.: ORİJİNAL ARAŞTIRMA
Investigation of the Relationship Between Hemophilia Joint Health Score and Haemophilia Early Arthropathy Detection with Ultrasound Score in Hemophilic Arthropathy
Hemofilik Artropatide Hemofili Eklem Sağlığı Skoru ve Hemofili Erken Artropati Taraması Ultrason Skoru Arasındaki İlişkinin Araştırılması
Turkiye Klinikleri J Med Sci. 2021;41(1):46-50
doi: 10.5336/medsci.2020-78772
Makale Dili: EN
Tam Metin
ABSTRACT
Objective: Hemophilia is classified according to the factor VIII or IX level as severe, moderate and mild. The Hemophilia Joint Health Score (HJHS) and Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) score used in recent years contribute significantly to the early diagnosis of arthropathy, which continues to be an important problem in severe hemophilia. It was aimed to investigate the compatibility between HJHS and HEAD-US scores. Material and Methods: The demographic and disease-related data, HJHS 2.1 and HEAD-US scores of the participants who participated in the Workshop of the Hemophilia Federation in 2020 were recorded. Results: The mean age of 32 participants was 20.6 (minimum: 15-maximum: 31). Twenty three were diagnosed as Hemophilia A, 8 were Hemophilia B, 1 was Von Willebrand disease. HJHS score of 30 patients was 6.7±6.2 (minimum: 0-maximum: 22). The mean of HEAD-US was 14.3±11.5 (minimum: 0-maximum: 34). HJHS and HEAD-US scores were compatible with each other (p=0.002). Arthropathy was present in 33 joints of 21 patients. There was a statistical significance between patients with and without arthropathy with both the HJHS and the HEAD-US scores (0.006 and 0.005 respectively). The most common arthropathy is in the right knee. HJHS and HEAD-US scores are compatible in both knee and elbow joints. Nonetheless, it is incompatible in both ankles. HJHS and HEAD-US scores were discordant in 21 joints of 7 patients. These joints were right ankle (n=7), left ankle (n=6), left elbow (n=4), right elbow (n=2) and right knee (n=2). Conclusion: A correlation was found between the HJHS and HEAD-US scores. HEAD-US is more sensitive than HJHS in detecting the early stage of arthropathy in the ankle joint. These scores should be done routinely to all patients for manifesting treatment deficiencies and incompatibilities.
Keywords: Hemophilia A; Hemophilia B; arthropathy
Objective: Hemophilia is classified according to the factor VIII or IX level as severe, moderate and mild. The Hemophilia Joint Health Score (HJHS) and Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) score used in recent years contribute significantly to the early diagnosis of arthropathy, which continues to be an important problem in severe hemophilia. It was aimed to investigate the compatibility between HJHS and HEAD-US scores. Material and Methods: The demographic and disease-related data, HJHS 2.1 and HEAD-US scores of the participants who participated in the Workshop of the Hemophilia Federation in 2020 were recorded. Results: The mean age of 32 participants was 20.6 (minimum: 15-maximum: 31). Twenty three were diagnosed as Hemophilia A, 8 were Hemophilia B, 1 was Von Willebrand disease. HJHS score of 30 patients was 6.7±6.2 (minimum: 0-maximum: 22). The mean of HEAD-US was 14.3±11.5 (minimum: 0-maximum: 34). HJHS and HEAD-US scores were compatible with each other (p=0.002). Arthropathy was present in 33 joints of 21 patients. There was a statistical significance between patients with and without arthropathy with both the HJHS and the HEAD-US scores (0.006 and 0.005 respectively). The most common arthropathy is in the right knee. HJHS and HEAD-US scores are compatible in both knee and elbow joints. Nonetheless, it is incompatible in both ankles. HJHS and HEAD-US scores were discordant in 21 joints of 7 patients. These joints were right ankle (n=7), left ankle (n=6), left elbow (n=4), right elbow (n=2) and right knee (n=2). Conclusion: A correlation was found between the HJHS and HEAD-US scores. HEAD-US is more sensitive than HJHS in detecting the early stage of arthropathy in the ankle joint. These scores should be done routinely to all patients for manifesting treatment deficiencies and incompatibilities.
