Turkiye Klinikleri Journal of Medical Sciences

.: ORIGINAL RESEARCH
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
Mehmet Can UĞURa, İpek TAMSELb, Necati Muhammed TATc, Kaan KAVAKLId
aClinic of Hematology, İzmir Bozyaka Training and Research Hospital, İzmir, TURKEY
bDepartment of Radiology, Ege University Faculty of Medicine, İzmir, TURKEY
cDepartment of Physiotherapy and Rehabilitation, Necmettin Erbakan University Faculty of Health Sciences, Konya, TURKEY
dDepartment of Pediatric Hematology, Ege University Faculty of Medicine, İzmir, TURKEY
Turkiye Klinikleri J Med Sci. 2021;41(1):46-50
doi: 10.5336/medsci.2020-78772
Article Language: EN
Full Text
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
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
REFERENCES:
  1. Roosendaal G, Lafeber FP. Pathogenesis of haemophilic arthropathy. Haemophilia. 2006;12 Suppl 3:117-21.[Crossref] [PubMed] 
  2. Srivastava A, Brewer AK, Mauser-Bunschoten EP, Key NS, Kitchen S, Llinas A, et al; Treatment Guidelines Working Group on Behalf of The World Federation Of Hemophilia. Guidelines for the management of hemophilia. Haemophilia. 2013;19(1):e1-47.[Crossref] [PubMed] 
  3. Berquist TH, Bancroft LW. Elbow and forearm. MR Imaging of the Musculoskeletal System. 5th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2006. p.708-11.
  4. Jaganathan S, Gamanagatti S, Goyal A. Musculoskeletal manifestations of hemophilia: imaging features. Curr Probl Diagn Radiol. 2011;40(5):191-7.[Crossref] [PubMed] 
  5. Manco-Johnson MJ, Abshire TC, Shapiro AD, Riske B, Hacker MR, Kilcoyne R, et al . Prophylaxis versus episodic treatment to prevent joint disease in boys with severe hemophilia. N Engl J Med. 2007;357(6):535-44.[Crossref] [PubMed] 
  6. Di Minno MN, Iervolino S, Soscia E, Tosetto A, Coppola A, Schiavulli M, et al. Magnetic resonance imaging and ultrasound evaluation of "healthy" joints in young subjects with severe haemophilia A. Haemophilia. 2013;19(3): e167-73.[Crossref] [PubMed] 
  7. Gouw SC, Timmer MA, Srivastava A, de Kleijn P, Hilliard P, Peters M, et al. Measurement of joint health in persons with haemophilia: A systematic review of the measurement properties of haemophilia-specific instruments. Haemophilia. 2019;25(1):e1-e10.[Crossref] [PubMed] [PMC] 
  8. Poonnoose PM, Manigandan C, Thomas R, Shyamkumar NK, Kavitha ML, Bhattacharji S, et al. Functional Independence Score in Haemophilia: a new performance-based instrument to measure disability. Haemophilia. 2005;11(6):598-602.[Crossref] [PubMed] 
  9. Chung SW, Seo YJ, You CW, Chun TJ, Jung KJ, Kim JH. A Combined Ultrasonographic and Conventional Radiographic Assessment of Hemophilic Arthropathy. Indian J Hematol Blood Transfus. 2017;33(3):380-8.[Crossref] [PubMed] [PMC] 
  10. Martinoli C, Della Casa Alberighi O, Di Minno G, Graziano E, Molinari AC, Pasta G, et al. Development and definition of a simplified scanning procedure and scoring method for Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US). Thromb Haemost. 2013;109(6):1170-9.[Crossref] [PubMed] 
  11. Abdelwahab M, Elsayed N. Radiological and clinical evaluation of hemophilic arthropathy in Egyptian patients. Acta Pediatr Esp 2010;68(2):71-8.[Link] 
  12. Bladen M, Main E, Hubert N, Koutoumanou E, Liesner R, Khair K. Factors affecting the Haemophilia Joint Health Score in children with severe haemophilia. Haemophilia. 2013;19(4):626-31.[Crossref] [PubMed] 
  13. Manco-Johnson MJ, Nuss R, Lear J, et al. 32P Radiosynoviorthesis in children with hemophilia. J Pediatr Hematol Oncol. 2002;24:534-9.[Crossref] [PubMed] 
  14. Feldman BM, Funk SM, Bergstrom BM, et al. Validation of a new pediatric joint scoring system from the International Hemophilia Prophylaxis Study Group: validity of the hemophilia joint health score. Arthritis Care Res (Hoboken). 2011;63:223-30.[Crossref] [PubMed] 
  15. Lundin B, Ljung R, Pettersson H. European Paediatric Network for Haemophilia M. MRI scores of ankle joints in children with haemophilia- comparison with clinical data. Haemophilia. 2005;11:116-22.[Crossref] [PubMed] 
  16. Bladen M, Main E, Hubert N, Koutoumanou E, Liesner R, Khair K. Factors affecting the Haemophilia Joint Health Score in children with severe haemophilia. Haemophilia. 2013; 19:626-31.[Crossref] [PubMed] 
  17. Foppen W, van der Schaaf IC, Fischer K. Value of routine ultrasound in detecting early joint changes in children with haemophilia using the ''Haemophilia Early Arthropathy Detection with Ultrasound'' protocol. Haemophilia. 2016;22(1):121-5.[Crossref] [PubMed] 
  18. Li J, Guo XJ, Ding XL, Lyu BM, Xiao J, Sun QL, et al. Clinical application and optimization of HEAD-US quantitative ultrasound assessment scale for hemophilic arthropathy. Zhonghua Xue Ye Xue Za Zhi. 2018;39(2): 132-6.
  19. Altisent C, Martorell M, Crespo A, Casas L, Torrents C, Parra R. Early prophylaxis in children with severe haemophilia A: clinical and ultrasound imaging outcomes. Haemophilia. 2016;22(2):218-24.[Crossref] [PubMed] 
  20. De la Corte-Rodriguez H, Rodriguez-Merchan EC, Alvarez-Roman MT, Martin-Salces M, Martinoli C, Jimenez-Yuste V. The value of HEAD-US system in detecting subclinical abnormalities in joints of patients with hemophilia. Expert Rev Hematol. 2018;11(3):253-61.[Crossref] [PubMed] 
  21. Sari TT, Chozie NA, Gatot D, Tulaar AB, Dharma R, Sukrisman L, et al. Clinical and ultrasound joint outcomes in severe hemophilia A children receiving episodic treatment in Indonesian National Hemophilia Treatment Center. Med J Indones. 2017;26:47.[Crossref] 
  22. Plut D, Kotnik BF, Zupan IP, Kljucevsek D, Vidmar G, Snoj Z, et al. Diagnostic accuracy of haemophilia early arthropathy detection with ultrasound (HEAD-US): a comparative magnetic resonance imaging (MRI) study. Radiol Oncol. 2019;53(2):178-86.[Crossref] [PubMed] [PMC] 

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