In three generations of a family investigation for coexpression of May
-Hegglin anomaly and hereditary nephritis was done by routine studies,
as well as electron microscopy of renal tissue and blood cells, plate
let aggregation studies, audiograms, and ophthalmologic evaluations. T
he propositus had typical May-Hegglin anomaly and a mild form of hered
itary nephritis. One son had May-Hegglin anomaly and possible heredita
ry nephritis, and one daughter had May-Hegglin anomaly and probable he
reditary nephritis. A grandson had May-Hegglin anomaly but no evidence
of hereditary nephritis at age 23. The mild form of hereditary nephri
tis described here was atypical for Alport's syndrome, but together wi
th similar reports, suggests that a combination of May-Hegglin anomaly
and mild hereditary nephritis may be a distinct disorder.