Turkiye Klinikleri Journal of Medical Sciences

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Joint by Joint Correlations Between Ultrasound HEAD-US Scores and HJHS Joint Scores in Hemophilia A Patients: A National, Multi-Center, Prospective, and Cross-Sectional Study
Hemofili A Hastalarında Ultrason HEAD-US ve HJHS Eklem Skorları Arasındaki Korelasyon: Ulusal, Çok Merkezli, Prospektif ve Kesitsel Bir Çalışma
Ramazan Kaan KAVAKLIa, İpek TAMSELb, Can BALKANa, Süleyman Emre KARAÜZÜMa, Ali Bülent ANTMENc, Tuğana AKBAŞd, Hatice İlgen ŞAŞMAZc, Şerife Şebnem ÖNEN GÖKTEPEe, Sinan GENÇf, Dilek GÜRLEK GÖKÇEBAYg, Tülin HAKAN DEMİRKANh, Şevkiye Selin AYTAÇ EYÜPOĞLUi, Adalet Elçin YILDIZj, Ekrem ÜNALk, Ayşenur PAÇ KISAARSLANl, Osman Bülent ZÜLFİKARm, İnci KIZILDAĞ YIRGINn, Sayime Başak KOÇ ŞENOLm, Canan ALBAYRAKo, Aysu Başak ÖZBALCIp, Zühre KAYAr, Öznur Leman BOYUNAĞAs, Ayşegül ÜNÜVARt, Mesut BULAKÇIu, Nazan SARPERv, Yonca ANIKy
aEge University Children's Hospital, Clinic of Pediatric Hematology, İzmir, Türkiye
bEge University Faculty of Medicine Hospital, Clinic of Radiology, İzmir, Türkiye
cAcıbadem Adana Hospital, Clinic of Pediatric Hematology, Adana, Türkiye
dAcıbadem Adana Hospital, Clinic of Radiology, Adana, Türkiye
eBehçet Uz Children's Hospital, Clinic of Pediatric Hematology, İzmir, Türkiye
fBehçet Uz Children's Hospital, Clinic of Radiology, İzmir, Türkiye
gAnkara City Hospital, Clinic of Pediatric Hematology, Ankara, Türkiye
hAnkara City Hospital, Clinic of Radiology, Ankara, Türkiye
iHacettepe University Children's Hospital, Clinic of Pediatric Hematology, Ankara, Türkiye
jHacettepe University Faculty of Medicine Hospital, Clinic of Radiology, Ankara, Türkiye
kErciyes University Hospital, Clinic of Pediatric Hematology, Kayseri, Türkiye
lErciyes University Hospital, Clinic of Radiology, Kayseri, Türkiye
mİstanbul University Institute of Oncology, Unit of Hereditary Bleeding Disorders, İstanbul, Türkiye
nİstanbul University Institute of Oncology, Department of Radiology, İstanbul, Türkiye
oOndokuz Mayıs University Children's Hospital, Clinic of Pediatric Hematology, Samsun, Türkiye
pOndokuz Mayıs University Hospital, Clinic of Radiology, Samsun, Türkiye
rGazi University Hospital, Clinic of Pediatric Hematology, Ankara, Türkiye
sGazi University Hospital, Clinic of Radiology, Ankara, Türkiye
tİstanbul University Hospital, Clinic of Pediatric Hematology, İstanbul, Türkiye
uİstanbul University Hospital, Clinic of Radiology, İstanbul, Türkiye
vKocaeli University Hospital, Clinic of Pediatric Hematology, Kocaeli, Türkiye
yKocaeli University Hospital, Clinic of Radiology, Kocaeli, Türkiye
Turkiye Klinikleri J Med Sci. 2024;44(3):97-104
doi: 10.5336/medsci.2024-101178
Article Language: EN
Full Text
ABSTRACT
 Objective: Hemophilia Joint Health Score (HJHS) and Hemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) Score are indispensable tools for the routine evaluation of joint health in individuals with hemophilia A. This study aims to meticulously evaluate the joint-specific correlations between the HJHS and HEAD-US scoring systems in hemophilia A patients, focusing on the elbow, knee, and ankle joints. Material and Methods: A comprehensive national, multicenter, prospective, cross-sectional, non-interventional observational study was conducted across 19 medical centers in Türkiye, involving 192 hemophilia A patients over a two-year duration. The inclusion criteria encompassed patients aged ≥6 years with moderate or severe hemophilia A (FVIII<2%), comprising 63.5% pediatric and 36.5% adult patients. Results: In the analysis of 192 patients under prophylactic treatment, a robust correlation was identified between the HJHS and HEAD-US total scores across all age groups. Particularly noteworthy was the finding that adults exhibited a more pronounced correlation than their pediatric counterparts for total scores. When exploring the correlation among the six joints, it became apparent that elbows exhibited the highest correlation (r: 0.408/0.581), while ankles demonstrated the lowest correlation (r: 0.397/0.311). Furthermore, the study uncovered an intriguing insight: a significant difference in correlation rates between children and adults, adding a layer of complexity to the joint evaluation process. Conclusion: The HEAD-US and HJHS scoring systems serve as valuable monitoring tools in hemophilia A, offering complementary insights. Our findings highlight varying correlation rates among joints, emphasizing elbows as the most correlated joints.

