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"Latar belakang: Perdarahan sendi berulang merupakan morbiditas utama pada pasien hemofilia karena dapat menimbulkan artropati hemofilik yang menyebabkan keterbatasan gerak dan disabilitas sehingga menurunkan kualitas hidup. Penelitian bertujuan mengetahui korelasi pemeriksaan klinis sendi, penilaian aktivitas fungsional dan kualitas hidup pada anak hemofilia.Metode: Penelitian dengan desain potong lintang di RSCM pada Agustus−November 2022 pada anak 4−16 tahun, hemofilia A atau B derajat sedang atau berat yang mengalami perdarahan sendi berulang. Penelitian dilakukan dengan menilai HJHS, PedHALshort serta Haemo-QoL dan mencari korelasi skor HJHS dengan skor PedHALshort dan Haemo-QoL.Hasil: Sebanyak 95 subyek hemofilia, dengan hemofilia A (77,3%) dan 70,1% hemofilia berat. Skor HJHS median 4 (1−9), skor PedHALshort median 74,5 (62,73-89,09), skor Haemo-QoL mean (SD) 74,51 (15,58). Skor HJHS berkorelasi negatif sedang dengan PedHALshort (r= -0,462, p< 0,0001), skor HJHS berkorelasi sedang dengan Haemo-QoL (r= 0,469, p< 0,001).Simpulan: Semakin tinggi skor HJHS menunjukkan adanya kerusakan pada sendi maka semakin rendah skor PedHALshort dan semakin tinggi skor Haemo-QoL yang menunjukkan semakin terganggu aktivitas fungsional serta kualitas hidupnya.......Background: Recurrent joint bleeding is the major morbidity in patient with hemophilia that can cause hemophilic arthropathy causes limitation of daily activities, disability, and reducing quality of life. Research objective are to determine the relationship between the clinical evaluation of joints, the assessment of functional activity determined and assessment of the quality of life with HJHS, so we can diagnose arthropathy, prevent disability and better management.Methods: Study with cross-sectional design at RSCM on August-November 2022, children aged 4-16, with moderate or severe hemophilia A and B with recurrent joint bleeding. The study was conducted by assessing HJHS, PedHALshort and Haemo-QoL, determine the relationship between HJHS with PedHALshort and Haemo-QoL score.Result: A total of 95 hemophilia subjects, with hemophilia A (77.3%) and 70.1% severe hemophilia. HJHS median score 4 (1-9), PedHALshort median score 74.5 (62.73-89.09), Haemo-QoL mean (SD) 74.51 (15.58). The HJHS score had a moderate negative correlation with PedHALshort (r= -0.462, p<0.0001), the HJHS score had a moderate correlation with Haemo-QoL (r= 0.469, p<0.001).Conclusion: The higher of HJHS score indicates a joint disorder, the lower of PedHALshort and the higher of Haemo-QoL indicates the more impaired functional activity and poorer quality of life."

"Latar belakang: Evaluasi sendi pada penyandang hemofilia memerlukan metode yang objektif dan terukur. USG sebagai metode yang relatif baru untuk artropati hemofilik AH belum memiliki konsensus sistem skor, sementara MRI telah memiliki sistem skor International Prophylaxis Study Group dari World Federation of Hemophilia IPSG-WFH . Penelitian ini mengembangkan sistem skor USG baru untuk artropati hemofilik lutut tahap dini, dan menilai keselarasannya dengan skor MRI IPSG-WFH dan kadar CTX-II urin.Metode: Penelitian ini menggunakan desain potong lintang. Formulasi skor USG berdasarkan pada studi pustaka terhadap 25 publikasi terkait sejak tahun 1999 - 2015 dan peer review. Terdapat 27 anak penyandang hemofilia A berat yang dipilih secara konsekutif. AH lutut tahap dini ditetapkan berdasarkan klasifikasi radiografi Arnold-Hilgartner derajat 0 - II. USG dan MRI lutut dilakukan dengan penilaian skor MRI IPSG-WFH dan skor USG yang baru. Kadar CTX-II urin ditetapkan dengan pemeriksaan ELISA. Data dianalisis dengan uji Spearman.Hasil: Sistem skor USG baru meliputi komponen efusi sendi, hipertrofi sinovium, hipervaskularisasi sinovium dengan Power Doppler, deposisi hemosiderin, dan kerusakan kartilago pada troklea femoris. Terdapat korelasi sedang antara skor USG dengan skor MRI. Tidak ada korelasi skor USG dengan CTX-II urin.Kesimpulan: Skor US baru ini dapat digunakan sebagai alternatif MRI pada AH lutut tahap dini.

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Introduction Assessment of knee haemophilic arthropathy HA required an objective measures. There was no consensus on preferrable US scoring system, while MRI already had a scoring system developed by the International Prophylaxis Study Group of the World Federation of Hemophilia IPSG WFH. This study developed a new US scoring system for early knee HA and its association with MRI scoring system and urinary CTX II level.Method The study was cross sectional. US scoring system was developed based on literature studies of 25 publications between 1999 - 2015 and peer review. Twenty seven children with severe haemophilia A was recruited consecutively. Early HA was confirmed by radiography as Arnold Hilgartner stage 0 - II. Knee MRI and US were scored using MRI IPSG WFH scoring system and the new US scoring system, while urinary CTX II level was measured using ELISA. Correlation was analyzed using Spearman test.Results US scoring system included joint effusion, synovial hypertrophy, synovial hypervascularization using Power Doppler, hemosiderin deposition, and cartilage damage. Moderate correlation was found between US score and MRI score. There was no correlation between US score and urinary CTX II level.Conclusion The new US score can be used as an alternative for MRI in the assessment of early knee HA."

"ABSTRAKLatar belakang dan tujuan: Artropati hemofilik merupakan faktor utama peningkatan morbiditas dan besarnya biaya pengobatan penderita hemofilia. Radiografi berperan dalam mengevaluasi progresifitas kelainan dan efek terapi yang diberikan. Penelitian ini bertujuan untuk mengurangi jumlah proyeksi dalam evaluasi artropati hemofilik lutut sehingga dapat mengurangi radiasi yang diterima pasien, sesuai dengan prinsip ldquo;As Low As Reasonably Achievable rdquo;.Metode: Uji korelatif dan asosiatif menggunakan desain potong lintang terhadap radiogram 100 lutut penderita hemofilia proyeksi AP dan lateral dengan kalsifikasi Arnold Hilgartner dan skor Pettersson.Hasil: Terdapat hubungan evaluasi artropati hemofilik lutut menggunakan kalsifikasi Arnold Hilgartner antara proyeksi AP dan AP-lateral dengan R=0,52 dan nilai

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ABSTRACTBackground and Objective Hemophilic arthropathy is a main factor which causes morbidity and is the main contributor to cost in treatment of hemophilic patients. Radiography has a role in monitoring progressivity and follow up after therapy. The aim of the study is to reduce radiography proections to minimize radiation dose in accordance with ldquo As Low As Reasonably Achievable rdquo radiology principle. Methods A correlative and associative research using cross sectional study from 100 knees radiogram of hemophilic arthropathy patients in AP and lateral view using Arnold Hilgartner and Petterson score. Results There is association between assessment of hemophilic arthropathy using Arnold Hilgartner in AP view and Ap lateral view with R 0.52 and p 0.001. There is positive correlation between evaluation of hemophilic arthropathy using Petterson score using AP and AP lateral with R 0.58 and p"