title: Liver fibrosis in young Egyptian beta-thalassemia major patients: relation to hepatitis C virus and  compliance with chelation.
abstract: BACKGROUND
  The main causes of liver fibrosis in transfusion-dependent thalassemia major are hepatitis C virus (HCV) infection and hepatic iron overload. The study aimed to assess liver fibrosis in Egyptian adolescents and young adult poly-transfused beta thalassemia patients infected with HCV using liver FibroScan in relation to iron overload and Liver iron concentration (LIC).
  
  
  METHODS
  Fifty-one regularly transfused beta thalassemia patients above 12 years old were subjected to measurement of serum alanine transaminase (ALT), serum ferritin (SF), HCV (antibody and RNA), LIC assessed by hepatic R2* and transient elastography (TE) (FibroScan). FibroTest and liver biopsy were done to 25 patients.
  
  
  RESULTS
  Eighty two% of studied thalassemia patients were HCV antibody positive; 21(49%) of them were viremic (HCV RNA positive); median LIC was 12 mg/gm dry weight. There were strong positive correlation between the degree of liver stiffness and Ishak fibrosis score assessed in liver biopsy specimens (P = 0.002) and between FibroScan and FibroTest results (P < 0.001). Patients with HCV viremia showed significantly higher ALT, γ-glutamyl transpeptidase (GGT), SF, LIC and increased liver stiffness compared to patients with no viremia (P = 0.0001, 0.001, 0.012, 0.006 and 0.001) respectively. Liver cirrhosis (TE values > 12.5kPa) was encountered in 23.5% and variable degrees of liver fibrosis (TE values > 6-12.5 kPa) in 35% of studied thalassemic patients.
  
  
  CONCLUSIONS
  Young beta thalassemia patients with active hepatitis C infection may have hepatic cirrhosis or fibrosis at young age when accompanied with hepatic siderosis. Non invasive Liver FibroScan and Fibro-Test were reliable methods to assess liver fibrosis in young thalassemic-patients.
authors.affiliation: Department of Pediatric Hematology, Ain Shams University, Cairo 11566, Egypt. elalfym@hotmail.com
authors.forename: Mohsen S
authors.initials: MS
authors.lastname: Elalfy
authors.forename: Gamal
authors.initials: G
authors.lastname: Esmat
authors.forename: Randa M
authors.initials: RM
authors.lastname: Matter
authors.forename: Hesham E
authors.initials: HE
authors.lastname: Abdel Aziz
authors.forename: Walid A
authors.initials: WA
authors.lastname: Massoud
mesh_terms: D000293:Adolescent; D000465:Algorithms; D016632:Apolipoprotein A-I / Q000097:blood; D001663:Bilirubin / Q000097:blood; D015415:Biomarkers / Q000097:blood; D062005:Biopsy, Large-Core Needle; D002648:Child; D003430:Cross-Sectional Studies; D004534:Egypt; D054459:Elasticity Imaging Techniques; D017707:Erythrocyte Transfusion / Q000009:adverse effects*; D005260:Female; D005293:Ferritins / Q000097:blood; D006242:Haptoglobins / Q000032:analysis; D006486:Hemosiderosis / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology; D016174:Hepacivirus / Q000235:genetics; D006526:Hepatitis C / Q000150:complications / Q000635:transmission*; D006801:Humans; D007502:Iron Chelating Agents / Q000627:therapeutic use*; D008099:Liver / Q000000981:diagnostic imaging / Q000473:pathology; D008103:Liver Cirrhosis / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D055118:Medication Adherence; D012367:RNA, Viral / Q000097:blood; D055815:Young Adult; D000511:alpha-Macroglobulins / Q000032:analysis; D017086:beta-Thalassemia / Q000150:complications / Q000628:therapy*; D005723:gamma-Glutamyltransferase / Q000097:blood
chemical_list: D016632:Apolipoprotein A-I; D015415:Biomarkers; C485359:HP protein, human; D006242:Haptoglobins; D007502:Iron Chelating Agents; D012367:RNA, Viral; D000511:alpha-Macroglobulins; D005293:Ferritins; D005723:gamma-Glutamyltransferase; C554782:gamma-glutamyltransferase, human; D001663:Bilirubin