engleski

Assessment of liver function in Croatian patients with haemophilia B

INTRODUCTION: Chronic liver disease is an important complication observed in haemophilia patients due to viral infections transmitted during treatment with factor concentrates. Our objective was to examine synthetic liver function parameters [fibrinogen, factor V (FV), albumin and Glu-plasminogen] in haemophilia B (HB) patients. METHODS: The study group consisted of 41 HB patients, aged 2-84 years, divided into two groups according to age: HB1≤18 years (N=15) and HB2>18 years (N=26), and 41 healthy male controls (HMC) aged 6-76 years. The Clauss method was used for the determination of fibrinogen concentrations (Dade Thrombin, Sysmex CS5100, Siemens) whereas FV activities were measured by the one-stage clotting assay (Atellica COAG360, Siemens). Albumin concentrations were measured using the bromocresol green colorimetric method (Cobas c501, Roche Diagnostics), whereas Glu-plasminogen was assayed using the ELISA method (Technoclone). The MedCalc® Software was used for statistical analysis. RESULTS: Higher median fibrinogen concentration (3.1 g/L) was encountered in HB patients (range: 2.7-3.6 g/L), compared to the HMC group (median 2.5 g/L ; range: 2.3-2.8 g/L), resulting in a statistically significant difference (P<0.001). Furthermore, slightly higher fibrinogen concentrations (median 3.1 g/L ; range: 2.4-5.6 g/L) were found in HB2 than in the HB1 group (median 2.8 g/L ; range: 2.3-3.8 g/L), but the difference was not statistically significant (P=0.218). Similar results between HB and HMC were obtained for albumin concentrations (47.7±3.3 g/L and 48.4±2.7 g/L, respectively), Glu-plasminogen concentrations (73.0±14.5 mg/L and 71.4±10.6 mg/L, respectively) and for FV activity (0.98±0.17 kIU/L and 0.95±0.18 kIU/L, respectively). Significantly higher albumin concentrations (P=0.017) were found in the HB2 group (median 48.8 g/L ; range 43.8- 53.6 g/L) compared to the HB1 group (median 46.8 g/L ; range 36.2-51.3 g/L). DISCUSSION/CONCLUSION: Examined parameters (fibrinogen, FV, albumin and Glu-plasminogen) showed that synthetic liver function was maintained in HB patients, regardless of patients' age. Significantly higher fibrinogen concentrations in HB patients as compared to the HMC group, especially in adult patients, could be an indicator of higher inflammatory stimulation in this patients due to haemophilic arthropathy.

haemophilia B, liver

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