Association Between Transient Elastography and Controlled Attenuated Parameter and Liver Ultrasound in Children With Cystic Fibrosis

• Authors: Alvin Freeman, Emory University; Wen Ye, University of Michigan; Daniel H Leung, Texas Children’s Hospital; Jean P Molleston, Indiana University School of Medicine; Simon C Ling, University of Toronto; Karen F Murray, University of Washington; Jennifer L Nicholas, Washington University School of Medicine; Suiyuan Huang, University of Michigan; Boaz W Karmazyn, Indiana Univ Sch Med; Roger K Harned, Children’s Hospital Colorado and University of Colorado; Prakash Masand, Texas Childrens Hospital; Adina Alazraki, Emory University; Oscar M Navarro, University of Toronto; Randolph K Otto, Seattle Childrens Hospital; Joseph J Palermo, Cincinnati Children’s Hospital Medical Center; Alexander J Towbin, Cincinnati Children’s Hospital Medical Center; Estella M Alonso, Ann & Robert H Lurie Childrens Hosp; Wikrom W Karnsakul, John Hopkins School of Medicine; Sarah Jane Schwarzenberg, University of Minnesota; Glenn F Seidel, Lucile Packard Children’s Hospital; Marilyn Siegel, Washington University; John C Magee, University of Michigan; Michael R Narkewicz, Children’s Hospital Colorado; Jay A Jay Freeman, Emory University
• Journal Title: HEPATOLOGY COMMUNICATIONS
• Volume: Volume 5, Number 8
• Publisher: JOHN WILEY & SONS LTD | 2021-05-13, Pages 1362-1372
• Type of Work: Article | Final Publisher PDF
• Publisher DOI: http://doi.org/10.1002/hep4.1719
• Subjects: Biology, Biostatistics; Health Sciences, Radiology
• Keywords: Science & Technology; Life Sciences & Biomedicine; Gastroenterology & Hepatology; FATTY LIVER; FOLLOW-UP; DISEASE; PREDICTION; PATTERNS; BIOPSY

Abstract

Methods to identify children with cystic fibrosis (CF) at risk for development of advanced liver disease are lacking. We aim to determine the association between liver stiffness measurement (LSM) by vibration-controlled transient elastography (VCTE) with research ultrasound (US) patterns and conventional hepatic markers as a potential means to follow liver disease progression in children with CF. ELASTIC (Longitudinal Assessment of Transient Elastography in CF) is a nested cohort of 141 patients, ages 7-21, enrolled in the Prediction by US of Risk of Hepatic Cirrhosis in CF (PUSH) Study. We studied the association between LSM with research-grade US patterns (normal [NL], heterogeneous [HTG], homogeneous [HMG], or nodular [NOD]) and conventional hepatic markers. In a subgroup (n = 79), the association between controlled attenuation parameter (CAP) and US pattern was explored. Among 133 subjects undergoing VCTE, NOD participants (n = 26) had a significantly higher median (interquartile range) LSM of 9.1 kPa (6.3, 15.8) versus NL (n = 72, 5.1 kPa [4.2, 7.0]; P < 0.0001), HMG (n = 17, 5.9 kPa [5.2, 7.8]; P = 0.0013), and HTG (n = 18, 6.1 kPa [4.7, 7.0]; P = 0.0008) participants. HMG participants (n = 14) had a significantly higher mean CAP (SD) (270.5 dB/m [61.1]) compared with NL (n = 40, 218.8 dB/m [46.5]; P = 0.0027), HTG (n = 10, 218.1 dB/m [60.7]; P = 0.044), and NOD (n = 15, 222.7 dB/m [56.4]; P = 0.041) participants. LSM had a negative correlation with platelet count (rs = (Formula presented.) 0.28, P = 0.0071) and positive correlation with aspartate aminotransferase–to-platelet ratio index (rs = 0.38, P = 0.0002), Fibrosis-4 index (rs = 0.36, P = 0.0007), gamma-glutamyltransferase (GGT; rs = 0.35, P = 0.0017), GGT-to-platelet ratio (rs = 0.35, P = 0.003), and US spleen size z-score (rs = 0.27, P = 0.0073). Conclusion: VCTE is associated with US patterns and conventional markers in patients with liver disease with CF.