[Effect of FVIII doses on joint structure and function in adolescents with severe hemophilia A: mid-term results of a prospective cohort study].

Nan fang yi ke da xue xue bao = Journal of Southern Medical University
L MengJ Sun

Abstract

To explore the clinical phenotype and changes in joint structure and function in adolescent patients with severe hemophilia A under different doses of FVIII. Forty- three adolescents with severe hemophilia A aged 4-18 years were divided into on-demand group (n=7), low-dose group (FVIII dose of 10-15 U/kg, 2-3 times a week, and ≤30 U/kg a week; n=17), and intermediate-dose group (FVIII dose of 15-20 U/kg, 2-3 times a week, and 45-60 U/kg a week (n=19). The 3 groups were compared for their clinical bleeding phenotype, annual bleeding rate (ABR), annual joint bleeding rate (AJBR), annual the most severe joint bleeding rate, joint imaging scores (ultrasound HEAD-US score and IPSG MRI score), Hemophilia Joint Health Score (HJHS) and Functional Independence Score in Hemophilia (FISH) within 24 months. Compared with that in on-demand group, the ABR was significantly reduced in the low- and intermediate-dose groups (P=0.004 and 0.000, respectively), and was reduced by 32.87% in the intermediate-dose group as compared with the low-dose group. The AJBR (P < 0.01) and annual the most severe joint bleeding rate (P < 0.05) also differed significantly among the 3 groups. The number of bleeding episodes increased progressively with time in th...Continue Reading

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