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HEREDITARY NEPHROPATHY, DEAFNESS, AND RENAL FOAM CELLS

JAMA. 1962;180(7):549-550. doi:10.1001/jama.1962.03050200033015.
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Perkoff and associates1 reported in 1951 the genetically predictable occurrence of chronic renal disease with associated nerve deafness in a large family. Among 59 patients studied, 14 had abnormal audiograms. Five of the patients with hearing deficit showed no evidence of renal disease at the time of examination, but 2 of these were found to be carriers of the trait for renal disease since the renal lesion was observed in their descendants. The pattern of inheritance was compatible with that of a partially sex-linked dominant trait. Graham reanalyzed this kindred and suggested that the mode of inheritance is more likely a sex-influenced dominant one.2

Several reports of hereditary renal disease with associated nerve deafness have appeared since 1951. Thus, Goldbloom et al.3 described 4 members of a single family with renal functional impairment; 2 had bilateral nerve deafness. In the kidneys of 1 patient grossly visible fatty

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