Clinical and ultrasound joint outcomes in severe hemophilia A children receiving episodic treatment in Indonesian National Hemophilia Treatment Center

Sari, Teny Tjitra; Chozie, Novie Amelia; Gatot, Djajadiman; Tulaar, Angela Bm; Dharma, Rahayuningsih; Sukrisman, Lugyanti; Bardosono, Saptawati; Achmad, Harry Raspati; Prasetyo, Marcel

doi:10.13181/mji.v26i1.1494

Cited by 3 publications

(4 citation statements)

References 19 publications

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“…In the current study, the mean HJHS was 16.8 ± 12.8. We found a nonstatistically association between HJHS and the presentation age; opposite to this finding, an Indonesian study by Sari et al 17 observed that children with severe hemophilia who were receiving episodic treatment evaluated by HJHS and HEAD-US scoring show more severe joint damage in older children. In the present study, when the HJHS was plotted against the age of the patients, we found a nonstatistically significant linear correlation between them.…”

Section: Discussioncontrasting

confidence: 99%

“…Ghany et al 9 also reported a nonstatistically significant correlation between FISH and age. Those findings are opposite to the reported in a similar study where FISH had a good connection with the age of patients with severe ( r =−0.63, P =0.01) and moderate hemophilia ( r =−0.55, P =0.02) 17 . We found that by increasing the number of affected joints, the lower the FISH score; the FISH score was significantly negatively correlated with the number of affected joints ( r =−0.352, P =0.012).…”

Section: Discussioncontrasting

confidence: 99%

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Hemophilia Joint Health Score, Functional Independence Score in Hemophilia, and Pettersson Score in Pediatric Patients With Severe Hemophilia A

Mabrouk

Abbas

Ezzat

et al. 2023

Journal of Pediatric Hematology/Oncology

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Hemophilia is an X-linked recessive disorder. Children with hemophilia go through spontaneous and trauma-provoked bleeding. Recurring joint bleeds lead to ongoing incapacity. Achieving healthy joints is the primary target of hemophilia management. The current study objective was to assess hemophilic joints in individuals with hemophilic arthropathy clinically, radiographically, and functionally. This cross-sectional study included 50 children with severe hemophilia A who were selected from the pediatric hematology clinic. All children were assessed for Hemophilia Joint Health Score (HJHS). Joint assessed functionally by Functional Independence Score in Hemophilia (FISH) and radiologically by plain radiograph and scored by the Pettersson scoring system. Data were analyzed using Statistical Package for Social Sciences. The mean age of the studied cases of hemophilia was 8.5±3.1 years. The mean FISH score among the studied patients was 26.8±4.2, the mean HJHS was 16.8±12.8, and the Pettersson score was 4.9±2.7. The number of affected joints showed a significant negative correlation to the FISH score and a significant positive correlation to HJHS. The frequency of hemarthrosis/month showed a significant positive correlation to HJHS. The number of affected joints showed a significant negative correlation to the FISH score and a significant positive correlation to HJHS. Frequency of hemarthrosis/month showed a significant positive correlation to HJHS.

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Section: Discussioncontrasting

confidence: 99%

Section: Discussioncontrasting

confidence: 99%

Hemophilia Joint Health Score, Functional Independence Score in Hemophilia, and Pettersson Score in Pediatric Patients With Severe Hemophilia A

Mabrouk

Abbas

Ezzat

et al. 2023

Journal of Pediatric Hematology/Oncology

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“…Repeated joint bleeding in haemophilia often leads to joint deformity and dysfunction, which seriously affects the quality of life of patients ( 9 ), and with age, these lesions become more apparent ( 10 ). US has recently attracted growing interest as a potential tool to assess joint status and identify early arthropathic changes in haemophilia patients, which can ensure initiation of treatment as soon as possible and thereby prevent joint damage ( 11 – 13 ).…”

Section: Discussionmentioning

confidence: 99%

Comparison of joint status using ultrasound assessments and Haemophilia Joint Health Score 2.1 in children with haemophilia

Wang

Pan

et al. 2023

Front. Med.

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IntroductionUltrasound (US) has gained popularity in the evaluation of haemophilic joint diseases because it enables the imaging of soft-tissue lesions in the joints and bone-cartilage lesions. We aimed to determine the correlation between US evaluations and clinical assessments performed using HJHS 2.1 and to evaluate their respective characteristics in assessing early haemophilic arthropathy.MethodsA total of 178 joints (32 knees, 85 elbows, and 61 ankles) in 45 haemophilia A patients (median age, 10 years; range, 6–15) were assessed using US and HJHS 2.1. Ultrasonographic scoring was performed in consensus assessments by one imager by using the US scores.ResultsThe total HJHS 2.1 and US scores showed a strong correlation (rS=0.651, P=0.000, CI: 0.553–0.763), with an excellent correlation for the elbows (rS=0.867, P=0.000, CI: 0.709–0.941) and a substantial correlation for the knees (rS=0.681, P=0.000, CI: 0.527–0.797). The correlation for the ankles was relatively moderate (rS=0.518, P=0.000, CI: 0.308–0.705). Nine subjects (15.5%) without abnormalities, as indicated by HJHS 2.1, showed haemophilic arthropathy in US scoring. All nine joints showed moderate (1/9) to severe (8/9) synovial thickening in the ankle (5/9) and elbow joints (4/9). In contrast, 50 joints (50.5%) showed normal US scores and abnormal changes as indicated by HJHS 2.1. S scores correlated well with HJHS 2.1 for overall and individual joints.DiscussionUS could identify some early pathological changes in joints showing normal clinical findings, but still cannot replace the HJHS; however, it can serve as an imaging examination complementing HJHS 2.

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“…However, this study was conducted on hemophilia patients who had received prophylaxis treatment. In Indonesia, most hemophiliac patients receive episodic treatment, due to financial challenges and unavailability of prophylaxis treatment [ 14 , 15 ]. Therefore, many pediatric hemophiliac patients have moderate to severe joint problems.…”

Section: Introductionmentioning

confidence: 99%

Correlation between Hemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) score and Hemophilia Joint Health Score (HJHS) in patients with hemophilic arthropathy

et al. 2021

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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

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Clinical and ultrasound joint outcomes in severe hemophilia A children receiving episodic treatment in Indonesian National Hemophilia Treatment Center

Cited by 3 publications

References 19 publications

Hemophilia Joint Health Score, Functional Independence Score in Hemophilia, and Pettersson Score in Pediatric Patients With Severe Hemophilia A

Hemophilia Joint Health Score, Functional Independence Score in Hemophilia, and Pettersson Score in Pediatric Patients With Severe Hemophilia A

Comparison of joint status using ultrasound assessments and Haemophilia Joint Health Score 2.1 in children with haemophilia

Correlation between Hemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) score and Hemophilia Joint Health Score (HJHS) in patients with hemophilic arthropathy

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