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[Portosinusoidal vascular disorder: A paradigm shift].

Luis Téllez1, Jesús Donate2, Agustín Albillos1

  • 1Servicio de Gastroenterología y Hepatología. Hospital Universitario Ramón y Cajal, Madrid, España; Instituto Ramón y Cajal de Investigación Sanitaria (IRYCIS), Madrid, España; Centro de Investigación en Red Enfermedades Hepáticas y Digestivas (CIBERehd), Madrid, España; Universidad de Alcalá, Madrid, España.

Medicina Clinica
|February 1, 2024
PubMed
Summary

Portosinusoidal vascular disorder (PSVD) involves liver vascular damage without cirrhosis, posing risks for portal hypertension complications. Early diagnosis is crucial for managing risks, despite no current cure.

Keywords:
AscitesAscitisCirrhosisCirrosisEnfermedad vascular portosinusoidalEsophageal váricesHiperplasia nodular focalHipertensión portalHipertensión portal idiopáticaHígadoIdiopathic portal hypertensionLiverLiver transplantationNodular focal hyperplasiaPortal hypertensionPortal vein thrombosisPortosinusoidal vascular disorderTrasplante hepáticoTrombosis venosa portalVárices esofágicas

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Area of Science:

  • Hepatology
  • Vascular Pathology
  • Internal Medicine

Background:

  • Portosinusoidal vascular disorder (PSVD) is a condition characterized by intrahepatic vascular damage.
  • Patients with PSVD do not have cirrhosis but are at risk of severe portal hypertension complications.
  • Various systemic diseases, genetic factors, and toxins are linked to PSVD, complicating diagnosis.

Purpose of the Study:

  • To highlight the diagnostic challenges of PSVD.
  • To emphasize the importance of recent advancements in diagnostic criteria and understanding pathophysiology.
  • To underscore the significance of early PSVD identification for managing portal hypertension.

Main Methods:

  • Review of clinical-pathological entity of PSVD.
  • Analysis of associated systemic diseases, genetic disorders, and toxic agents.
  • Evaluation of recent diagnostic criteria and pathophysiological insights.

Main Results:

  • PSVD diagnosis is challenging due to diverse etiologies.
  • Uniform diagnostic criteria and improved pathophysiology understanding aid early detection.
  • Early identification enables preventive strategies against portal hypertension complications.

Conclusions:

  • PSVD requires careful diagnosis due to its varied causes and potential severe outcomes.
  • Advancements in diagnostic criteria and pathophysiology knowledge improve early patient identification.
  • While etiological treatments are lacking, early diagnosis facilitates proactive management of portal hypertension risks.