Natural history of hepatitis C in HIV-negative patients with congenital coagulation disorders

Background/Aims: Knowledge of the natural history of hepatitis C is useful for counselling patients and planning treatment, More data are needed from unselected patient groups without concomitant disease, The aim of this stud y was to describe the natural history of hepatitis C, two decades after inf ection, in a homogeneous, and well-defined group of HIV-negative patients w ith congenital coagulation defects who had not received specific therapy fo r chronic hepatitis C, Methods: Medical history physical examination, laboratory tests and abdomin al ultrasonography were performed in 45 HCV-RNA positive, HIV-negative pati ents, mainly haemophiliacs, from a single centre, Patients were classified according to results of ultrasonography. Results: Two patients had experienced an episode of variceal bleeding; all others were asymptomatic, None had ascites, HCV-RNA titres were >500 000 co pies/mlin 23 patients, genotype was 1 in 31 patients. Forty (89%) had eleva ted transaminases, liver synthesis function was diminished in 7 (16%), and platelet count in 8 (18%), Ultrasonography was normal in 26 (58%) patients, 12 (27%) had isolated splenomegaly, and 7 (16%) had liver nodularity compa tible with cirrhosis, Univariate analysis disclosed higher transaminases an d gamma GT, higher age at acquisition of infection and higher present age a s risk, factors for more advanced disease. Of these, only higher present ag e was an independent predictor in multivariate analysis. Conclusions: Median 19 years after infection, 58% of patients had no other signs of liver disease than raised transaminases, 16% had cirrhosis on ultr asonography, Only 2/45 patients had symptomatic disease. Higher present age is the main risk factor for advanced disease in this group.