Spanish clinical practice guidelines for the diagnosis and management of cholestatic liver diseases in adult and pediatric population: Joint report from AEEH and SEGHNP
Por:
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 and Londoño MC
Publicada:
1 abr 2026
Ahead of Print:
1 abr 2026
Resumen:
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 "Asociaci & oacute;n Espanola para el Estudio del Higado" (AEEH) and the "Sociedad Espanola de Gastroenterologia, Hepatologia y Nutrici & oacute;n Pedi & aacute;trica" (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. (c) 2025 The Author(s). Published by Elsevier Espana, S.L.U. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).
Filiaciones:
Díaz-González Á:
Gastroenterology and Hepatology Department, Clinical and Translational Research in Digestive Diseases Group, Valdecilla Research Institute (IDIVAL), Marqués de Valdecilla University Hospital, Santander, Spain
Molera C:
Pediatric Gastroenterology, Hepatology and Nutrition Department, Hospital Sant Joan de Déu, Universitat de Barcelona, Spain
Gómez-Domínguez E:
Digestive Diseases Department, Hospital Universitario 12 de Octubre, Madrid, Spain
Muñoz-Bartolo G:
Department of Paediatric Hepatology, Instituto de Investigación Hospital Universitario La Paz (IdiPAZ), La Paz Children's University Hospital, Madrid, Spain
Ampuero J:
Digestive Diseases Department, Hospital Universitario Virgen del Rocío, Sevilla, Spain
Instituto de Biomedicina de Sevilla, Spain
CIBEREHD, Madrid, Spain
Universidad de Sevilla, Sevilla, Spain
Fernández-Rodríguez C:
Digestive Diseases Department, Hospital Universitario Fundación Alcorcón, University Rey Juan Carlos, Madrid, Spain
Quintero J:
Pediatric Hepatology and Liver Transplant Unit, Hospital Universitari Vall d'Hebron, Barcelona, Spain
Molina E:
Digestive Diseases Department, Complexo Hospitalario Universitario de Santiago, Santiago, Spain
Salcedo M:
Digestive Diseases Department. Hospital General Universitario Gregorio Marañón, Madrid, Spain
Olveira A:
Gastroenterology Department, Hospital Universitario La Paz, Madrid, Spain
Morillas R:
Hepatology Unit, Hospital Universitari Germans Trias i Pujol, Centro de investigación biomédica en red Enfermedades Hepáticas y Digestivas (CIBEREHD), Barcelona, Spain
Díez MA:
Department of Paediatric Hepatology, Instituto de Investigación Hospital Universitario La Paz (IdiPAZ), La Paz Children's University Hospital, Madrid, Spain
Casado M:
Gastroenterology department Torrecárdenas Hospital, Almeria, Spain
García-Buey L:
Digestive Diseases Department, Hospital Universitario de la Princesa, Instituto de Investigación Sanitaria Princesa (IIS-IP), Universidad Autónoma de Madrid, Madrid, Spain
Rodríguez-Tajes S:
Liver Unit, Hospital Clínic Barcelona, Fundació de Recerca Clínic Barcelona-Institut d'Investigacions Biomèdiques August Pi i Sunyer (FRCB-IDIBAPS), Universitat de Barcelona, Centro de investigación biomédica en red Enfermedades Hepáticas y Digestivas (CIBEREHD), Barcelona, Spain
Jorquera F:
Gastroenterology and Hepatology, Complejo Asistencial Universitario de León, Spain
Berenguer M:
Liver Transplantation and Hepatology Unit, Hospital Universitari i Politècnic La Fe, IISLaFe, Universidad de Valencia, Centro de investigación biomédica en red Enfermedades Hepáticas y Digestivas (CIBEREHD), Valencia, Spain
Montero JL:
UGC Ap. Digestivo. Unidad de Hepatología y Trasplante Hepático. Hospital Universitario Reina Sofía, Córdoba, Spain
Londoño MC:
Liver Unit, Hospital Clínic Barcelona, Fundació de Recerca Clínic Barcelona-Institut d'Investigacions Biomèdiques August Pi i Sunyer (FRCB-IDIBAPS), Universitat de Barcelona, Centro de investigación biomédica en red Enfermedades Hepáticas y Digestivas (CIBEREHD), Barcelona, Spain
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