Keywords: Hemophilia A; Hemophilia B; arthropathy
ÖZET
Amaç: Hemofili, faktör VIII veya IX düzeyine göre ağır, orta ve hafif olarak sınıflandırılır. Son yıllarda kullanılan, Hemofili Eklem Sağlığı Skoru [Hemophilia Joint Health Score (HJHS)] ve Hemofili Erken Artropati Taraması [Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US)], ultrason skoru ağır hemofilide önemli bir sorun olmaya devam eden artropatinin erken teşhisine önemli katkı sağlamaktadır. Bu çalışmada, HJHS ve HEAD-US skorları arasındaki uyumluluğun araştırılması amaçlanmıştır. Gereç ve Yöntemler: 2020 yılında Hemofili Federasyonu Çalıştayına katılan katılımcıların, demografik ve hastalık ilişkili verileri HJHS 2,1 ve HEAD-US puanları kaydedilmiştir. Bulgular: Otuz iki katılımcının ortalama yaşı 20,6 (minimum: 15-maksimum: 31) idi. Yirmi üçü Hemofili A, 8'i Hemofili B, 1'i Von Willebrand hastalığı tanılıydı. Otuz hastanın ortalama HJHS skoru 6,7 (minimum: 0-maksimum: 22) idi. Ortalama HEAD-US skoru 14,3 (minimum: 0-maksimum: 34) idi. HJHS ve HEAD-US skorları birbiriyle uyumluydu (p=0,002). Yirmi bir hastanın 33 ekleminde artropati mevcuttu. Hem HJHS hem de HEAD-US skorları ile (sırasıyla p=0,006 ve p=0,005) artropatisi olan ve olmayan hastalar arasında, istatistiksel anlamlılık vardı. Artropati en sık sağ dizdeydi. HJHS ve HEAD-US skorları, hem diz hem de dirsek eklemlerinde uyumlu, her 2 ayak bileğinde ise uyumsuzdu. Yedi hastanın 21 ekleminde HJHS ve HEAD-US skorları uyumsuzdu. Bu eklemler; sağ ayak bileği (n=7), sol ayak bileği (n=6), sol dirsek (n=4), sağ dirsek (n=2) ve sağ diz (n=2) idi. Sonuç: HJHS ve HEAD-US skorları arasında bir korelasyon saptanmıştır. HEAD-US, ayak bileği ekleminde artropatinin erken evresini tespit etmede HJHS'den daha hassastır. Bu skorlar, tedavi eksikliklerinin ve uyumsuzluklarının ortaya çıkması için tüm hastalara rutin olarak uygulanmalıdır.
Anahtar Kelimeler: Hemofili A; Hemofili B; artropati
Amaç: Hemofili, faktör VIII veya IX düzeyine göre ağır, orta ve hafif olarak sınıflandırılır. Son yıllarda kullanılan, Hemofili Eklem Sağlığı Skoru [Hemophilia Joint Health Score (HJHS)] ve Hemofili Erken Artropati Taraması [Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US)], ultrason skoru ağır hemofilide önemli bir sorun olmaya devam eden artropatinin erken teşhisine önemli katkı sağlamaktadır. Bu çalışmada, HJHS ve HEAD-US skorları arasındaki uyumluluğun araştırılması amaçlanmıştır. Gereç ve Yöntemler: 2020 yılında Hemofili Federasyonu Çalıştayına katılan katılımcıların, demografik ve hastalık ilişkili verileri HJHS 2,1 ve HEAD-US puanları kaydedilmiştir. Bulgular: Otuz iki katılımcının ortalama yaşı 20,6 (minimum: 15-maksimum: 31) idi. Yirmi üçü Hemofili A, 8'i Hemofili B, 1'i Von Willebrand hastalığı tanılıydı. Otuz hastanın ortalama HJHS skoru 6,7 (minimum: 0-maksimum: 22) idi. Ortalama HEAD-US skoru 14,3 (minimum: 0-maksimum: 34) idi. HJHS ve HEAD-US skorları birbiriyle uyumluydu (p=0,002). Yirmi bir hastanın 33 ekleminde artropati mevcuttu. Hem HJHS hem de HEAD-US skorları ile (sırasıyla p=0,006 ve p=0,005) artropatisi olan ve olmayan hastalar arasında, istatistiksel anlamlılık vardı. Artropati en sık sağ dizdeydi. HJHS ve HEAD-US skorları, hem diz hem de dirsek eklemlerinde uyumlu, her 2 ayak bileğinde ise uyumsuzdu. Yedi hastanın 21 ekleminde HJHS ve HEAD-US skorları uyumsuzdu. Bu eklemler; sağ ayak bileği (n=7), sol ayak bileği (n=6), sol dirsek (n=4), sağ dirsek (n=2) ve sağ diz (n=2) idi. Sonuç: HJHS ve HEAD-US skorları arasında bir korelasyon saptanmıştır. HEAD-US, ayak bileği ekleminde artropatinin erken evresini tespit etmede HJHS'den daha hassastır. Bu skorlar, tedavi eksikliklerinin ve uyumsuzluklarının ortaya çıkması için tüm hastalara rutin olarak uygulanmalıdır.
Anahtar Kelimeler: Hemofili A; Hemofili B; artropati
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