Cases reported "Ochronosis"

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1/4. Arthroscopic diagnosis and management of ochronotic arthropathy of the knee.

    Ochronotic arthropathy is a progressive joint disorder resulting from the deposition of a derivative of homogentisic acid into connective tissues, especially meniscal and articular cartilage. patients may present with symptoms, physical examination results, and radiographic changes consistent with degenerative joint disease. We present a case in which an operative arthroscopy of the knee was highly suggestive of ochronotic arthropathy. The definitive diagnosis of ochronosis was subsequently confirmed by laboratory and pathologic evaluation.
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2/4. Hip arthroplasty for ochronosis.

    alkaptonuria is a metabolic disorder in which homogentisic acid oxidase is absent. Therefore, homogentisic acid accumulates in cartilage and connective tissues. We can diagnose ochronotic arthropathy, a manifestation of long standing alkaptonuria, through careful radiological, physical, and laboratory examination. In this report, we describe 4 cases of ochronotic arthropathy to which we applied cementless total hip prosthesis due to severe hip involvement.
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3/4. ochronosis and lumbar disc herniation.

    alkaptonuria is a rare, autosomal recessive metabolic disorder in which the homogentisic acid oxidase activity is absent. Its incidence is as low as 0.001%. ochronosis is the pigmentation of connective tissues and this pigmentation leads to degenerative changes in alkaptonuric patients.alkaptonuria most prominently involves the lumbar region, but lumbar disc herniation as the presenting feature of alkaptonuria is not common. Only a few patients required surgical intervention.Herewith we report an alkaptonuric patient, who was operated on for lumbar disc herniation. His discectomy material was black and the metabolic disorder was diagnosed retrospectively.This metabolic disease is often recognized on physical re-examination after the black disc material was seen during the operation. Therefore urinalysis for homogentisic acid should be performed in all patients with degenerative changes of the vertebral column. The results of disc surgery in this patient group is successful.
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4/4. Hip arthroplasty for alkaptonuric ochronosis. A case report.

    Ochronotic arthropathy is present in about one third of the patients with alkaptonuria. The large joints, as well as the spinal column, are affected. In a typical case with a grave classic ochronotic arthro- and spondylopathy, arthroplasties of the hips greatly increased the patient's physical activity.
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