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

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 discolor...

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Published inArthroplasty today Vol. 6; no. 4; pp. 689 - 693
Main Authors Al-Ajlouni, Jihad M., Alisi, Mohammed S., Yasin, Mohamad S., Khanfar, Aws, Hamdan, Mohammad, Halaweh, Ahmad Abu, Al Hawamdeh, Hashem, Tayyem, Mohammed, Alsbou, Mohammad S.
Format Journal Article
LanguageEnglish
Published Elsevier Inc 01.12.2020
Elsevier
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Summary: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.
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ISSN:2352-3441
2352-3441
DOI:10.1016/j.artd.2020.07.037