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 patients with CF. METHODS: The change of LSM, expressed as kPa/year and %/year, was measured using transient elastography (Fibroscan) in 82 children with CF (median age: 6.8 years, interquartile range [IQR]: 5.8). Mean time interval between the 2 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, that is, 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: The present study shows that the slope of worsening of liver stiffness is greater in patients who will develop CFLD, suggesting that annual transient elastography may be useful to detect risk of severe liver disease at an earlier stage.
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 patients with CF. METHODS: The change of LSM, expressed as kPa/year and %/year, was measured using transient elastography (Fibroscan) in 82 children with CF (median age: 6.8 years, interquartile range [IQR]: 5.8). Mean time interval between the 2 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, that is, 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: The present study shows that the slope of worsening of liver stiffness is greater in patients who will develop CFLD, suggesting that annual transient elastography may be useful to detect risk of severe liver disease at an earlier stage.
Authors: Raphael Enaud; Eric Frison; Sophie Missonnier; Aude Fischer; Victor de Ledinghen; Paul Perez; Stéphanie Bui; Michael Fayon; Jean-François Chateil; Thierry Lamireau Journal: Pediatr Res Date: 2021-03-17 Impact factor: 3.953
Authors: Wen Ye; Daniel H Leung; Jean P Molleston; Simon C Ling; Karen F Murray; Jennifer L Nicholas; Suiyuan Huang; Boaz W Karmazyn; Roger K Harned; Prakash Masand; Adina L Alazraki; Oscar M Navarro; Randolph K Otto; Joseph J Palermo; Alexander J Towbin; Estella M Alonso; Wikrom W Karnsakul; Sarah Jane Schwarzenberg; Glenn F Seidel; Marilyn Siegel; John C Magee; Michael R Narkewicz; A Jay Freeman Journal: Hepatol Commun Date: 2021-05-13
Authors: Dorota Wicher; Irena Jankowska; Patryk Lipiński; Paulina Szymańska-Rożek; Jakub Kmiotek; Wojciech Jańczyk; Jacek Rubik; Krystyna Chrzanowska; Piotr Socha Journal: Front Pediatr Date: 2019-01-11 Impact factor: 3.418