AUTOSOMAL-DOMINANT OSTEOSCLEROSIS TYPE STANESCU - THE 3RD FAMILY

We describe a family with Stanescu osteosclerosis, The propositus and his mother were short and had cortical sclerosis of the long bones, de ficient facial sinus development, cranial bone malformations, and norm al intelligence. To the best of our knowledge, only two such families have been described previously, The autosomal dominant pattern of inhe ritance of this skeletal dysplasia is reinforced, as there are many ot her reportedly affected relatives, including the maternal grandfather, uncles, and aunts of the propositus. The findings of wormian bones an d calcification of the fair, not previously described, may be added to the phenotype. (C) 1995 Wiley-Liss, Inc.