https://www.ncbi.nlm.nih.gov/pubmed/30234762 Cystic fibrosis
J Pediatr Gastroenterol Nutr. 2018 Sep 18. doi: 10.1097/MPG.0000000000002154. [Epub ahead of print]
Hepatic Steatosis is Prevalent following Orthotopic Liver Transplantation in Children with Cystic Fibrosis.
Cortes-Santiago N1, Leung DH, Castro E, Finegold M, Wu H, Patel K.

Abstract

OBJECTIVES:
Systematic study of allograft liver histology in children undergoing orthotopic liver transplantation (LT) for cystic fibrosis-related liver disease (CFLD).

METHODS:
Retrospective clinicopathologic review of explants and allograft liver biopsies from 13 children and adolescents with CFLD.

RESULTS:
In this study, the median age at LT for CFLD was 15.7 years. Notably, 10 of 13 (77%) CF explants had >5% steatosis and 8 of 13 (61.5%) demonstrated variable fibrosis. The median age, gender, type of transplant (liver vs liver-lung), pancreatic insufficiency (PI) status, BMI%ile, genotype and prevalence of diabetes were comparable in those with and without explant steatosis. More than half of allograft biopsies showed significant steatosis (17/31, 54.8%) and lobular inflammation (16/31, 51.6%). Hepatocyte ballooning was less frequent (5/31, 16.1%). Overall, 6 patients (46.2%) had allograft steatosis that worsened over time in 2 patients (33%). None had advanced fibrosis (≥stage 3). Patients with allograft steatosis had significantly more biopsies, were more likely to be 'liver only' recipients, had a shorter interval since transplant and higher BMI%ile (though <85). Patients without explant steatosis never demonstrated allograft steatosis, while 60% of patients with explant steatosis (n = 6) developed varying degrees of allograft steatosis. The degree of explant steatosis did not predict its severity in allografts (p = 0.3).

CONCLUSION:
This is the first study highlighting the development of allograft steatosis in CF patients. Our findings suggest that allograft steatosis in patients with CF may be related to pre-existing steatosis in native livers, regardless of other risk factors and may have implications on patient management and long-term graft/patient survival.