Medical University of Plovdiv, Plovdiv, Bulgaria.
Folia Med (Plovdiv). 2020 Dec 31;62(4):762-768. doi: 10.3897/folmed.62.e50550.
Blood-induced joint damage as a hallmark of haemophilia continues to occur despite the widespread prophylaxis. Pre-cise assessment and follow-up of joint status are crucial for tailoring their treatment.
To study the correlation between the bleeding phenotype, the functional joint status, and the magnetic resonance imaging score in pediatric patients with haemophilia.
Eighty-six joints (ankles, knees, and elbows) in patients aged 10.7±0.5 (range 4 - 20) years with severe/moderate haemophilia A, severe haemophilia B and haemophilia A with inhibitors were included in the study. The joints were assessed by Haemophilia Joint Health Score 2.1 (HJHS2.1) one month after the last hemarthrosis in a non-bleeding state. The magnetic reso-nance imaging was performed on 40 (46.5%) of the examined hemophilic joints (16 ankles, 11 knees and 13 elbows).
Joint bleeds were present in 37 (38.9%) of the joints with ankles being the most commonly affected. Sixty joints (69.8%) had normal HJHS2.1 score. Only the loss of flexion score differed significantly between the joints and the ankles had highest score. The cumulative number of hemarthrosis in the joint correlated moderately with hemosiderin deposition and strongly with the formation of subchondral cysts on magnetic resonance imaging. The magnetic resonance imaging scores for soft tissue and osteochondral domains correlated moderately with the cumulative number of hemarthrosis in the joint and only with the presence of pain and crepitus of mo-tion from the physical examination.
Magnetic resonance imaging is more sensitive than the bleeding phenotype and physical examination in detecting early signs of haemophilic arthropathy.
尽管广泛应用了预防治疗,但血液引起的关节损伤仍是血友病的一个显著特征。精确评估和随访关节状况对于制定治疗方案至关重要。
研究儿童血友病患者的出血表型、关节功能状态和磁共振成像评分之间的相关性。
研究纳入了 86 个关节(踝关节、膝关节和肘关节),这些关节分别来自患有重度/中度血友病 A、重度血友病 B 和血友病 A 伴抑制剂的 10.7±0.5 岁(4-20 岁)的患者。在非出血状态下,于上次关节内血肿后一个月,使用 Haemophilia Joint Health Score 2.1(HJHS2.1)对关节进行评估。对 40 个(46.5%)受检的血友病关节(16 个踝关节、11 个膝关节和 13 个肘关节)进行了磁共振成像检查。
37 个(38.9%)关节存在关节内出血,其中踝关节最常受累。60 个(69.8%)关节的 HJHS2.1 评分正常。仅关节活动丧失评分在关节和踝关节之间存在显著差异,且踝关节评分最高。关节内血肿的累积次数与含铁血黄素沉积中度相关,与磁共振成像上的软骨下囊肿形成高度相关。磁共振成像的软组织和骨软骨域评分与关节内血肿的累积次数中度相关,仅与体检时的疼痛和活动时的摩擦音相关。
磁共振成像在检测血友病性关节病的早期征象方面比出血表型和体格检查更敏感。