Prophylaxis for severe XXXX : experience from Europe and the United States.

@entity1 @entity2720 @entity548 @entity447
Since the late 1950s, prophylactic therapy has been used in @entity1 with severe @entity447 in order to convert their phenotype into the phenotype of moderate @entity447 . In the early years, prophylaxis was mostly started after the development of @entity2720 . This secondary prophylaxis decreased the number of @entity548 but did not halt the development of @entity2720 . With the start of primary prophylaxis, however, it was shown that both joint @entity548 and @entity2720 could be prevented. Although its benefits have been proved, prophylaxis has been used as standard treatment for @entity1 in only few countries. There are now favorable results with prophylaxis on @entity548 and @entity2720 with more than 25 years of follow-up. In general, the strategies used for prophylaxis can be divided into those that are targeted at preinfusion clotting factor levels of > 1% and those that are guided by clinical @entity548 patterns of individual @entity1 . In both types of strategies, treatment should be started early, before the start of joint damage. However, clotting factor consumption in the first group of strategies is expected to be higher, whereas @entity548 frequencies are expected to be slightly lower. The differences in long-term outcome between both strategies may only become apparent after more than two decades. In addition, some @entity1 may be able to stop taking prophylaxis in adulthood while maintaining a low @entity548 frequency. In conclusion, although the optimal prophylactic regimen is still under debate, it is well established that prophylaxis reduces or even prevents joint @entity548 and @entity2720 . Therefore, prophylaxis should be offered to all @entity1 with severe @entity447 , at least until adulthood.
