Reply to Drs. Sauvegrain and Maroteaux

Abstract

We read with interest the letter by Sauvegrain and Maroteaux concerning our article on "Familial Congenital Micromelic Dysplasia With Dislocation of Radius and Distinct Face: A New Skeletal Dysplasia Syndrome" [Borochowitz et al., 19911. We were delighted to read that they agree that our cases were similar only to their cases 4 and 5 [Maroteaux et al., 19891, as well as to Viljoen's case 1 [Viljoen et al, 19873. We also appreciate their conclusion that omodysplasia has a different inheritance (autosoma1 dominant), as opposed to that in the family we studied (autosomal recessive). Thus, it is most appropriate to consider these groups as 2 different disorders, and not as clinical variants of a single entity. Examination of chondroosseous biochemistry and morphology may clarify this matter. REFERENCES