Literature DB >> 7753441

[A rare case of high dorsal radicular-medullary compression in a patient with ochronotic arthropathy. Clinico-radiological features].

C Marsile1, C Menozzi, C Menozzi.   

Abstract

Alkaptonuria is a rare, hereditary, metabolic disease in which homogentisic acid, an intermediary product in the metabolism of phenylalanine and tyrosine, cannot be further metabolized. The metabolic defect causes a characteristic triad of homogentisic aciduria, ochronosis, and arthritis. The cause of this disease is a constitutional lack of the enzyme homogentisic acid oxidase. The condition is inherited as an autosomal recessive disease. The ochronotic arthritis affects mainly male subjects after fourty. Authors present a case of a 60 years old man suffering from a generalized ochronotic arthritis since 1976. From 1992 summer to the beginning of 1993 the patient developed a progressive weakness in the lower limbs associated with thoracic back pain and subsequently a spastic paraparesis. These symptoms were related to a vertebral compression due to D4-D5 pathologic fracture. In spite of a decompressive operation the neurologic symptoms didn't improve.

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Year:  1995        PMID: 7753441

Source DB:  PubMed          Journal:  Minerva Med        ISSN: 0026-4806            Impact factor:   4.806


  3 in total

Review 1.  Alkaptonuric ochronosis with aortic valve and joint replacements and femoral fracture: a case report and literature review.

Authors:  Alexander A Fisher; Michael W Davis
Journal:  Clin Med Res       Date:  2004-11

2.  Radiologic features of lumbar spine in ochronosis in late stages.

Authors:  Petek Bayindir; Gülgün Yilmaz Ovali; Yüksel Pabuşçu; Cüneyt Temiz; Tuncay Duruoz
Journal:  Clin Rheumatol       Date:  2005-10-25       Impact factor: 2.980

3.  Ochronotic arthritis: case reports and review of the literature.

Authors:  Ercan Cetinus; Ilhan Cever; Cemal Kural; Haldun Erturk; Mustafa Akyildiz
Journal:  Rheumatol Int       Date:  2004-12-03       Impact factor: 2.631

  3 in total

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