BRACHYDACTYLOUS DWARFS OF MSELENI

Eight individuals with an unusual dwarfing skeletal dysplasia have bee n investigated in a remote rural area in Northern Zululand, South Afri ca. These dwarfed individuals are members of a community numbering som e 10,000 persons; approximately 20% have a degenerative arthropathy af fecting mainly the major joints of the hips, knees, ankles, and spine. The pathogenesis of the latter disorder, which is known as Mseleni jo int disease (MJD), remains obscure. The dwarfed persons have some radi ological findings in keeping with MJD, but they are, in addition, of s hort stature and have marked brachydactyly of their fingers and toes. The pathogenetic relationship between these conditions, if any, is unc lear but may have important implications for their causal elucidation. (C) 1993 Wiley-Liss, Inc.