Hepatic Dry Copper Weight in Pediatric Autoimmune Liver Disease.

Jeremy S Nayagam, Deepak Joshi, Richard J Thompson, Anil Dhawan, Nedim Hadzic, Claudia Mestre-Alagarda, Maesha Deheragoda, Marianne Samyn

Journal of pediatric gastroenterology and nutrition 2023 Feb 01

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Elevated hepatic dry copper weig ht is recognized in adults with autoimmune liver disease (AILD) and chronic cholestasis. We aim to review hepatic dry copper weig ht in pediatric AILD. Retrospective review of pediatric AILD managed at our institution from 1999 to 2018, and 104 patients with hepatic dry copper weig ht assessment were included. Median age at presentation was 13.4 years (interquartile range, IQR, 11.7-14.9), 60% female, 54% autoimmune hepatitis, 42% autoimmune sclerosing cholangitis, and 4% primary sclerosing cholangitis. Histological features of advanced liver fibrosis in 68%. Median hepatic dry copper weig ht was 51.1 µg/g dry weight (IQR, 28.0-103.8). Elevated hepatic dry copper weig ht (>50 µg/g dry weight) was present in 51%, and was not associated with AILD subtype ( P = 0.83), age at presentation ( P = 0.68), or advanced fibrosis ( P = 0.53). Liver transplantation (LT) was performed in 10%, who had higher hepatic dry copper weig ht (148.5 µg/g dry weight [IQR, 39.5-257.3] vs 47.5 [IQR, 27.8-91.5], P = 0.04); however this was not associated with LT on multivariate analysis (hazard ratio 1.002, 95% CI 0.999-1.005, P = 0.23). In 8 (7.7%) patients ATP7B was sequenced and potentially disease causing variants were identified in 2 patients, both who required LT. Elevations in hepatic dry copper weig ht are common in pediatric AILD. Unlike in adults, it is not associated with AILD subtypes with cholestasis. Higher dry copper weig ht was detected in patients who required LT. While further work is needed to identify the significance of copper deposition in pediatric AILD, we recommend close monitoring of patients with elevated levels for progressive liver disease. Copyright © 2022 by European Society for European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.

Citation

Jeremy S Nayagam, Deepak Joshi, Richard J Thompson, Anil Dhawan, Nedim Hadzic, Claudia Mestre-Alagarda, Maesha Deheragoda, Marianne Samyn. Hepatic Dry Copper Weight in Pediatric Autoimmune Liver Disease. Journal of pediatric gastroenterology and nutrition. 2023 Feb 01;76(2):e21-e26