Assessment of Liver Disease Progression in cystic Fibrosis Using Transient Elastography

2017 Journal of Pediatric Gastroenterology and Nutritio In Press;

OBJECTIVES: Cystic fibrosis related liver disease (CFLD) can develop silently in early life and approximately 10% of children with cystic fibrosis (CF) become cirrhotic before adulthood. Clinical, biological and ultrasound criteria used to define CFLD often reveal liver involvement at an advanced stage. The aim of this retrospective study was to assess the progression of liver stiffness measurement (LSM) in pediatric CF patients. METHODS: The change of LSM, expressed as kPa/year and %/year, was measured using transient elastography (TE, Fibroscan) in 82 CF children (median age: 6.8 years, IQR: 5.8). Mean time interval between the two LSM was 3.5 years. RESULTS: Median initial liver stiffness was 3.7 kPa (IQR: 1.3)., and then progressed by 0.23 kPa/year, i.e. 6%/year. The 7 patients who developed CFLD had a higher initial level of alanine aminotransferase (50 [IQR:15] vs 30 [IQR:18], p = 0.0001) and presented a more rapid progression of LSM (0.94 vs 0.23 kPa/year, p = 0.02). CONCLUSIONS: This study shows that the slope of worsening of liver stiffness is greater in patients who will develop CFLD, suggesting that annual TE may be useful to detect risk of severe liver disease at an earlier stage.

Pubmed : 29112089