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Alport’s Syndrome

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Book cover International Yearbook of Nephrology 1993

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Abstract

Every nephrologist knows what Alport’s syndrome is, but no one can define it to the satisfaction of all. Cecil Alport emphasized the association of deafness with nephritis in the family he studied (1). In deference to his contribution, Alport’s syndrome commonly denotes hereditary hematuric nephritis with deafness. This simple definition has several drawbacks. Firstly, true deafness, defined as inability to hear and understand speech even with amplification (2), is rarely found in Alport patients; hearing loss is a more precise term. Secondly, families with and others without appreciable hearing loss have clinically, histologically, ultrastructurally, and immunochemically indistinguishable renal disease (3). Thirdly, mutations in COL4A5*, the gene responsible for many or most cases of Alport’s syndrome, have been found in families with (4, 5) and without (6–8) hearing loss. Hearing loss is best regarded as one of the features shown by some kindreds but not others: ocular abnormalities, abnormalities of the formed elements of the blood, and esophageal, tracheobronchial and genital leiomyomatosis fall into the same category.

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  1. Divisions of Internal Medicine and Nephrology, Department of Medicine, University of Utah School of Medicine, Salt Lake City, Utah, 84132, USA

    Martin C. Gregory

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  1. Department of Nephrology, University of Naples, Naples, Italy

    Vittorio E. Andreucci

  2. Middlesex School of Medicine, The Rayne Institute, University College, London, UK

    Leon G. Fine

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Gregory, M.C. (1992). Alport’s Syndrome. In: Andreucci, V.E., Fine, L.G. (eds) International Yearbook of Nephrology 1993. Springer, London. https://doi.org/10.1007/978-1-4471-1948-7_8

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  • DOI: https://doi.org/10.1007/978-1-4471-1948-7_8

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