Combination of Fibromuscular Hyperplasia, Renal Aplasia, Hypoplasia or Dysplasia and Otosclerosis Occurring in the Same Individual or the Same Family

Abstract

Three reports of fibromuscular hyperplasia (FMH) of the renal arteries with hypertension occurring in 2 siblings and a few instances of renal agenesis and unilateral renal aplasia occurring in the same family were found. FMH of the renal arteries is reported in 2 women, 1 giving birth to a child with renal agenesis, the other to a child with a focally dysplastic hypoplasia of the kidneys. A 3rd family is reported, heavily loaded with hypertension and otosclerosis, in which 2 siblings with FMH and otosclerosis were found. Another 4 patients without known heredity for hypertension, but with FMH combined with renal or urinary tract anomalies, are also reported. The findings are discussed, particularly in relation to the findings in a large material of chronic nonobstructive pyelonephritis, where in those with well maintained renal function, hypertension below the age of 40 was found predominantly in females with a positive family history of hypertension and signs highly suggestive of infected hypoplasia or dysplasia of the kidney.