Alkaptonuria and ochronotic arthropathy
نویسندگان
چکیده
Four cases of operative treatment for ochronosis arthropathy in patients with alcaptonuria are presented. In 3 hip joint and 1 patient knee were affected. Prior to surgery all treated conservatively. Two successfully underwent total replacement. Intertrochanteric femur osteotomy was performed case. the fourth arthroplasty allograft from rib cartilage failed due suppurative arthritis resulted resection arthrodesis.
منابع مشابه
Two Cases of Severe Ochronotic Arthropathy
Ochronotic spondylarthropathy, a long-term musculoskeletal manifestation of alkaptonuria and involvement of joints may lead to a radiographic appearance similar to that of degenerative joint disease. We report the cases of two siblings with progressive familial ochronotic arthropaty treated with total hip arthroplasty.
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The post-mortem examination of an unsuspected case of alkaptonuria revealed extensive ochronosis. Histological examination of undecalcified sections of tracheal, costal, femoral and patellar cartilage revealed, in addition to ochronotic pigment, extensive calcium pyrophosphate dihydrate (CPPD) deposition. Similar deposits were present in intervertebral discs and were related to ossification of ...
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Alkaptonuria is believed to have been described as early as 1584 (Garrod 1923). Marcet in 1823 (Rose 1957) recorded the case ofa patient whose urine caused black stains on the clothing. An accurate account of the condition was given by Boedeker (1859), who described a patient with glycosuria in whom a second reducing substance was found in the urine. This he named “alkapton” on account of its b...
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Alkaptonuria is a rare inborn error of metabolism, which is classified as an orphan disease. It is due to the lack of an enzyme homogentisate 1,2-dioxygenase, which results in an accumulation of homogentisic acid in different areas of the body, including sclera, skin, cardiac valves, articular cartilage of the large joints and intervertebral disks. We present two cases of alkaptonuria resulting...
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Ochronosis or black joints disorder is a rare autosomal recessive disorder caused by deficiency of homogentisic acidoxidase. Orthopaedic manifestations are common and mostly involve spine and large joints such as knee and hip.Arthropathy is progressive and will eventually leads to arthroplasty. Not being familiar with this disorder might lead todevastating complications. We present a 57 year-ol...
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ژورنال
عنوان ژورنال: Vestnik travmatologii i ortopedii imeni N.N. Priorova
سال: 2022
ISSN: ['2658-6738', '0869-8678']
DOI: https://doi.org/10.17816/vto97136