Department of Orthopaedic and Traumatology, Hospital Británico de Buenos Aires.
Department of Orthopaedic and Traumatology , Hospital Británico de Buenos Aires.
Acta Biomed. 2022 Jun 7;93(S1):e2022108. doi: 10.23750/abm.v92iS1.10439.
Alkaptonuria is a rare disease characterized by the accumulation of homogentisic acid (HGA). Over time, these patients may develop disabling ochronotic arthropathy. We present 2 cases of patients with end-stage arthropathy treated with total knee arthroplasty (TKA).
Both patients complained of disabling knee pain and reported limited walking distance (200-300 m). One had a history of osteotomy for medial knee arthtritis and ignored his underlying condition. The other presented with valgus gonoarthrosis and diagnosis of alkaptonuria.
Intraoperatively, the characteristic dark-blue color in the joint was observed. Both patients evolved favorably after TKA with excellent results according to the Knee Society Scores (KSS) at three years of follow-up.
We believe TKA is the right treatment for patients with end-stage disease because it offers considerable relief from pain and allows patients to recover function.
黑尿酸尿症是一种罕见疾病，其特征是大量同型胱氨酸酸（HGA）的积累。随着时间的推移，这些患者可能会发展为致残性褐黄病性关节病。我们报告了 2 例接受全膝关节置换术（TKA）治疗的终末期关节病患者。
两名患者均主诉膝关节疼痛导致活动障碍，并自述行走距离有限（200-300 米）。其中 1 例有内侧膝关节骨关节炎切开术的病史，但忽视了其潜在疾病。另 1 例为膝内翻骨关节炎，诊断为黑尿酸尿症。
术中观察到关节呈特征性的深蓝色。两名患者在 TKA 后均有良好的进展，根据膝关节协会评分（KSS），在 3 年的随访中均取得了优异的结果。
我们认为 TKA 是终末期疾病患者的正确治疗方法，因为它可以极大地缓解疼痛并使患者恢复功能。
