Annals of Pediatric Gastroenterology and Hepatology ISPGHAN

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VOLUME 2 , ISSUE 3-4 ( July-Sep/Oct-Dec, 2020 ) > List of Articles

Hepatology Award Paper

Is Exchangeable Copper, a Better Prognostic Tool for Assessing Treatment Control in Hepatic Wilsonšfs Disease in Children?

Jayendra Seetharaman, Amrita Mathias, Surender Kumar Yachha, Anshu Srivastava, Ujjal Poddar

Citation Information : Seetharaman J, Mathias A, Yachha SK, Srivastava A, Poddar U. Is Exchangeable Copper, a Better Prognostic Tool for Assessing Treatment Control in Hepatic Wilsonšfs Disease in Children?. Ann Pediatr Gastroenterol Hepatol 2020; 2 (3-4):14-14.

DOI: 10.5005/jp-journals-11009-0063

License: CC BY-NC 4.0

Published Online: 06-07-2022

Copyright Statement:  Copyright © 2020; The Author(s).


Abstract

Standard assessment of chelation adequacy in Wilson's Disease (WD) by liver function tests (LFT), non-ceruloplasmin copper (NCC) and 24- hour urine copper (UCu) have fallacies. Exchangeable copper (ExCu) is a newer concept in the diagnosis and management of neurological WD. The aim of the study is to explore the role of ExCu as a prognostic tool for assessment of therapy in liver disease. Hepatic WD patients on. 1 y chelation therapy were prospectively enrolled. Newly diagnosed chelation-naive WD children were controls. ExCu in ultrafiltrates was analysed by atomic mass spectrophotometry. Relative exchangeable copper (REC) was defined as percentage ratio of ExCu and total serum copper. Sub-group analysis of ExCu on degree of chelation [well-chelated (UCu 200-500 μgday); poorly chelated (UCu <200 or > 500 μgday)] and liver disease [stable (normal liver synthetic function and transaminases < 2 times upper limit of normal) and unstable (poor synthetic functions and elevated transaminases] The authors found that 96 children (n = 61 on chelation, n = 35 controls) aged 12.5 ± 4.4y were assessed. Chelated WD vs. controls had lower ExCu (0.9 ± 0.6 vs 3 ± 7 μmol/L, p = 0.03) and higher REC (43 ± 27.4% vs 30.4 ± 14.3%, p = 0.01). AUROC of ExCu in chelated vs. controls was 0.8 (cut-off 0.97 μmol/L, sensitivity 80%, specificity of 68%, p<0.01) and stable vs. unstable liver disease was 0.73 (cut-off 0.87 μmol/L, sensitivity 77%, specificity of 65% p = 0.01). ExCu had positive correlation with NCC (r = 0.92, p < 0.001), UCu (r = 0.52, p < 0.001) and Pediatric end-stage liver disease (PELD) scores (r = 0.47, p = 0.01). Multivariate analysis of the entire cohort showed Child Turcotte Pugh scores (p = 0.002) and duration of treatment (> 1-year vs < 1-year) (p = 0.002) significantly influences ExCu values. Median follow-up duration was 10 (0-24) months. 14/96 (15%) either died or were referred for liver transplantation. Survival on Kaplan Meier curves were significantly higher (p = 0.01) if baseline ExCu was ⩽ 0.87. The conclusion from the study was Exchangeable copper is an effective monitoring tool to assess chelation. New chelators reduce ExCu levels to <0.97 μmol/L after 1 year of chelation and thereafter achieve liver stability if values are <0.87 μmol/L.


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