisSCT, stem-cell transplantationTIPS, transjugular intrahepatic portosystemic shunt placementTIPSS, transjugular intrahepatic portosystemic shuntVOD, veno-occlusive diseasecongenital hepatic fibrosisextra-hepatic portal venous obstructionnodular regenerative hyperplasianoncirrhotic intrahepatic portal hypertensionportal vein thrombosisportosystemic shuntingschistosomiasisveno-occlusive disease

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Area of Science:

  • Hepatology
  • Vascular Medicine
  • Gastroenterology

Background:

  • Portal hypertension, elevated portal pressure (>10 mmHg), often stems from liver cirrhosis.
  • Noncirrhotic portal hypertension (NCPH) is a distinct condition without cirrhosis, prevalent in developing nations, particularly Asia.
  • NCPH carries a significantly better prognosis compared to cirrhotic portal hypertension.

Purpose of the Study:

  • To elucidate the heterogeneous nature of noncirrhotic portal hypertension (NCPH).
  • To classify NCPH based on vascular lesion locations and types.
  • To outline the clinical manifestations and management strategies for NCPH.

Main Methods:

  • Review of intrahepatic and extrahepatic etiologies of NCPH.
  • Classification of vascular lesions: endothelial damage, intimal thickening, thrombotic obliterations, scarring.
  • Analysis of clinical presentations and sequelae of portal hypertension in NCPH patients.

Main Results:

  • NCPH involves diverse vascular lesions affecting portal or hepatic venous circulation.
  • Clinical manifestations include gastrointestinal bleeding, splenomegaly, ascites, hypersplenism, growth retardation, and jaundice.
  • Sequelae encompass hyperdynamic circulation, pulmonary issues, and portosystemic encephalopathy.

Conclusions:

  • NCPH is a heterogeneous vascular disorder with varied etiologies and lesion types.
  • Effective management focuses on reducing portal pressure and treating complications.
  • Pharmacologic and endoscopic therapies are primary treatment modalities for portal hypertension.