The overall prevalence of inhibitors in hemophilia is estimated a

The overall prevalence of inhibitors in hemophilia is estimated at 5–7% for hemophilia A and 1.5–4% for hemophilia B. Inhibitor incidence is higher in severe hemophilia A patients at initial treatment (previously untreated patients, PUPs, average incidence 25%),

with a peak incidence after 14–20 days of treatment (exposure days, ED). In severe hemophilia B patients the incidence varies Tanespimycin datasheet between 9 and 23%, with a peak incidence at 7–10 ED. The incidence of inhibitors in hemophilia A patients after the initial treatment period (previously treated patients, PTPs) is estimated at around 0.2/100 patient years, with the upper limit of the confidence interval at 0.4. Very little is known about the natural history of inhibitors in the absence of treatments aiming at their eradication (immunotolerance induction, ITI). “
“The principles of pharmacokinetic (PK) dose tailoring in clinical practice, using

limited blood sampling and Bayesian PK analysis, have been described for factor VIII (FVIII). This study applied the same procedure to recombinant FIX (rFIX), i.e. population PK modelling and the use of a simplified (one-compartment) model to describe only the terminal part of the coagulation factor vs. time curve. Data from a previous study on rFIX in 56 patients (4–56 years, 18–133 kg) were used to define a three-compartment population PK model. The average FIX clearance was 8.4 mL h−1 kg−1. Selleck EGFR inhibitor Elimination half-life ranged between 14 and 27 h. Data obtained from 24 h after the infusion were found to define the terminal phase of FIX disposition. second Doses to produce a target trough FIX level (set at 0.01 IU mL−1) at 72 h predicted by the Bayesian analysis, with blood sampling at either 24, 48 and 72 h or at only 24 and 48 h, were within −40% to +67% of those predicted using the three-compartment model, and within −57% to +125% for targeting a level at 96 h. These errors were lower than the overall interindividual variance in dose requirements. As three-compartment models are needed to characterize the PK of both plasma-derived

FIX and rFIX, simplification to a one-compartment model is less straightforward than for FVIII, and the methodology should be investigated further before clinical application. Limited blood sampling and Bayesian analysis could still, however, be potentially useful for targeting rFIX trough levels during prophylaxis. “
“Summary.  Radiosynoviorthesis (RS) is an intra-articular injection of a radioactive colloid for the treatment of synovitis administered most often to patients with rheumatoid arthritis or haemophilia. Although highly cost-effective in comparison with surgical or arthroscopic synovectomy, the risk of cancer associated with this treatment is not well known. We evaluated the incidence of cancer in a group of patients treated with RS.

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