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. 2020 Aug 23;6(4):689-693.
doi: 10.1016/j.artd.2020.07.037. eCollection 2020 Dec.

Long-Term Outcomes of the Knee and Hip Arthroplasties in Patients with Alkaptonuria

Affiliations

Long-Term Outcomes of the Knee and Hip Arthroplasties in Patients with Alkaptonuria

Jihad M Al-Ajlouni et al. Arthroplast Today. .

Erratum in

Abstract

Alkaptonuria is a rare autosomal recessive metabolic disorder. It is characterized by the accumulation of homogentisic acid in the body due to a lack of enzymes that degrade it. Over time, it results in joint degeneration and eventually leads to ochronosis. Ochronosis refers to bluish-black discoloration of connective and other tissues within the body. In this study, we present 5 distinct cases diagnosed with alkaptonuria. They have undergone 8 total joint replacement surgeries (4 hips and 4 knees) within 8 years (2010-2018). All patients had an excellent outcome over several years. The follow-up period ranged from 2 to 10 years. Although none of the presented cases had intraoperative or postoperative adverse sequelae, we must take care when dealing with patients with ochronotic arthropathy. They carry a higher risk of complications than other patients with osteoarthritis disease. These complications include fractures due to fragile bone quality, muscle or tendon rupture, joint instability, and anesthesia-related complications. Total joint arthroplasty is a valid and safe option in the management of hip and knee ochronotic arthropathy.

Keywords: Alkaptonuria; Arthroplasty; Hip replacement; Knee replacement; Ochronosis.

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Figures

Figure 1
Figure 1
(a) Dark meniscus and tibial articular surface (an intraoperative photograph). (b) Dark synovial tissue and patellar articular surface (an intraoperative photograph).
Figure 2
Figure 2
Black discoloration of the femur head with its overlying cartilage (an intraoperative photograph).

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