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      Hip and knee replacement in patients with ochronosis: Clinical experience and literature review

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          Abstract

          Patients with alkaptonuria can present ochronotic degenerative arthropathy due to the accumulation of pigments in the cartilages. Ochronotic arthropathy initially affects the spine, then there is the involvement of the other large joints, with greater frequency of the knees. In this article we will present two patients with alkaptonuria who have been effectively treated with knee and hip replacement, comparing our experience with what is available in the literature.

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          Most cited references29

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          Nitisinone arrests ochronosis and decreases rate of progression of Alkaptonuria: Evaluation of the effect of nitisinone in the United Kingdom National Alkaptonuria Centre

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            The role of calcified cartilage and subchondral bone in the initiation and progression of ochronotic arthropathy in alkaptonuria.

            Alkaptonuria is a genetic disorder of tyrosine metabolism, resulting in elevated circulating concentrations of homogentisic acid. Homogentisic acid is deposited as a polymer, termed ochronotic pigment, in collagenous tissues, especially cartilages of weight-bearing joints, leading to a severe osteoarthropathy. We undertook this study to investigate the initiation and progression of ochronosis from the earliest detection of pigment through complete joint failure.
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              Orthopedic Manifestations of Ochronosis: Pathophysiology, Presentation, Diagnosis, and Management

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                Author and article information

                Journal
                Orthop Rev (Pavia)
                OR
                Orthopedic Reviews
                PAGEPress Publications, Pavia, Italy
                2035-8237
                2035-8164
                26 June 2020
                29 June 2020
                : 12
                : Suppl 1
                : 8687
                Affiliations
                [1 ]Department of Orthopedics, Fondazione Policlinico Universitario Agostino Gemelli IRCCS; Università Cattolica del Sacro Cuore , Rome
                [2 ]Department of Oncologic and Reconstructive Orthopedic Surgery, Azienda Ospedaliera Universitaria Careggi , Florence, Italy
                Author notes
                epartment of Orthopaedics, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Università Cattolica del Sacro Cuore Roma, Largo F. Vito 1, 00168 Rome, Italy. Tel. +393289511465. rovere292@ 123456hotmail.com

                Contributions: the authors contributed equally.

                Conflict of interest: the authors declare no potential conflict of interest.

                Availability of data and materials: All data and materials are reported in the text.

                Ethics approval and consent to participate: Not applicable.

                Informed consent: Not applicable.

                Article
                10.4081/or.2020.8687
                7459366
                32913615
                8a1c2a4a-497d-4912-9408-d436c563beab
                ©Copyright: the Author(s)

                This work is licensed under a Creative Commons Attribution NonCommercial 4.0 License (CC BY-NC 4.0).

                History
                : 15 June 2020
                : 17 June 2020
                Page count
                Figures: 3, Tables: 0, Equations: 0, References: 30, Pages: 4
                Funding
                Funding: none.
                Categories
                Case Report

                Orthopedics
                ochronosis,alkaptonuria,hip and knee replacement
                Orthopedics
                ochronosis, alkaptonuria, hip and knee replacement

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