P311 Clinical features of cystic fibrosis patients with chronic liver disease in the Turkish National Cystic Fibrosis Registry
Conclusions: Using modified serum lab thresholds, addition of liver fibrosis indices, and/or abdominal US can increase detection of liver nodularity in CF. A combination of GGT, GPR, and APRI can help direct which CF children should undergo US evaluation. These tools may improve earlier identification of fibrosis and/or cirrhosis in CF patients.
No abstract available
Conclusions: Liver US patterns in children with CF correlate with platelet count, spleen size and indices of liver fibrosis. Multivariable models of these biomarkers have excellent discriminating ability for NL versus NOD, and good ability to distinguish other US patterns, suggesting that US patterns correlate with clinically relevant liver disease.
This study investigated the potential of Supersonic shear-wave elastography (SSWE) to non-invasively detect CFLD and assess hepatic fibrosis severity in children with CF.
Learning point for cliniciansThis case illustrates the limitations of using non-invasive criteria for the diagnosis of hepatocellular carcinoma. The criteria may not be valid in rarer cause of cirrhosis such as cystic fibrosis. If the diagnosis precludes life-saving treatment such as liver transplantation a confirmatory liver biopsy may be wise.
Nature Reviews Gastroenterology &Hepatology, Published online: 05 June 2019; doi:10.1038/s41575-019-0156-4New knowledge has been steadily acquired in genetic and congenital cholangiopathies. Here, the authors discuss Alagille syndrome, polycystic liver diseases, fibropolycystic liver diseases and cystic fibrosis-related liver disease and the insights that these conditions provide into the mechanisms of acquired cholangiopathies.