A recent multicenter case-controlled study (MJ Siegel, J Freeman, W Ye et al. J Pediatr 2020; 219: 62-9) show that ultrasonography can identify children with cystic fibrosis (CF) at increased risk for developing advanced CF liver disease; it is well-recognized that currently advanced CF liver disease occurs predominantly in children.

Among 722 participants who underwent ultrasonography as part of a planned 9 year research study, 65 had heterogeneous liver pattern and 592 had a normal liver pattern at baseline.

Key findings at planned 4-year interim analysis in a subgroup of 55 with (baseline) heterogeneous liver pattern and 116 with (baseline) normal liver pattern:

• Those with heterogeneous liver pattern had higher median biochemical markers of liver disease: ALT 42 vs 32, GGT 36 vs 15, AST to platelet ratio index: 0.7 vs 0.4
• Those with heterogeneous liver pattern were at ~9-fold increased risk of developing a nodular liver pattern (23% vs. 2.6%).
• The authors did not identify specific clinical risk factors that predict the development of nodular liver ultrasound pattern

My take: Even among those with a heterogeneous pattern, the majority will not develop a ultrasonography (nodular) pattern of advanced liver disease.  Much more insight in this area is needed with the advancement in medical treatments and with the more availability of elastography.

Related blog posts:

• Outcomes and Risk Factors in Cystic Fibrosis Liver Disease
• Big Advance in Cystic Fibrosis -Who Will Benefit?
• Ursodeoxycholic Acid, Cystic Fibrosis, and the Problem with Surrogate Markers
• Characterizing Severe Liver Disease with Cystic Fibrosis
• Liver Shorts April 2019

Island Ford Nat’l Recreation Area/Chattahoochee River