Introduction Prophylaxis has commonly become standard treatment for severe haemophilia patients. The World Federation of Hemophilia (WFH) recommends low‐dose prophylaxis in countries with resource constraints. Objective To determine efficacy and safety of low‐dose factor VIII (FVIII) tertiary prophylaxis compared to on‐demand treatment in severe haemophilia A children in Indonesia. Methods Eligible patients were randomly assigned to prophylaxis and on‐demand groups. Patients in the prophylaxis group received infusion of FVIII 10 IU/kg body weight, two times per week. Primary outcomes were the numbers of joint bleeding and total bleeding episodes; secondary outcomes were evidence of FVIII inhibitor, Hemophilia Joint Health Score (HJHS) and Hemophilia Early Arthropathy Detection Ultrasound (HEAD‐US) score. Patients were monitored for 12 months. Results Fifty patients, all with tertiary prophylaxis, 4‐18 years of age, were randomized into prophylaxis (n = 25) and on‐demand (n = 25) groups. The mean follow‐up time was 12.8 ± 0.86 vs 12.3 ± 0.54 months, respectively. Numbers of total and joint bleeding episodes were significantly lower in the prophylaxis group (P < 0.001, 95% CI −24.6;−10.7 and P < 0.001, 95% CI −14;−3, respectively). The prophylaxis group showed improvement of joint function (P = 0.004; CI 95% −3;−0.5); on the contrary, we found deterioration in the on‐demand group (P = 0.001; CI 95% 1;3). HEAD‐US scores showed improvement at month 6 in the prophylaxis group, but there was no significant difference between groups at month 12. Conclusion Low‐dose FVIII tertiary prophylaxis was effective in reducing joint bleeding episodes and improvement of HJHS compared to on‐demand FVIII treatment in severe haemophilia A children.
Background Hemophilic arthropathy, a condition manifested as joint destruction due to spontaneous joint bleeding, is one complication of hemophiliac patients. Early detection and intervention may improve the outcome, in which ultrasonography can be an ideal modality with the introduction of HEAD-US (Hemophilia Early Arthropathy Detection with Ultrasound) protocol. Studies have shown US benefit in hemophiliac patients, including its potential as an alternative for the Hemophiliac Joint Health Score (HJHS) system. However, many of the studies were conducted in countries with better management of hemophilia using prophylaxis treatment. It is unclear whether HEAD-US has a correlation with HJHS in countries using episodic treatment only, like in Indonesia. Purpose This study aimed to explore the correlation between HEAD-US and HJHS in hemophiliac patients with joint problems in Indonesia. Materials and methods A cross-sectional correlation study between HEAD-US and HJHS was performed with primary data collected from 120 hemophilic patients. US examination was performed on elbow, knee and ankle joints using the HEAD-US scoring method by a musculoskeletal radiologist. HJHS examination was conducted by a trained physiotherapist and a medical rehabilitation specialist. All examiner is member of multidisciplinary Hemophiliac Management Team in Cipto Mangunkusumo General Hospital in Jakarta, Indonesia. Results The mean age of the participant was 9.3 (5–14) years old. The median score of HEAD-US was 8 (1–28) with most of the joint abnormalities found on the ankles. The median score of HJHS was 3 (0–35), with most joint abnormalities found on the knees. There was a moderate correlation between HEAD-US and HJHS score (p < 0.05, r = 0.65). Conclusion HEAD-US shows a moderate correlation to HJHS in hemophiliac patients who received episodic treatment. HEAD-US can provide additional value in the anatomical evaluation of the joint and could be complementary to HJHS in assessing the joint status in hemophilic patient
Background and objectives Fat infiltration of multifidus muscle is an important parameter to assess the efficacy of spinal stabilization training in chronic low back pain (LBP) patients. As a CT scan shows a specific attenuation value for fat, it can be used as a ratio to evaluate fat infiltration of the muscle relative to its cross-sectional area. This study aims to compare the fat infiltration ratio of multifidus muscle between subjects with and without chronic LBP in Indonesia. Methods Comparative cross-sectional study of 20 subjects with chronic LBP and 20 subjects without LBP. Fat infiltration ratio calculation of the multifidus muscle was obtained from the database of abdominal CT at the level of the superior and inferior endplate of L4 and L5 vertebral body. Results The fat infiltration ratio of multifidus muscle in the chronic LBP subjects group was significantly higher than the subjects group without NPB (p < 0.05). The cut-off value of the fat infiltration ratio at the level of the inferior endplate of L4 was 0.125 (75 % sensitivity and 80 % specificity). Conclusion The calculation of the multifidus muscle fat infiltration ratio at the inferior endplate L4 using CT is a potential method to evaluate multifidus muscle quality in chronic LBP patients.
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