Department of Pediatrics, Emory University School of Medicine, Atlanta, GA.
Division of Pediatric Gastroenterology, Hepatology and Nutrition, Children's Healthcare of Atlanta, Atlanta, GA.
Liver Transpl. 2019 Apr;25(4):640-657. doi: 10.1002/lt.25421. Epub 2019 Mar 20.
Approximately 5%-10% of patients with cystic fibrosis (CF) will develop advanced liver disease with portal hypertension, representing the third leading cause of death among patients with CF. Cystic fibrosis with advanced liver disease and portal hypertension (CFLD) represents the most significant risk to patient mortality, second only to pulmonary or lung transplant complications in patients with CF. Currently, there is no medical therapy to treat or reverse CFLD. Liver transplantation (LT) in patients with CFLD with portal hypertension confers a significant survival advantage over those who do not receive LT, although the timing in which to optimize this benefit is unclear. Despite the value and efficacy of LT in selected patients with CFLD, established clinical criteria outlining indications and timing for LT as well as disease-specific transplant considerations are notably absent. The goal of this comprehensive and multidisciplinary report is to present recommendations on the unique CF-specific pre- and post-LT management issues clinicians should consider and will face.
约 5%-10%的囊性纤维化 (CF) 患者会发展为伴有门静脉高压的晚期肝病，这是 CF 患者的第三大死亡原因。伴有晚期肝病和门静脉高压的囊性纤维化 (CFLD) 是患者死亡的最大风险因素，仅次于 CF 患者的肺部或肺部移植并发症。目前，尚无治疗或逆转 CFLD 的医学疗法。对于伴有门静脉高压的 CFLD 患者，肝移植 (LT) 可显著提高生存率，优于未接受 LT 的患者，尽管尚不清楚何时能最大程度地发挥这一益处。尽管 LT 在选定的 CFLD 患者中有价值且有效，但目前尚缺乏明确的临床标准来概述 LT 的适应证和时机以及特定于疾病的移植考虑因素。本全面和多学科报告的目标是就临床医生应考虑并将面临的独特的 CF 特定的 LT 前后管理问题提出建议。
