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Review
. 1999;23(2):122-5.
doi: 10.1007/s002640050325.

Ochronotic arthropathy: case report and review of the literature

N Hamdi  1 , T D Cooke, B Hassan
Affiliations
Review

Ochronotic arthropathy: case report and review of the literature

N Hamdi et al. Int Orthop. 1999.

Abstract
in English, French

Alkoptonuria is an inherited metabolic disorder which is associated with various systemic abnormalities and related to the deposition of homogentisic acid pigment in connective tissues. These pigmentary changes are termed "ochronosis". We describe two patients with ochronotic arthropathy who presented with progressive and advanced degenerative changes in the lumbo-sacral spine. The literature, differential diagnosis and management of this rare condition are reviewed in this article. Management is usually conservative, but replacement surgery may be offered for severely affected major joints.

L’ochronose est une manifestation musculo-squélettique de l’alkaptonurie dûe à un désordre métabolique qui accompagne diverses anomalies systemiques et associé au depôt de pigments acides homogénetiques dans les tissues congonctifs. Nous decrivons deux cas d’ arthopathie presentant des douleurs progressives et des symptômes de dégénerescence avancée de la region lombo-sacrée. Cet article inclut la bibliographie, les diagnostics differentiels et le traitement de cette maladie rare. La therapie classique est toujours d’ actualité, mais l’implantation de prothéses peut être offerte dans des cas d’ atteintes severes des articulations majeures.

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