The syndrome of hereditary nephritis, first described in the early 1900´s, was more fully delineated by Alport in 1927 and has received increasing attention since Perkoff´s report in 1951. Although a large number of kindreds have now, been described in foreign countries, hereditary nephropathy Is still generally regarded as an uncommon disease.
Authors experienced a 30 year old male patient with chronic glomerulonephritis and auditory deafness, who received intermittent hemodialysis weekly but two months later died of uremia. From his amily history, it was disclosed that many members were affected by nephropathy. Among 16 family members, 14 cases were affected, and all, four brothers of the proband died of uremia before the age of 28. His two sisters had continuous proteinuria and hematuria without any clinical symptom. Except the proband who showed clinical hearing loss and moderate degree of perceptive deafness on audiometry, no extrarenal, manifestation was found in the affected family members. To authors´ knowledge, this report documents the first family with Alport´s syndrome in Korea.
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