RESUMEN
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 ß-thalassemia major (ß-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: ß-TM patients asymptomatic for heart disease were studied stressing on chelation therapy, serum ferritin, liver iron concentration (LIC), cardiac T2* and YKL-40. Echocardiography and TE were performed. RESULTS: Liver cirrhosis (METAVIR F4; TE values>12.5kPa) 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 ß-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 ß-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 ß-TM patients. The combination of YKL-40 and TE provides a reliable method to assess hepatic fibrosis in young ß-TM patients.