title: Serum YKL-40 in young patients with β-thalassemia major: Relation to hepatitis C virus infection, liver stiffness by transient elastography and cardiovascular complications.
abstract: BACKGROUND
  YKL-40 is an inflammatory glycoprotein involved in endothelial dysfunction and elevated in sera of patients with liver diseases.
  
  
  OBJECTIVE
  To determine serum YKL-40 among 50 children and adolescents with β-thalassemia major (β-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
  β-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 β-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 β-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*.
  
  
  CONCLUSIONS
  YKL-40 is a promising marker of cardiovascular disease and liver siderosis in β-TM patients. The combination of YKL-40 and TE provides a reliable method to assess hepatic fibrosis in young β-TM patients.
authors.affiliation: Department of Pediatrics, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
authors.forename: Mohamed Abo
authors.initials: MA
authors.lastname: El-Asrar
authors.affiliation: Department of Pediatrics, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
authors.forename: Nancy Samir
authors.initials: NS
authors.lastname: Elbarbary
authors.affiliation: Department of Clinical Pathology, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
authors.forename: Eman Abdel Rahman
authors.initials: EA
authors.lastname: Ismail
authors.affiliation: Department of Pediatrics, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
authors.forename: Ahmed Mohamed
authors.initials: AM
authors.lastname: Elshenity
mesh_terms: D054392:Adipokines / Q000097:blood*; D000293:Adolescent; D002648:Child; D000071451:Chitinase-3-Like Protein 1; D003430:Cross-Sectional Studies; D054459:Elasticity Imaging Techniques; D005260:Female; D006331:Heart Diseases / Q000097:blood / Q000150:complications*; D006526:Hepatitis C / Q000097:blood / Q000150:complications*; D006801:Humans; D037102:Lectins / Q000097:blood*; D008099:Liver / Q000473:pathology; D008103:Liver Cirrhosis / Q000097:blood / Q000150:complications* / Q000473:pathology; D008297:Male; D017086:beta-Thalassemia / Q000097:blood* / Q000150:complications*
chemical_list: D054392:Adipokines; C084533:CHI3L1 protein, human; D000071451:Chitinase-3-Like Protein 1; D037102:Lectins
keywords: Cardiac disease; Hepatitis C; Iron overload; Liver fibrosis; Transient elastography; YKL-40; β-thalassemia