A CASE OF ALPORT'S SYNDROME

A 19-year-old female belonging to a family of Alport's syndrome was autopsied and her kidneys were examined in detail light and electron microscopically. The basement membrane was examined chiefly and the laminated thickening and/or splitting, looseness, irregularity and rail-like appearance of lamina densa were found in the glomerular, Bowman's capsular, tubular and interstitial capillary basement membranes. These findings were strongly suggestive of Alport's syndrome, whether or not the particles are seen in the basement membrane. In addition, Japanese reports on Alport's syndrome (total 48 families) were summarized and renal lesions were examined in comparison. It has been said that the prognosis is worse in the male than in the female, but according to our Investigation on case reports in Japan, the prognosis showed no difference between male and female.