“
“Only very few pharmacokinetic (PK) studies comparing plasma derived FVIII (pd-FVIII) against recombinant FVIII (rFVIII) concentrates are available. The studies have been generally conducted to demonstrate the bioequivalence of a new product with an old one. The switch from a plasma-derived find more FVIII (pd-FVIII) to a rFVIII concentrate is a good moment to enrol the patients

in a comparative PK study. To achieve information on the PK characteristics of two different classes of FVIII concentrates, according to two different designs: a 10 FVIII concentration/time point design and a reduced 4-point design. A single dose PK comparing pd- and rFVIII concentrates has been performed in four Haemophilia Centres of Italy. Seventeen haemophilia A patients underwent two subsequent

single dose PK studies at the moment of switching. Two-compartment- and Non-compartment-analysis did not show significant differences between the outcomes of PK of pd-FVIII and rFVIII, due to inter-patient variability. In vivo recovery (IVR) of rFVIII was slightly higher than that of pd-FVIII and rFVIII/pd-FVIII AUC ratio was 1.37 in 11/17 patients. The difference is only due to the initial distribution phase because after the first 10 h from the end of the infusion, the two decay curves are overlapping. The elimination half-life of the concentrates was very selleck chemicals similar even though a complete bioequivalence was not demonstrated because of a higher AUC

of rFVIII concentrates, limited to the distribution phase. The higher Cmax and IVR of rFVIII may be due to the presence of heterodimers activated forms of the recombinant molecules. “
“Assessment of joint involvement mafosfamide in hemophilia is based on physical examination, radiography, magnetic resonance imaging (MRI), and, recently, ultrasonography. Scales for the scoring of hemophilic arthropathy have been developed and used in clinical trials to evaluate different treatment protocols such as prophylaxis versus treatment on demand and also different prophylactic regimens. During the 1980s, the World Federation of Hemophilia (WFH) endorsed a physical examination (PE) scale and a radiographic (Pettersson) scale. Improved hemophilia treatment has resulted in less pronounced hemophiliac arthropathy, which has prompted updating of the PE scale in terms of its sensitivity and suitability for children. The new scale is named the Hemophilia Joint Health Score (HJHS). The advent of MRI has increased the ability to detect early joint disease, and an international MRI scale has been developed. These new scales have the potential to contribute to the development and evaluation of better prophylactic protocols, but further prospective studies are needed. “
“Summary.