Nan Fang Yi Ke Da Xue Xue Bao 2021 Apr;41(4):536-542
Department of Hematology, Nanfang Hospital, Southern Medical University, Guangzhou 510515, China.
Objective: To explore the clinical phenotype and changes in joint structure and function in adolescent patients with severe hemophilia A under different doses of FVIII.
Objective: Forty- three adolescents with severe hemophilia A aged 4-18 years were divided into on-demand group (=7), low-dose group (FVIII dose of 10-15 U/kg, 2-3 times a week, and ≤30 U/kg a week; =17), and intermediate-dose group (FVIII dose of 15-20 U/kg, 2-3 times a week, and 45-60 U/kg a week (=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. Read More