Impact of the HEAD-US Scoring System for Observing the Protective Effect of Prophylaxis in Hemophilia Patients: A Prospective, Multicenter, Observational Study

Publisher: Galenos Yayinevi Country of Publication: Turkey NLM ID: 9606065 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1308-5263 (Electronic) Linking ISSN: 13007777 NLM ISO Abbreviation: Turk J Haematol Subsets: MEDLINE

Publication: 2012- : Ankara, Turkey : Galenos Yayinevi
Original Publication: Ankara, Türkiye : Turkish Society of Haematology

Hemophilia A/*prevention & control ; Joint Diseases/*diagnosis ; Research Design/*statistics & numerical data ; Ultrasonography/*methods; Adolescent ; Adult ; Aged ; Child ; Diagnosis, Differential ; Early Diagnosis ; Follow-Up Studies ; Hemophilia A/diagnosis ; Hemophilia A/therapy ; Hemorrhage/diagnosis ; Hemorrhage/etiology ; Humans ; Joint Diseases/prevention & control ; Joints/diagnostic imaging ; Joints/pathology ; Male ; Middle Aged ; Point-of-Care Testing ; Prospective Studies ; Protective Factors ; Research Design/trends ; Severity of Illness Index ; Turkey/epidemiology

Objective: This study aimed to observe the preventive effect of prophylactic treatment on joint health in people with hemophilia (PwH) and to investigate the importance of integration of ultrasonographic examination into clinical and radiological evaluation of the joints.
Materials and Methods: This national, multicenter, prospective, observational study included male patients aged ≥6 years with the diagnosis of moderate or severe hemophilia A or B from 8 centers across Turkey between January 2017 and March 2019. Patients were followed for 1 year with 5 visits (baseline and 3 ^th , 6 ^th , 9 ^th , and 12 ^th month visits). The Hemophilia Joint Health Score (HJHS) was used for physical examination of joints, the Pettersson scoring system was used for radiological assessment, point-of-care (POC) ultrasonography was used for bilateral examinations of joints, and the Hemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) score was used for evaluation of ultrasonography results.
Results: Seventy-three PwH, of whom 62 had hemophilia A and 11 had hemophilia B, were included and 24.7% had target joints at baseline. The HJHS and HEAD-US scores were significantly increased at the 12 ^th month in all patients. These scores were also higher in the hemophilia A subgroup than the hemophilia B subgroup. However, in the childhood group, the increment of scores was not significant. The HEAD-US total score was significantly correlated with both the HJHS total score and Pettersson total score at baseline and at the 12 ^th month.
Conclusion: The HEAD-US and HJHS scoring systems are valuable tools during follow-up examinations of PwH and they complement each other. We suggest that POC ultrasonographic evaluation and the HEAD-US scoring system may be integrated into differential diagnosis of bleeding and long-term monitoring for joint health as a routine procedure.

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Keywords: Hemophilic arthropathy; Joint Scores; HJHS; Ultrasonography
Local Abstract: [Publisher, Turkish] Bu çalışmada hemofili hastalarında profilaksinin eklem sağlığı üzerindeki koruyucu etkisini gözlemlemek ve eklemlerin klinik ve ultrasonografik olarak değerlendirilmesinin öneminin araştırılması amaçlandı. [Publisher, Turkish] Ulusal, çok merkezli, prospektif ve gözlemsel olan araştırma Ocak 2017 - Mart 2019 arasında 8 ayrı merkezde takip edilen erkek ve 6 yaşından büyük orta seviyede veya ağır hemofili-A ve hemofili-B hastalarını kapsıyordu. Hastalar 1 yıllık takip sırasında toplam 5 ayrı vizitte (başlangıç, 3. ay, 6. ay, 9. ay ve 12. ay) değerlendirildi. Hemofili Eklem Sağlığı Skoru (HJHS) eklemlerin fiziksel bakısında kullanılırken Petterson skorlama sistemi radyolojik bakı sırasında değerlendirildi. Ayrıca, hasta başı ultrasonografisiyle bilateral eklem incelemeleri yapılarak HEAD-US skorlama yöntemiyle eklem skorları belirlendi. [Publisher, Turkish] Çalışmada 62’si hemofili A ve 11’I hemofili B olan 73 hastanın %24,7’sinde hedef eklem varlığı mevcuttu. HJHS ve HEAD-US skorlarının tüm hastalarda takibin 12. ayında anlamlı olarak arttığı gözlendi. Hemofili A hastalarında daha yüksek skorlar saptandı. Skor artımı alt gruplarda değerlendirildiğinde çocukluk yaş grubunda artışın anlamlı olmadığı gözlendi. Başlangıçta ve 12. ayda yapılan üçlü bakıda HJHS, HEAD-US ve Petterson skorlarının anlamlı olarak korele olduğu saptandı. [Publisher, Turkish] HJHS eklem skoru ile HEAD-US radyolojik skorlamasının hemofili hastalarının eklem sağlığının takibinde çok değerli olup birbirlerini destekledikleri yakından gözlendi. Hasta başı US skorlama sistemlerinin günümüzde hemofilide eklem sağlığının rutin takip ve izlemi sürecinde hem kanama ayırıcı tanısı hem de uzun dönemli takip açısından oldukça değerli bir yeri olacağını düşünüyoruz.

Date Created: 20210129 Date Completed: 20211116 Latest Revision: 20211116

20231215

PMC8171214

10.4274/tjh.galenos.2021.2020.0717

33508912