Objectives: Wilson's disease (WD) is a metabolic disorder leading to hepatic and extrahepatic copper deposition. Several studies have reported that besides copper (Cu), iron (Fe) and zinc (Zn) are also accumulated at varying levels in various tissues in WD. However, there is not an adequate number of studies investigating the effects of Fe and Zn status on WD presentation and prognosis. We aimed to evaluate serum levels of ferritin (SFr), copper (SCu), and zinc (SZn) in WD and determine their role in disease presentation and prognosis.
Materials-Method: We retrospectively reviewed the medical records of 97 pediatric patients with WD who were diagnosed and followed at Inonii University Pediatric Gastroenterology, Hepatology and Nutrition Department between January 2006 and May 2017. Serum Cu and Zn levels were analyzed by using flame atomic absorption spectrophotometer. Ferritin was analyzed by chemiluminescence immunoassay method.
Results: Analysis of serum levels of the elements according to the type of presentation, there was no significant difference between the groups for ceruloplasmin. However, SCU, FSCu, SFr and 24 h urinary copper levels were significantly higher (p = 0.002, p = 0.003, p = 0.023 and p < 0.001, respectively) and SZn and CSZn levels were significantly lower (fulminant WD). p < 0.001, p < 0.001). There was a positive correlation between SFr, SCu serum levels and mortality scores (respectively, r: 0.501, 0.564 for PELD, r:0.490, 0.504 for MELD, r: 0.345, 0.374 for Dhwan), and a negative correlation between SZn level and mortality scores. (r:-0.650 for PELD, r:0.703 for MELD, r:-0.642 for Dhwan) We used the ROC curves to determine the worst prognosis for fulminant Wilson disease. According to these limit values, we found that the sensitivity and specificity of FWD development was significantly higher. (for SZn sensitivity of 91.5%, a specificity of 100%, p= < 0,001, for SCu predicted FWD development with a sensitivity of 100%, a specificity of 73.7%, p = < 0,001, for SFr predicted FWD development with a sensitivity of 92.9%, a specificity of 66.2%, p < 0,001)
Conclusion: Our study suggests that SFr, SCu, SZn levels might have prognostic importance for WD.
C1 [Gungor, Sukru; Selimoglu, Mukadder Ayse; Varol, Fatma Ilknur] Inonu Univ, Fac Med, Dept Pediat Gastroenterol Hepatol & Nutr, Malatya, Turkey.
[Gungor, Serdal] Inonu Univ, Fac Med, Dept Pediat Neurol, Malatya, Turkey.
[Uremis, Muhammed Mehdi] Inonu Univ, Fac Med, Dept Med Biochem, Malatya, Turkey.