Fibrous dysplasia and cherubism as an hereditary familial disease. Follow-up of four generations.
Five cases of dysplasia of the jaws in one family which has been under our observation since 1970 are reported. The disease appeared as a mixed display of jaw lesions, in some members as fibrous dysplasia and in others as cherubism. We were able to trace the disorder through an unbroken line of four generations, and thus to demonstrate autosomal dominant inheritance. Cytogenetic analysis performed on three members of this family revealed a significantly increased rate of chromosomal breakage.
Zohar
Y
Y
Dept. of Otolaryngology and Jaw Surgery, Hasharon Hospital, Petah-Tiqva, Israel.
Grausbord
R
R
Shabtai
F
F
Talmi
Y
Y
D000328:Adult; D002636:Cherubism / Q000235:genetics*; D002648:Child; D002675:Child, Preschool; D002869:Chromosome Aberrations; D005260:Female; D005357:Fibrous Dysplasia of Bone / Q000235:genetics*; D005359:Fibrous Dysplasia, Polyostotic / Q000235:genetics*; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008336:Mandibular Diseases / Q000235:genetics*; D010375:Pedigree