抄録
We reported a case of ochronotic arthropathy in the hip joint. The patient was 64-years-old woman who complained severe pain of the right hip and dyabasia. The radiograph of the right hip showed rapid collapse of the femoral head. Total hip arthroplasty was carried out by the diagnosis of rapidly destructive coxarthopathy. The articulr cartilage of the femoral head and synovium displayed black pigmentation. And the urine turned black after alkalization and homogentisic acid was detected. Therefore we diagnosed as arcaptonuria. 1 year after the total hip arthroplasty, she can walk with a cane without pain and the prosthesis has no loosening.
Alcaptonuria is rare hereditary metabolic disorder that results from a deficiency of the enzyme homogentisic acid oxidase. It is associated with various systemic abnormalities related to the deposition of homogentisic acid pigment in connective tissues. These pigmentary changes are termed ochronosis. Diagnosis is usually made by the triad of degenerative arthritis, ochronotic pigmentation, and change of urine color. Nonsteroidal anti-inflammatory drugs and physiotherapy reduce joint symptoms in the early stage of ochronotic arthritis. In the late stage of arthritis, arthroplasty is undergone. In our case total hip arthroplasty was carried out.
