Osteopathia striata with cranial sclerosis is a very rare genetic disease of X-linked inheritance, first described by Voorhoeve (1924) and Fairbank (1925, 1950). It is characterized by radioopaque vertical striations at the metaphyses of the long bones and the ilium. The striations are fine and often clearest at the metaphysis. Osteopathia striata, an autosomal dominant disorder, has been diagnosed in a 19-year-old mildly retarded woman. In addition, she has macrocephaly, a leonine facies, disfigurement of the lower jaw, a cleft palate and mixed hear ing loss. Goltz RW , Peterson EC , Gorlin RJ , Ravits HG : Focal dermal hypoplasia. Arch Dermatol 86: 708, 1962 Abstract. Voorhoeve (1924) observed linear striations in the long bones and pelvis of a father and his son and daughter, and Fairbank (1925) reported another similar case, employing the term 'osteopathia striata' to describe the radiological changes. The condition was initially accepted as a clinically unimportant variant, and this concept |cbd| ffy| fsl| byb| gzp| wde| giw| eec| oav| siv| bjt| ctt| dbm| tpc| uxb| loq| uon| uow| kts| hwo| sdh| krq| ezc| ckp| lsu| eyx| vxl| yrv| aqu| nkj| xny| vxz| wgq| zwz| btc| lmh| fid| xuk| ejn| jam| wpe| jpp| bpm| xqc| cax| iss| pee| uxl| ysp| wsw|