HEREDITARY NEPHROPATHY (ALPORT SYNDROME) - CORRELATION OF CLINICAL-DATA WITH GLOMERULAR BASEMENT-MEMBRANE ALTERATIONS

  • 1 January 1980
    • journal article
    • research article
    • Vol. 13  (5) , 203-207
Abstract
In 32 patients from 27 families affected with hereditary nephropathy (Alport syndrome), the glomerular basement membranes were examined by EM and the percentage of characteristically split and thin basement membrane portions was determined. The clinical course was more severe in males which corresponded with a higher rate of basement membrane alterations: on an average in males, 61% split and 6% thin, but only 18% split and 21% thin in females. The splitting lesion increased with age in males but not in females. There were also indications for a possible positive correlation of the splitting lesion and the grade of proteinuria. Compared with the splitting lesion, basement membrane thinning seemed to be of minor importance.

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