Keywords: Hemophilia A; joint diseases; ultrasonography; prospective studies; correlation analysis
ÖZET
 Amaç: Hemofili Eklem Sağlığı Skoru [Hemophilia Joint Health Score (HJHS)] ve Hemofili Erken Artropati Taraması Ultrason [Hemophilia Early Arthropathy Detection with Ultrasound (HEAD-US)] Skoru, rutin uygulamada eklem sağlığını değerlendirmek için vazgeçilmez araçlardır. Bu çalışma, hemofili A hastalarında HJHS ve HEAD-US skorlama sistemlerinin eklem bazında değerlendirmesini yapmayı hedeflemiş olup özellikle dirsek, diz ve ayak bileği eklemlerindeki korelasyonun gücünü değerlendirmiştir. Gereç ve Yöntemler: Türkiye genelinde 19 merkezde gerçekleştirilen kapsamlı, ulusal, çok merkezli, prospektif, kesitsel, müdahalesiz gözlemsel bir çalışma olup 192 hemofili A hastasını içeren 2 yıllık bir dönemi kapsamıştır. FVIII<%2 olan orta ve ciddi hemofili A hastalarından, yaşları ≥6 olan hastalar çalışmaya dâhil edilmiş olup, bu hastaların %63,5'i pediatrik ve %36,5'i erişkin hastalardır. Bulgular: Profilaktik tedavi almakta olan 192 hastanın analizi, tüm yaş gruplarında HJHS ve HEADUS toplam skorları arasında önemli korelasyon olduğunu göstermiştir. Özellikle erişkinlerin toplam skorlarında çocuklardan daha güçlü korelasyon gözlemlenmesi dikkat çekicidir. Değerlendirilen altı eklem incelendiğinde, dirsekler en yüksek korelasyonu gösterirken (r: 0,408/0,581), ayak bilekleri en düşük korelasyonu göstermiştir (r: 0,397/0,311). Ayrıca, çocuklar ve erişkinler arasında korelasyon oranlarında belirgin bir fark bulunması, eklem değerlendirme sürecine karmaşıklık katmaktadır. Sonuç: HEAD-US ve HJHS skorlama sistemleri, hemofili A'da değerli izleme araçları olarak kullanılmakta ve birbirini tamamlayan bilgiler sunmaktadır. Bulgularımız, eklemler arasında değişen korelasyon oranlarını vurgulayarak dirseklerin en korele eklem olduğunu göstermektedir.

Anahtar Kelimeler: Hemofili A; eklem hastalıkları; ultrasonografi; prospektif çalışma; korelasyon analizi
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