Wilson Hastalarında Serum Değişken Ve Göreceli Değişken Bakır Düzeylerinin Klinik Parametreler ve Multiparametrik Karaciğer Mrı Bulgularıyla Karşılaştırılması

Abstract

Durukan BM. The Evaluation of Exchangeable and Relative Exchangeable Copper with Clinical Parameters and Multiparametric Liver MRI Findings in Patients with Wilson Disease. Hacettepe University Faculty of Medicine, Thesis in Internal Medicine Department, Ankara, 2022. Objective: This study aimed to investigate the correlation between exchangeable (EC) and relative exchangeable copper (REC) with 24-hour urinary copper excretion and the relationship of them with Multiparametric Liver MRI that is sensitive to fibrosis and steatosis in liver diseases. Materials and Methods: Wilson’s Disease patients followed up at our gastroenterology and neurology clinics were included. Dyspepsia patients who followed up at the gastroenterology clinic were included as a control group. Face-to-face interviews were done with all the participants for the history of liver disease, comorbidities, and drug treatments. The electronic system records were reviewed for laboratory results, radiologic imaging, and liver biopsy findings. A 10 cc sample of blood was taken for EC and serum total copper analyzes. Multiparametric liver MRIs and measurement of 24-hour urinary copper excretion were done in patients with Wilson’s Disease. If available, the previous liver biopsy of patients was also re-examined. EC and serum total copper levels were measured by inductively coupled plasma mass spectrometry (ICP-MS), and the 24-hour urinary copper level was measured by atomic absorption spectrometry. Results: 43 controls and 38 Wilson’s Disease patients were included in the study. Hepatic involvement was observed in %97 Wilson's Disease patients. The median stiffness of the liver parenchyma of the patients was 2.45 (1.71-6.2) kPa which is within normal limits. MR-PDFF technique showed that 62% of the patients had no fatty liver disease. The liver stiffness was a positively correlated with and age (r=0.514; p=0.002), AST (r=0.445; p=0.007), GGT (r=0.549; p=0.001), ALP (r=0.365; p=0.031), INR (r=0.404; p=0.016), creatinine (r=0.344; p=0.043), FIB-4 score (r=0.694; p<0.0001), and also a negatively correlated with white blood cell count (r=-0.49; p=0.003) and platelet count (r=-0.607; p<0.0001). There was a positive correlation between MR-PDF fat fraction and serum triglyceride level (r=0.532; p=0.013). No correlation was found between serum copper tests and 24-hour urinary copper excretion. Except for ceruloplasmin (p=0.348; r=0.032), EC didn’t correlate with any clinical or laboratory parameter. REC positively correlated with ALP (r=0.338; p=0.003) and GGT (r=0.36; p=0.001), and negatively correlated with platelet count (r=-0.507; p<0.0001). EC positively correlated with MRE-PDFF fat fraction (r=0.534; p=0.005) and T1 time (r=0.371; p=0.028). There was a negative correlation between REC and T2* time (r=-0.422; and p=0.012). Conclusion: There was no correlation between 24-hour urinary copper excretion which is a test of choice for routine clinical follow-up of patients, and EC or REC measurements which have the potential to be used as alternative tests for evaluating body copper load. In addition, further studies are needed to investigate the role of clinical parameters, EC, and REC correlating with multiparametric liver MRI findings such as fibrosis, steatosis, and iron load. Keywords: Wilson disease, Exchangeable copper, Relative exchangeable copper, Multiparametric liver MRI.