{"title":"随访威尔逊病患者的可交换铜:重新考虑正常范围或改变方法","authors":"Zoe Mariño, Clàudia Garcia-Solà, José Ríos, Ariadna Bono, Sonia García, Anna Miralpeix, Rocío Andreu, Cristina Aguado, Xavier Forns, Mercè Torra, Marina Berenguer","doi":"10.1097/hep.0000000000001105","DOIUrl":null,"url":null,"abstract":"Determining suitable copper parameters for monitoring Wilson disease remains a topic of ongoing discussion. International recommendations currently rely on the combination of urinary copper excretion and non-specific liver markers when considering therapy and time elapsed since diagnosis. The emergence of exchangeable copper (CuEX) as a novel measurement reflecting the “<jats:italic toggle=\"yes\">free copper pool</jats:italic>” held promise as a valuable target to ensure metabolic stability during follow-up, although validation of target ranges remain unknown. We aimed at evaluating CuEX quantification in repeated samples from 92 real-world WD patients during a 2-year period. Patients were classified as “stable” if diagnosis had been made more than one year before and were compliant to stable anti-copper drug and dose. Otherwise, patients were classified as “non-stable”. Two-hundred and thirteen CuEX samples were obtained per clinical practice. Overall, 57% of CuEX measurements fell below the reference “range of normality”, whereas only 34% were within and 9% were above normal levels. There was no association of CuEX levels with therapy, elapsed time from diagnosis or clinical stability, although most of the samples above normality corresponded to non-stable patients. Only 23.4% of the CuEX samples were aligned with data obtained from concomitant urinary copper excretion. Our findings suggest that CuEX is a suboptimal tool for assessing copper homeostasis when used alone and should be used with caution if no additional information is available. Normal reference intervals for WD-treated patients should be redefined, as most of CuEX quantifications fell in the lower range, with no sign of overtreatment in these patients.","PeriodicalId":177,"journal":{"name":"Hepatology","volume":null,"pages":null},"PeriodicalIF":12.9000,"publicationDate":"2024-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Exchangeable copper for patients with Wilson disease at follow-up: rethinking normal ranges or changing methodology\",\"authors\":\"Zoe Mariño, Clàudia Garcia-Solà, José Ríos, Ariadna Bono, Sonia García, Anna Miralpeix, Rocío Andreu, Cristina Aguado, Xavier Forns, Mercè Torra, Marina Berenguer\",\"doi\":\"10.1097/hep.0000000000001105\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Determining suitable copper parameters for monitoring Wilson disease remains a topic of ongoing discussion. International recommendations currently rely on the combination of urinary copper excretion and non-specific liver markers when considering therapy and time elapsed since diagnosis. The emergence of exchangeable copper (CuEX) as a novel measurement reflecting the “<jats:italic toggle=\\\"yes\\\">free copper pool</jats:italic>” held promise as a valuable target to ensure metabolic stability during follow-up, although validation of target ranges remain unknown. We aimed at evaluating CuEX quantification in repeated samples from 92 real-world WD patients during a 2-year period. Patients were classified as “stable” if diagnosis had been made more than one year before and were compliant to stable anti-copper drug and dose. Otherwise, patients were classified as “non-stable”. Two-hundred and thirteen CuEX samples were obtained per clinical practice. Overall, 57% of CuEX measurements fell below the reference “range of normality”, whereas only 34% were within and 9% were above normal levels. There was no association of CuEX levels with therapy, elapsed time from diagnosis or clinical stability, although most of the samples above normality corresponded to non-stable patients. Only 23.4% of the CuEX samples were aligned with data obtained from concomitant urinary copper excretion. Our findings suggest that CuEX is a suboptimal tool for assessing copper homeostasis when used alone and should be used with caution if no additional information is available. Normal reference intervals for WD-treated patients should be redefined, as most of CuEX quantifications fell in the lower range, with no sign of overtreatment in these patients.\",\"PeriodicalId\":177,\"journal\":{\"name\":\"Hepatology\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":12.9000,\"publicationDate\":\"2024-09-24\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Hepatology\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1097/hep.0000000000001105\",\"RegionNum\":1,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"GASTROENTEROLOGY & HEPATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Hepatology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1097/hep.0000000000001105","RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"GASTROENTEROLOGY & HEPATOLOGY","Score":null,"Total":0}
Exchangeable copper for patients with Wilson disease at follow-up: rethinking normal ranges or changing methodology
Determining suitable copper parameters for monitoring Wilson disease remains a topic of ongoing discussion. International recommendations currently rely on the combination of urinary copper excretion and non-specific liver markers when considering therapy and time elapsed since diagnosis. The emergence of exchangeable copper (CuEX) as a novel measurement reflecting the “free copper pool” held promise as a valuable target to ensure metabolic stability during follow-up, although validation of target ranges remain unknown. We aimed at evaluating CuEX quantification in repeated samples from 92 real-world WD patients during a 2-year period. Patients were classified as “stable” if diagnosis had been made more than one year before and were compliant to stable anti-copper drug and dose. Otherwise, patients were classified as “non-stable”. Two-hundred and thirteen CuEX samples were obtained per clinical practice. Overall, 57% of CuEX measurements fell below the reference “range of normality”, whereas only 34% were within and 9% were above normal levels. There was no association of CuEX levels with therapy, elapsed time from diagnosis or clinical stability, although most of the samples above normality corresponded to non-stable patients. Only 23.4% of the CuEX samples were aligned with data obtained from concomitant urinary copper excretion. Our findings suggest that CuEX is a suboptimal tool for assessing copper homeostasis when used alone and should be used with caution if no additional information is available. Normal reference intervals for WD-treated patients should be redefined, as most of CuEX quantifications fell in the lower range, with no sign of overtreatment in these patients.
期刊介绍:
HEPATOLOGY is recognized as the leading publication in the field of liver disease. It features original, peer-reviewed articles covering various aspects of liver structure, function, and disease. The journal's distinguished Editorial Board carefully selects the best articles each month, focusing on topics including immunology, chronic hepatitis, viral hepatitis, cirrhosis, genetic and metabolic liver diseases, liver cancer, and drug metabolism.