Sarcopenia is associated with osteopenia and impaired quality of life in children with genetic intrahepatic cholestatic liver disease Hepatology Communications
Abstract INTRODUCTION METHODS RESULTS Footnotes Contributor Information REFERENC Abstract Background: Sarcopenia occurs in pediatric chronic liver disease, although the prevalence and contributing factors in genetic intrahepatic cholestasis are not well-described. The objective of this study was to measure muscle mass in school-aged children with genetic intrahepatic cholestasis and assess relationships between sarcopenia, clinical variables, and outcomes. Methods: Estimated skeletal muscle mass (eSMM) was calculated on dual-energy x-ray absorptiometry obtained in a Childhood Liver Disease Research Network study of children with bile acid synthesis disorders(BASD) alpha-1 antitrypsin deficiency (a1ATd), chronic intrahepatic cholestasis (CIC), and Alagille syndrome (ALGS). Relationships between eSMM, liver disease, and transplant-free survival were assessed. Results: eSMM was calculated in 127 participants (5–18 y): 12 BASD, 41 a1ATd, 33 CIC, and 41 ALGS. eSMM z-score was lower in CIC (−1.6 ± 1.3) and ALGS (−2.1 ± 1.0) than BASD (-0.1 ± 1.1) and a1ATd (−0.5 ± 0.8, p < 0.001). Sarcopenia (defined as eSMM z-score ≤− 2) was present in 33.3% of CIC and 41.5% of ALGS participants. eSMM correlated with bone mineral density in the 4 disease groups ( r =0.52–0.55, p < 0.001–0.07), but not serum bile acids, bilirubin, aspartate aminotransferase/platelet ratio index, or clinically evident portal hypertension. Of the 2 patients who died (1 with sarcopenia) and 18 who underwent liver transplant (LT, 4 with sarcopenia), eSMM z-score did not predict transplant-free survival. eSMM z-score correlated with the Physical Pediatric Quality of Life Inventory score ( r =0.38–0.53, p = 0.007–0.04) in CIC and a1ATd. Conclusion: Severe sarcopenia occurs in some children with ALGS and CIC. The lack of correlation between eSMM and biochemical cholestasis suggests mechanisms beyond cholestasis contribute to sarcopenia. While sarcopenia did not predict transplant-free survival, LT and death were infrequent events. Future studies may define mechanisms of sarcopenia in genetic intrahepatic cholestasis.
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Julia M Boster, Nathan P Goodrich, Cathie Spino et al.. Sarcopenia is associated with osteopenia and impaired quality of life in children with genetic intrahepatic cholestatic liver disease. Hepatology Communications. (2023). https://doi.org/10.1097/HC9.0000000000000293 Julia M Boster, Nathan P Goodrich, Cathie Spino, Kathleen M Loomes, Estella M Alonso, Binita M Kamath, Ronald J Sokol, Saul Karpen, Alexander Miethke, Benjamin L Shneider, Jean P Molleston, Rohit Kohli, Simon P Horslen, Philip Rosenthal, Pamela L Valentino, Jeffrey H Teckman, Thomas N Hangartner, Shikha S Sundaram
1Department of Pediatrics, Pediatric Liver Center, Digestive Health Institute and Section of Pediatric Gastroenterology, Hepatology & Nutrition, Children’s Hospital Colorado, University of Colorado School of Medicine, Aurora, Colorado, USA 2Arbor Research Collaborative for Health, Ann Arbor, Michigan, USA 3Department of Biostatistics, University of Michigan, Ann Arbor, Michigan, USA 4Division of Gastroenterology, Hepatology and Nutrition, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA 5Ann and Robert Lurie Children’s Hospital, Chicago, Illinois, USA 6Division of Gastroenterology, Hepatology and Nutrition, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada 7Children’s Healthcare of Atlanta, Atlanta, Georgia, USA 8Cincinnati Children’s Hospital Medical, Cincinnati, Ohio, USA 9Baylor College of Medicine, Texas Children’s Hospital, Houston, Texas, USA 10Riley Hospital for Children, Indianapolis, Indiana, USA 11Children’s Hospital Los Angeles, Los Angeles, California, USA 12UPMC Children’s Hospital of Pittsburgh, Pittsburgh, Pennsylvania, USA 13UCSF Benioff Children’s Hospital, San Francisco, California, USA 14Seattle Children’s Hospital, Seattle, Washington, USA 15Saint Louis University School of Medicine, St. Louis, Missouri, USA 16Department of Biomedical, Industrial & Human Factors Engineering, Wright State University, Dayton, Ohio, USA 