MSELENI AND HANDIGODU FAMILIAL OSTEOARTHROPATHIES - SYNDROMIC IDENTITY

Citation
Ss. Agarwal et al., MSELENI AND HANDIGODU FAMILIAL OSTEOARTHROPATHIES - SYNDROMIC IDENTITY, American journal of medical genetics, 72(4), 1997, pp. 435-439
Citations number
16
Categorie Soggetti
Genetics & Heredity
ISSN journal
01487299
Volume
72
Issue
4
Year of publication
1997
Pages
435 - 439
Database
ISI
SICI code
0148-7299(1997)72:4<435:MAHFO->2.0.ZU;2-X
Abstract
Mseleni joint disease (MJD) and Handigodu joint disease (HJD) are fami lial skeletal disorders that affect several hundred persons in norther n Zululand, South Africa and in the Shimoga district of southern India , respectively, Severe precocious, progressive degenerative osteoarthr opathy, which occurs in both conditions, causes marked physical handic ap by adulthood, The clinical and radiological manifestations of MJD a nd HJD are very similar and it is possible that they represent the sam e entity, HJD appears to be inherited as an autosomal dominant trait, while MJD clusters in families without a definite Mendelian pattern, I t is possible that an environmental factor is active in the pathogenes is of both disorders and comparative investigations would be fruitful, The predominance of severe degenerative osteoarthropathy in both diso rders may have important implications for the elucidation of the patho genesis of the common conventional forms of osteoarthropathy. (C) 1997 Wiley-Liss, Inc.