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Chapter-021 Ochronosis

BOOK TITLE: Textbook of Orthopedics & Trauma (4 Volumes)

Author
1. Kulkarni GS
2. Menon P
3. Kulkarni Milind G
4. Saxena Sagar
ISBN
9789385891052
DOI
10.5005/jp/books/12869_22
Edition
3/e
Publishing Year
2016
Pages
3
Author Affiliations
1. Postgraduate Institute and Swasthiyog Pratishthan, Miraj, India, Postgraduate Institute of Swasthiyog Pratisthan, Miraj, Swasthiyog Pratisthan, Postgraduate Institute of Orthopedics, Miraj, Maharashtra, India, Postgraduate Institute of Swasthiyog Pratishthan, Miraj, Maharashtra, India; Sandhata Medical Research Society, Miraj, Maharashtra, India
2. GS Medical College, Mumbai, Postgraduate Institute of Swasthiyog Pratishthan, Fracture and Orthopedics Hospital, Miraj, Maharashtra, India
3. Postgraduate Institute of Swasthiyog Pratishthan, Fracture and Orthopedics Hospital, Miraj, Maharashtra, India
4. Postgraduate Institute of Swasthiyog Pratishthan, Fracture and Orthopedics Hospital, Miraj, Maharashtra, India
Chapter keywords
ochronosis, alkaptonuria, metabolism, tendons, cardiovascular, cardiac murmurs, dysphagia, osteoporosis, ochronotic arthropathy

Abstract

The objective of this chapter is to introduce the ochronosis. Ochronosis is the syndrome caused by the accumulation of homogentisic acid in connective tissues. Alkaptonuria was first described by Rudolf Virchow, is a rare hereditary disorder characterized by absence of the enzyme homogentisic acid oxidase and accumulation of homogentisic acid (aromatic acid), which is produced during the metabolism of phenylalanine and tyrosine. This chapter also discusses clinical features, radiologic feature, extraspinal abnormalities, and management of ochronosis. There is no specific treatment for ochronosis except symptomatic treatment. A diet free of tyrosine and phenylalanine with intake of vitamin C to reduce the excretion of homogentisic acid is advisable but it has no effect on the progress of disease.

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