Sakhuja et al reported predictors of rapid progression of fibrosis in children with cystic fibrosis-related liver disease. The authors are from Texas Children's Hospital, Baylor College of Medicine, Prisma Health Children’s Hospital Midlands, QIMR Berghofer Medical Research Institute, Queensland Children's Hospital, and University of Queensland.
Patient selection: child with cystic fibrosis-related liver disease
Outcome: time to F3-F4
Predictors of rapid progression of liver fibrosis:
(1) FEV1 as percent of predicted < 64%
(2) GGT to platelet ratio (GPR) >= 0.09, especially if >= 0.41
(3) serum GGT >= 25.5 U/L
(4) FIB-4 >= 0.13, especially if >= 0.55
(5) APRI >= 0.36, especially if >= 0.78
(6) isolation of Pseudomonas aeruginosa from a respiratory culture prior to 2 years of age
(7) F508del heterozygous genotype
(8) rapid decrease in platelet count (even if within normal range)
Factors associated with slower progression:
(1) pancreatic insufficiency
(2) normal abdominal ultrasonography
