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Inicio Medicina Clínica (English Edition) Portosinusoidal vascular disorder: A paradigm shift
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Vol. 162. Issue 9.
Pages 439-447 (May 2024)
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Vol. 162. Issue 9.
Pages 439-447 (May 2024)
Review
Portosinusoidal vascular disorder: A paradigm shift
Enfermedad vascular portosinusoidal: un cambio de paradigma
Luis Télleza,b,c,d,
Corresponding author
luis.tellez@salud.madrid.org

Corresponding author.
, Jesús Donatea, Agustín Albillosa,b,c,d
a Servicio de Gastroenterología y Hepatología, Hospital Universitario Ramón y Cajal, Madrid, Spain
b Instituto Ramón y Cajal de Investigación Sanitaria (IRYCIS), Madrid, Spain
c Centro de Investigación en Red Enfermedades Hepáticas y Digestivas (CIBERehd), Madrid, Spain
d Universidad de Alcalá, Madrid, Spain
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Table 1. Histological findings of porto-sinusoidal vascular disease.
Abstract

The term portosinusoidal vascular disorder (PSVD) refers to a clinical–pathological entity that encompasses those patients with intrahepatic vascular damage without cirrhosis at risk of developing severe complications of portal hypertension. Numerous systemic diseases, genetic disorders, and toxic agents have been associated with this pathology, making its diagnosis an important clinical challenge. The recent description of uniform diagnostic criteria and a better understanding of its pathophysiology will allow for better identification of patients, even in early stages of the disease. Although there is currently no effective etiological treatment available, early diagnosis allows for the development of preventive strategies for some severe complications of portal hypertension.

Keywords:
Liver
Portosinusoidal vascular disorder
Portal hypertension
Idiopathic portal hypertension
Cirrhosis
Esophageal várices
Nodular focal hyperplasia
Ascites
Portal vein thrombosis
Liver transplantation
Resumen

El término de enfermedad vascular portosinusoidal (EVPS) hace referencia a una entidad clínico-patológica que engloba a aquellos pacientes con daño vascular intrahepático sin cirrosis en riesgo de desarrollar complicaciones graves de la hipertensión portal. Numerosas enfermedades sistémicas, alteraciones genéticas y agentes tóxicos se han relacionado con este trastorno, lo que hace que su diagnóstico sea un reto clínico importante. La reciente descripción de unos criterios diagnósticos uniformes y el mejor conocimiento de su fisiopatología permitirá una óptima identificación de los pacientes, incluso en fases precoces de la enfermedad. Aunque a día de hoy no se dispone de un tratamiento etiológico eficaz, el diagnóstico temprano permite establecer estrategias preventivas sobre algunas complicaciones graves de la hipertensión portal.

Palabras clave:
Hígado
Enfermedad vascular portosinusoidal
Hipertensión portal
Hipertensión portal idiopática
Cirrosis
Várices esofágicas
Hiperplasia nodular focal
Ascitis
Trombosis venosa portal
Trasplante hepático

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