Skin potentials (SP) were evoked by peripheral nerve stimulation from the hands and feet of 41 and from the genital skin of 28 male, controls. The same methods were also applied in 10 functionally impotent cases, 32 diabetic impotent and 8 diabetic normopotent cases. The SP was easily obtained from all 3 sites in all normal subjects and in 10 functionally impotent cases. The SP recorded from the genital skin was either absent or abnormal in 53% of diabetic impotent cases with or without polyneuropathy, while the incidence of abnormalities encountered in the hand and/or foot recordings was 28%. In 8 diabetic normopotent cases the SP recorded from the genital and extremity skin were completely normal. SP recorded from the genital skin is a useful method of evaluating the autonomic-sympathetic dysfunction causing impotence, especially in patients who may have autonomic disorders either located in lumbosacral spinal cord and its efferents, or generalized autonomic-peripheral dysfunction.
INTRODUCTIONAlkaptonuria is an autosomal recessive disorder of metabolism. The pathogenesis of alkaptonuria includes chronic inflammation, degeneration, and eventually osteoarthritis. Ochronotic arthropathy is a rare condition found in patients with alkaptonuria.PRESENTATION OF CASEA 60-year-old female presented for evaluation after a 10-year history of low back pain, right hip pain, and bilateral knee pain. A cementless right total hip and a cemented left knee replacement were performed. Intraoperatively, the joint surfaces, neighboring ligaments, and tendons were black with pieces of black cartilage tissue. Histological sections of bone and soft tissue demonstrated classic findings of ochronosis, including multiple pigmented areas, reactive giant cells, and a thickened, inflamed synovium.DISCUSSIONThe management of ochronotic arthropathy in alkaptonuria patients is usually conservative, but replacement surgery is offered for severely affected hip and knee joints. A few reports of the surgical treatment of ochronotic arthropathy have been published. This report describes a case of ochronotic hip and knee arthritis treated with total hip and knee arthroplasties.CONCLUSIONJoint replacement has excellent outcomes in a patient with significant degenerative arthropathy due to ochronosis.
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