The natural history of childhood-acquired hepatitis C infection in patients with inherited bleeding disorders.

BACKGROUND

Although many patients with inherited bleeding disorders have been infected with hepatitis C in early childhood, the natural history of infection in this patient group remains poorly defined.

STUDY DESIGN AND METHODS

A total of 212 patients with inherited bleeding disorders born between 1976 and 1992 were evaluated for hepatitis C virus (HCV) infection, spontaneous clearance, and (by noninvasive tests) progressive liver disease.

RESULTS

A total of 120 of 212 patients had been exposed to non-HCV-inactivated clotting products, and 68 of these 120 patients (57%) were anti-HCV-positive. Of these patients, 44 (65%) had chronic hepatitis C (HCV RNA-positive) and 24 (35%) showed spontaneous clearance (HCV RNA-negative). Five patients with hepatitis C were coinfected with hepatitis B virus and/or human immunodeficiency virus (HIV). Multivariate analysis indicated that hepatitis C infection was independently associated with longer treatment period (odds ratio [OR], 1.6; 95% confidence interval [CI], 1.3-1.9) and exposure to a larger number of donors (OR, 2.1; 95% CI, 1.1-3.9). Spontaneous HCV clearance was associated with a younger age at first exposure to clotting product (p = 0.02). After a mean infection period of 21 years, evidence of cirrhosis was present in 2 patients (5%), both of whom were coinfected with HIV.

CONCLUSION

Spontaneous HCV clearance is associated with young age at infection. Despite frequent childhood-acquired hepatitis C infection among patients with inherited bleeding disorders, progression to cirrhosis after 21 years of infection is rare. The diagnosis of cirrhosis without biopsy, however, remains challenging in this population, and new, noninvasive means must be developed to accurately identify cirrhotic patients.