title: A multicenter prospective study on the risk of acquiring liver disease in anti-hepatitis C virus negative patients affected from homozygous beta-thalassemia.
abstract: Although the risk of transfusion-transmitted hepatitis has been recently reduced, transfusion-dependent beta-thalassemia patients may still develop liver disease due to viral infection or iron overload. We assessed the frequency and causes of liver dysfunction in a cohort of anti-hepatitis C virus (HCV) negative thalassemics. Of 1,481 thalassemics enrolled in 31 centers, 219 (14.8%) tested anti-HCV- by second-generation assays; 181 completed a 3-year follow-up program consisting of alanine-aminotransferase (ALT) measurement at each transfusion and anti-HCV determination by third-generation enzyme-immunoassay (EIA-3) at the end of study. Serum ferritin levels were determined at baseline and at the end of follow-up. Ten patients were anti-HCV+ by EIA-3 at the end of follow-up. Of them, seven were already positive in 1992 to 1993 when the initial sera were retested by EIA-3, one tested indeterminate by confirmatory assay, and two had true seroconversion (incidence, 4. 27/1,000 person years; risk of infection, 1/7,100 blood units, 95% confidence interval [CI], 1 in 2,000-1 in 71,000 units). At baseline, 67 of 174 thalassemics had abnormal ALT. Of those with normal ALT, seven subsequently developed at least one episode of moderate ALT increase (incidence, 24.6/1,000 person-years). All of the 20 patients with ferritin values >/=3,000 ng/mL had clinically relevant ALT abnormalities, as compared with 53 of 151 with <3,000 ng/mL (P < .005). Hepatic dysfunction is still frequent in thalassemics. Although it is mainly attributable to siderosis and primary HCV infection, the role of undiscovered transmissible agents cannot be excluded.
authors.affiliation: Centro Trasfusionale e di Immunologia dei Trapianti, Servizio Autonomo per il Prelievo e la Conservazione di Organi e Tessuti, Milano, Italy.
authors.forename: D
authors.initials: D
authors.lastname: Prati
authors.forename: A
authors.initials: A
authors.lastname: Zanella
authors.forename: E
authors.initials: E
authors.lastname: Farma
authors.forename: C
authors.initials: C
authors.lastname: De Mattei
authors.forename: P
authors.initials: P
authors.lastname: Bosoni
authors.forename: M
authors.initials: M
authors.lastname: Zappa
authors.forename: A
authors.initials: A
authors.lastname: Picone
authors.forename: F
authors.initials: F
authors.lastname: Mozzi
authors.forename: P
authors.initials: P
authors.lastname: Rebulla
authors.forename: M D
authors.initials: MD
authors.lastname: Cappellini
authors.forename: J P
authors.initials: JP
authors.lastname: Allain
authors.forename: G
authors.initials: G
authors.lastname: Sirchia
mesh_terms: D000293:Adolescent; D000328:Adult; D000410:Alanine Transaminase / Q000097:blood; D002648:Child; D002675:Child, Preschool; D005260:Female; D005293:Ferritins / Q000097:blood; D020022:Genetic Predisposition to Disease; D016174:Hepacivirus; D018937:Hepatitis C Antibodies / Q000097:blood; D006525:Hepatitis, Viral, Human / Q000635:transmission; D006720:Homozygote; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D019190:Iron Overload / Q000097:blood / Q000209:etiology; D007558:Italy / Q000453:epidemiology; D008107:Liver Diseases / Q000097:blood / Q000453:epidemiology / Q000209:etiology*; D008111:Liver Function Tests; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D012306:Risk; D065227:Transfusion Reaction; D017086:beta-Thalassemia / Q000150:complications* / Q000235:genetics / Q000628:therapy
chemical_list: D018937:Hepatitis C Antibodies; D005293:Ferritins; D000410:Alanine Transaminase