Serum YKL-40 in young patients with β-thalassemia major: Relation to hepatitis C virus infection, liver stiffness by transient elastography and cardiovascular complications

Background: YKL-40 is an inflammatory glycoprotein involved in endothelial dysfunction and elevated in sera of patients with liver diseases. Aim: To determine serum YKL-40 among 50 children and adolescents with beta-thalassemia major (beta-TM) compared to 35 healthy controls and assess its relation to liver stiffness by transient elastography (TE), markers of hemolysis, iron overload and various hemolysis-associated complications. Methods: beta-TM patients asymptomatic for heart disease were studied stressing on chelation therapy, serum ferritin, liver iron concentration (MC), cardiac T2* and YKL-40. Echocardiography and TE were performed. Results: Liver cirrhosis (METAVIR F4; TE values > 12.5 kPa) was encountered in 32%. HCV-positive patients had significantly higher WBC count, alanine transaminase (ALT) and serum ferritin than HCV-negative patients. YKL-40 levels were significantly higher in beta-TM patients compared with control (p < 0.001). YKL-40 was significantly higher among patients with heart disease (p = 0.014) or hepatitis C virus (p = 0.004) than those without YKL-40 was correlated with liver stiffness and the degree of hepatic fibrosis being highest among patients with F4 stage (p < 0.001). The YKL-40 cutoff to identify beta-TM patients with liver cirrhosis or heart disease was determined. Patients treated with combined chelation therapy had significantly lower levels of YKL-40 than the monotherapy group (p < 0.001). YKL-40 was positively correlated with transfusion index, ALT, lactate dehydrogenase, serum ferritin and LIC but negatively correlated with cardiac T2*. Conclusion: YKL-40 is a promising marker of cardiovascular disease and liver siderosis in beta-TM patients. The combination of YKL-40 and TE provides a reliable method to assess hepatic fibrosis in young beta-TM patients. (C) 2015 Elsevier Inc. All rights reserved.