Clinical and ultrasound joint outcomes in severe hemophilia A children receiving episodic treatment in Indonesian National Hemophilia Treatment Center
Abstract: Recurrent joint
bleeds leading to arthropathy is the main problem in severe hemophilia
children. This study aimed to investigate joint status in severe hemophilia A
children receiving episodic treatment in Cipto Mangunkusumo Hospital, Jakarta.
Methods: A cross-sectional study was conducted in Cipto Mangunkusumo
Hospital as Indonesian National Hemophilia Treatment Center on children (4–18
years) with severe hemophilia A, who previously received episodic treatment,
with no history of inhibitor factor VIII. Hemophilia Joint Health Score was
evaluated according to HJHS version 2.1 2011. Joint ultrasonography was done
for six index joints (bilateral elbows, knees and ankles) using Haemophilia
Early Arthropathy Detection with Ultrasound (HEAD-US) methods. Data of age of
first joint bleed, number of target joints and inhibitor factor VIII were
obtained from the Pediatric Hemophilia Registry and medical records.
Results: There were 59 subjects aged 4 to 18 years. Twenty-nine out of 59
(49.2%) subjects experienced first joint bleed before of 2 years of age. The
most common of joint bleeds was a right ankle. Mean total HJHS was 8.71±8.73.
Subjects aged 4–10 years showed lower HJHS (4.6±3.7) as compared to subjects
aged >10–18 years (12.3±10.3), p<0.001; 95% CI=4.9–13. Mean HEAD-US
scores in subjects aged 4–10 years (18.7±5.6) was lower than in subjects aged
>10–18 years (28±7.9), p<0.001, 95% CI= -12.9–-5.6.
Conclusion: HJHS and HEAD-US scores of severe hemophilia A children
receiving episodic treatment aged 4–10 years are lower compared to subjects
aged >10–18 years, indicating more severe joint destruction in older
children and progressivity of joint damage over time. It is important to start
prophylactic treatment to prevent progressivity of joint damage.
Keywords: arthropathy;
HEAD-US; hemophilia; HJHS; inhibitor factor VIII
Author: Teny T. Sari, Novie A.
Chozie, Djajadiman Gatot, Angela B.M. Tulaar, Rahayuningsih Dharma, Lugyanti
Sukrisman, Saptawati Bardosono, Harry R. Achmad, Marcel Prasetyo
Journal Code: jpkedokterangg170196
