Spanish clinical practice guidelines for the diagnosis and management of cholestatic liver diseases in adult and pediatric population: Joint report from AEEH and SEGHNP.

Cholestatic liver diseases comprise a heterogeneous group of disorders affecting both adult and pediatric population, characterized by alterations in bile formation, secretion, or flow, leading to the accumulation of bile acids and other toxic substances in the liver. In recent years, advances in ne...

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Detalles Bibliográficos
Autores: Díaz-González Á, Molera C, Gómez-Domínguez E, Muñoz-Bartolo G, Ampuero J, Fernández-Rodríguez C, Quintero J, Molina E, Salcedo M, Olveira A, Morillas R, Díez MA, Casado M, García-Buey L, Rodríguez-Tajes S, Jorquera F, Berenguer M, Montero JL, Londoño MC
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2026
País:España
Institución:Fundació Sant Joan de Déu
Repositorio:r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
OAI Identifier:oai:dnet:r-fsjd______::aa235e7a258041e3d5df21c4fd6bc50c
Acceso en línea:https://fsjd.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=29432
Access Level:acceso abierto
Palabra clave:Cholestasis
Colangitis biliar primaria
Colangitis esclerosante primaria
Colestasis
Colestasis genética
Genetic cholestasis
Guidelines
Guías
Primary biliary cholangitis
Primary sclerosing cholangitis
Descripción
Sumario:Cholestatic liver diseases comprise a heterogeneous group of disorders affecting both adult and pediatric population, characterized by alterations in bile formation, secretion, or flow, leading to the accumulation of bile acids and other toxic substances in the liver. In recent years, advances in new pharmacological therapies, the availability of next-generation genetic sequencing techniques, and the development of specific treatments for genetic cholestasis have transformed the diagnostic and therapeutic approach to these conditions. This document, jointly prepared by the "Asociación Española para el Estudio del Hígado" (AEEH) and the "Sociedad Española de Gastroenterología, Hepatología y Nutrición Pediátrica" (SEGHNP), presents a national evidence-based guideline for the diagnosis and management of hepatic cholestasis in Spain. It addresses recommendations for differential diagnosis, diagnostic algorithms, indications for genetic studies, treatment and follow-up criteria in diseases such as primary biliary cholangitis, primary sclerosing cholangitis, genetic cholestasis, intrahepatic cholestasis of pregnancy, and vanishing bile duct syndrome. In addition, recommendations are included for the management of extrahepatic complications, indications for liver transplantation, and special considerations in pregnancy and childhood. The guideline emphasizes the importance of a multidisciplinary approach, the use of non-invasive tools for risk stratification, and the incorporation of new targeted therapies, with the aim of improving the prognosis and quality of life of patients affected by cholestatic liver diseases.