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Musculoskelet Surg. 2012 May;96 Suppl 1:S93-9. doi: 10.1007/s12306-012-0184-5. Epub 2012 Mar 24.

Shoulder arthroplasty in alkaptonuric arthropathy: a clinical case report and literature review.

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1
Unit of Shoulder and Elbow Surgery, D Cervesi Hospital, Via L Van Beethoven 1, 47841 Cattolica, RN, Italy. giovannimerolla@hotmail.com

Abstract

Alkaptonuria is a rare hereditary metabolic disease of autosomal recessive inheritance, resulting from deficiency of the enzyme homogentisic acid oxidase. The term "alkaptonuria" was first used in 1859 by Boedeker to describe a patient's urinary reducing compound, and in 1866, Virchow coined the term "ochronosis" due to typical yellow pigmentation. Deposition of this pigment in articular cartilage leads to ochronotic arthropathy, the most incapacitating complication of alkaptonuria. We report a rare case of shoulder ochronotic arthritis, treated with total shoulder arthroplasty, achieving a successful long-term clinical and radiological outcomes.

PMID:
22447274
DOI:
10.1007/s12306-012-0184-5
[Indexed for MEDLINE]
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