Article

Alport’s syndrome (hereditary nephritis)

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Abstract

UP to 1958, more than 50 families with Alport’s syndrome had been reported.1 This syndrome consists of a high incidence of renal disease that occurs in several generations of a family, associated with perceptive nerve deafness and occasional ocular abnormalities. According to Alport2 early mention of a possible hereditary factor in nephritis was made by Dickinson in 1875. Reports then began to be published, describing families with a tendency toward the development of hematuria and progressive renal failure. Hurst in 1915 described such a family, and 12 years later this family was examined by Alport,2 for whom the syndrome is named.

Alport’s2 original description was of a clan of 25 persons in seven family groups. There were 10 males and 15 females; of the 10 males, seven were apparently affected and died in childhood. Although almost all the females described were either deaf or had recurrent hematuria, apparently none died with nephritis. Many of his patients had a history of hematuria that was related to eating certain berries, a phenomenon of interest, of undetermined significance, but not commented upon in recent reviews.

Report of cases

Case 1. A boy aged six years, was first examined at the Cleveland Clinic in 1959. He had three attacks of pneumonia during the first year of life. Throughout early childhood he had had frequent “bronchial infections” and episodes of otalgia. Over the years he had also had urgency and nocturia, and episodes of asthma, followed by hematuria. The family history revealed that his mother . . .


 

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