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.
Background Cross-sectional area (CSA) measurement of the ulnar nerve in the adult population by using ultrasonography (US) at elbow extension and flexion has previously been reported, but not much evidence showed a significant difference between elbow extension and flexion position. Purpose To compare the ulnar nerve CSA between elbow extension and flexion position. Methods A comparative cross-sectional study was conducted by involving 36 healthy adults with normally functioning ulnar nerve proven by Nerve Conduction Study (NCS) or Electroneurography. The ulnar nerve CSA was measured on each elbow by using US at the level of the medial epicondyle, 2 cm distal and 2 cm proximal from the medial epicondyle. Results The average ulnar nerve CSA at the medial epicondyle, 2 cm distal and proximal to the medial epicondyle at elbow extension respectively were 5.95 ± 0.74 mm2, 6.27 ± 0.92 mm2, and 5.92 ± 0.73 mm2. At elbow flexion, the average ulnar nerve CSA at the positions was 5.70 ± 0.83 mm2, 5.23 ± 0.87 mm2, dan 5.73 ± 0.71 mm2 respectively. The CSA of the ulnar nerve at elbow extension was significantly larger compared to the flexion position in the three areas observed in this study (p < 0.001). Conclusion The CSA of the ulnar nerve at elbow extension position was larger compared to the flexion position. Elbow position should be considered in measuring CSA of the ulnar nerve.
Lipoblastoma is a benign lesion of immature fat cells that is found almost exclusively in pediatric population. This tumor is a rare tumor that occurs in infancy and early childhood, accounting for less than 1% of all childhood neoplasm. It is more common in male than in female and often presents as an asymptomatic, rapidly enlarging, soft lobular mass on the extremity. Although benign, it gives great difficulty in its management, due to its extensions into different facial planes, especially in lipoblastomatosis. Thus, complete surgical excision is the treatment of choice.
Background Repeated bleeding in hemophilic arthropathy (HA) may result in severe degenerative changes and joint destruction. The gradient-recalled echo (GRE) sequence MR is proved to be the best method to detect hemosiderin deposition. However, MR is not widely available in developing countries, including Indonesia. Some studies have proposed ultrasonography (US) as an alternative tool in evaluating hemophilic joint. However, there is still some disagreement on the ability of US to detect hemosiderin deposition. Objective To evaluate the association between US and GRE-sequence MR imaging in detecting hemosiderin deposition in hemophilic ankle joint. Material and methods A total of 102 sites from 17 ankle joints of 11 boys with severe hemophilia A underwent US examination using a high-frequency linear array transducer. GRE-sequence MR examination was performed in sagittal view consistent with the sites scanned by US. Both examinations were performed on the same day, but MR interpretation was performed blindly at different times. The association between US and GRE-sequences in detecting hemosiderin deposition was analyzed using McNemar’s test. Results Statistical analysis showed a significant association (p value < 0.001) between US and GRE MR in detecting hemosiderin deposition, but the association is weak (R = 0.26). Sensitivity and specificity of US for detecting hemosiderin deposition were 46.84% (95%CI: 35.51–58.40) and 95.65% (95%CI: 78.05–99.89), respectively, with positive predictive value 97.37% (95%CI: 84.29–99.61), negative predictive value 34.38% (95%CI: 29.50–39.60) and accuracy 57.84% (95%CI: 47.66–67.56). Conclusion There was a weak association between US and GRE-sequences in detecting hemosiderin deposition of hemophilic ankle joint.
Introduction : Magnetic Resonance (MR) imaging using gadolinium contrast media is an essential imaging modality in diagnosing spondylitis. However, gadolinium contrast is not widely available in Indonesia and relatively expensive. Many MR studies in Indonesia are performed without contrast administration. It is unclear how confident non-contrast MR can diagnose tuberculous spondylitis in comparison to standard contrast MR. Purposes : This study aims to evaluate the concordance between the contrast MR and non-contrast spine MR in diagnosing tuberculous spondylitis. We also evaluate the interobserver agreement between the general radiologist and musculoskeletal radiologist in interpreting non-contrast MR of spondylitis. Materials and Methods : A cross-sectional study using secondary data was performed to evaluate the concordance between the MR results regarding the usage of contrast media in diagnosing spondylitis. The inclusion criteria were patients over 17 years old who underwent complete sequences of contrast-enhanced MR examination of the spine, referred to radiology with the clinical diagnosis of suspected tuberculous spondylitis, spondylodiscitis, or both. All of the non-contrast and contrast-enhanced MR results were read and interpreted by two independent observers, a musculoskeletal radiologist and a general radiologist, blindly. The interobserver agreement analysis of the MR examination was conducted using Kappa and McNemar test. Results : There was no significant difference between the contrast and non-contrast MR in diagnosing spondylitis ( P= 0.368) and no significant difference in the interpretation of MR between the first and the second observer ( P = 0.343). The concordance between the contrast and non-contrast spine MR in diagnosing spondylitis (R: 0.88, P < 0.001) and the interpretation of MR between both observers (R: 0.65, P < 0.001) were showed in this study. Conclusion : There is a high concordance between the contrast and non-contrast MR in diagnosing tuberculous spondylitis. Although contrast MR is preferred as the standard imaging method of spondylitis, in case gadolinium contrast is unavailable, non-contrast MR can still provide valuable information in diagnosing spondylitis.
Background: Tuberculous septic arthritis is an infection that occurs inside the joint or synovial fluid and joint tissues caused by Mycobacterium tuberculosis. It may show wide variability of clinical symptoms and imaging appearance, ranging from asymptomatic with a normal radiographic examination to severe joint pain along with joint destruction, osteomyelitis, and abscess formation. This article presents radiographic and MR imaging appearance from a case of tuberculous septic arthritis with large abscess formation mimicking soft tissue tumor. Case presentation: We reported a 32-year-old female with a slowly enlarging lump on her right proximal thigh within the last 4 months along with slowly progressing joint pain. Both radiographic and MR images showed destruction of the femoral head and acetabular roof, with a formation of large rim-enhanced abscess that extending superficially and distally until mid-thigh. The patient underwent open drainage surgery and excisional biopsy. Histopathological examination showed chronic granulomatous inflammation caused by tuberculous infection. Conclusion: MR imaging combined with radiographic and clinical information played a very important role in the diagnosis of tuberculous septic arthritis with abscess, and to differentiate it from soft tissue neoplasms.
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