Liver transplant in children and adults with cystic fibrosis: Impact of growth failure and nutritional status. Issue 1 (2nd September 2021)

• Record Type: Journal Article
• Authors: Cheng, Katherine; Rosenthal, Philip; Roberts, John P.; Perito, Emily R.
• Is Part Of: American journal of transplantation. Volume 22:Issue 1(2022)
• Page Start: 177
• Page End: 186
• Publication Date: 2021-09-02
• Subjects: guidelines -- liver disease -- organ allocation -- patient survival -- united network for organ sharing (UNOS); Transplantation of organs, tissues, etc -- Periodicals; 617.95
• DOI: 10.1111/ajt.16791
• Languages: English
• ISSNs: 1600-6135

Abstract

Abstract : Nutritional status is known to strongly predict health outcomes in people with cystic fibrosis (CF), but the prevalence of and consequences for CF children with growth failure and underweight CF adults on the liver transplant (LT) waitlist has not been delineated. We utilized UNOS registry data from 2003 to 2017 to investigate the impact of growth failure and underweight on outcomes in liver transplant candidates with CF. Almost 1 in 3 children and adults with CF had growth failure or were underweight, respectively, at listing. Body mass index under‐estimated growth failure compared to height and weight z‐scores for children. In multivariate analysis of children, growth failure (HR 2.1, 95% CI 1.7–2.6), and CF (HR 2.7, 95% CI 1.6–4.6, vs. Non‐CF) were independent risk factors for waitlist death among children; having both increased death risk almost fourfold (SHR 3.88, 95% CI 1.42–10.58). However, among children who did receive a LT, CF was not associated with death within 1‐year post‐LT. Underweight adult CF candidates were less likely to receive LT, again suggesting the importance of nutritional evaluation and early intervention in this at‐risk cohort. Abstract : While liver transplant can potentially be life‐saving for children and adults with cystic fibrosis, growth failure increases the risk of waitlist and post‐transplant death.