The effects of postponing prophylactic treatment on long-term outcome in XXXX with severe @entity447 .

@entity2720 @entity1 @entity548 @entity447
To prevent @entity2720 , prophylactic treatment of @entity1 with severe @entity447 should be started before joint damage has occurred. However, treatment is expensive, and the burden of regular venipunctures in young @entity1 is high. With the aim of providing information on starting prophylaxis on the basis of individual @entity1 characteristics, the effect of postponing prophylaxis on long-term @entity2720 was studied in a cohort of 76 @entity1 with severe @entity447 born between 1965 and 1985. The median age at first @entity548 was 2.2 years (range, 0.2-5.8). Prophylaxis was started at a median age of 6 years (interquartile range [IQR], 4-9), and the median annual clotting factor use on prophylaxis was 1750 IU/kg/y (31 IU/kg/wk). @entity2720 was measured by the Pettersson score (maximum, 78 points). At a median age of 19 years, the median Pettersson score was 7 points (IQR, 0-17). After 2 decades of follow-up, the Pettersson score was 8% higher (95% confidence interval, 1%-16%) for every year prophylaxis was postponed after the first @entity548 . This effect was independent of age at Pettersson score, age at first @entity548 , and prophylactic dose used. In conclusion, most @entity1 have their first @entity548 after the age of 2 years. @entity1 who start prophylaxis soon after the first @entity548 @entity2720 in adulthood. The longer the start of prophylaxis is postponed after the first @entity548 , the higher the risk of developing @entity2720 .
