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Alkaptonuric Ochronosis; Hip Arthropathy - A Rare Case Treated with Total Hip Replacement
Mehmet YETİŞ1, Zafer ÜNVEREN1, Erdal UZUN2, Mustafa ÖZCAMDALLI1, Turan Bilge KIZKAPAN3, Adülhamit MISIR3
1Ahi Evran Üniversitesi Eğitim ve Araştırma Hastanesi, Ortopedi ve Travmatoloji Anabilim Dalı, Kırşehir, Türkiye
2Kayseri Eğitim ve Araştırma Hastanesi, Ortopedi ve Travmatoloji Kliniği, Kayseri, Türkiye
3Baltalimanı Metin Sabancı Eğitim ve Araştırma Hastanesi, Ortopedi ve Travmatoloji Kliniği, İstanbul, Türkiye

Alkaptonuria is a rare autosomal-recessive metabolic disease caused by congenital homogentisic acid (HGA) oxidase enzyme deficiency which affects one in 100,000 to 250,000 individuals. Circulating HGA pass into various tissues through-out the body, mainly in cartilage and connective tissues, where its oxidation products polymerize and deposit as a melanin-like pigment. Currently, there is no specific treatment for alkaptonuria. A 63-year-old male patient presented to our clinic, complaining of chronic hip pain that had worsened over the previous 3 years. The patient also had darkly stained sclera and pinnae characteristic of ochronosis. A cementless right total hip replacement was performed. At the 5-year follow-up, the patient had returned to full activities, reported no hip pain, and was very satisfied with the outcome.

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