Vicente-Santamaría Saioa, Tabares-González Ana, Gascón-Galindo Celia, Tutau-Gómez Carlos, Álvarez-Beltrán Marina, Muñoz-Codoceo Rosa Ana, Rubio-Murillo María, De-Los-Santos Marianela, Salcedo-Lobato Enrique, Masip-Simó Etna, García-Romero Ruth, Fernández-Lorenzo Ana Estefanía, Moreno-Álvarez Ana, Serrano-Nieto Juliana, Hierro-Llanillo Loreto, Loverdos-Eseverri Inés, Crehuá-Gaudiza Elena, Juste-Ruiz Mercedes, Blitz-Castro Enrique, Morales-Tirado Ana, López-Cárdenes Concepción Marina, Bousoño-García Carlos, González-Jiménez David
Cystic Fibrosis Unit, Department of Pediatrics, Ramón y Cajal University Hospital, Madrid.
Department of Medical Sciences, Universidad de Alcalá, Alcalá de Henares.
Eur J Gastroenterol Hepatol. 2025 Apr 1;37(4):505-509. doi: 10.1097/MEG.0000000000002917. Epub 2025 Jan 21.
Cystic fibrosis-related liver disease (CFRLD) is a health problem that can affect as many as 30-40% of cystic fibrosis patients by the age of 12 years. We studied the epidemiology of CFRLD thanks to the first exclusively pediatric CFRLD patient registry to date.
Descriptive cross-sectional study. Information from medical records from January 2018 to December 2020 is collected. CFRLD was classified according to the European Society of Paediatric Gastroenterology, Hepatology and Nutrition 2017 criteria.
Data were collected from 168 pediatric patients diagnosed with CFRLD (90.5% liver involvement without cirrhosis and 8.5% multinodular cirrhosis).
In this national registry, including exclusively pediatric population, liver disease is diagnosed around 7 years of age. Liver involvement without cirrhosis is the most frequent finding among our patients but about 9% of the patients already had cirrhosis. CFRLD is one of the challenges faced by pediatric gastroenterologists in the future and national registries give us the opportunity to further study and broaden our knowledge.
囊性纤维化相关肝病（CFRLD）是一个健康问题，到12岁时，多达30%-40%的囊性纤维化患者可能会受其影响。借助迄今为止首个专门针对儿科CFRLD患者的登记系统，我们对CFRLD的流行病学进行了研究。
描述性横断面研究。收集了2018年1月至2020年12月医疗记录中的信息。CFRLD根据欧洲儿科胃肠病学、肝病学和营养学会2017年标准进行分类。
收集了168例诊断为CFRLD的儿科患者的数据（90.5%为无肝硬化的肝脏受累，8.5%为多结节性肝硬化）。
在这个仅纳入儿科人群的全国性登记系统中，肝病诊断年龄约为7岁。在我们的患者中，无肝硬化的肝脏受累最为常见，但约9%的患者已经出现肝硬化。CFRLD是儿科胃肠病学家未来面临的挑战之一，全国性登记系统让我们有机会进一步研究并拓宽知识面。
