ABSTRACT
UNLABELLED: Cherubism is a rare, painless, disfigurating disease primarily affecting bones of the jaws. OBJECTIVE: To report on five patients with cherubism. The symptoms of the disease, methods of management and possible mode of inheritance are discussed and literature is reviewed. PATIENTS: The study involves five cherubs, members of one family. The diagnoses were based on history, physical examination, laboratory tests, X-ray parameters, and clinical follow-up. One member of the family had surgical intervention. The remaining cases were left for observation. RESULTS: Good aesthetic and long lasting effect was reached in the operated patient. CONCLUSIONS: Treatment is unnecessary unless functional or emotional disturbances develop. An autosomal recessive pattern of inheritance is suggested for these cases, although autosomal dominant transmission has been previously established.
Subject(s)
Cherubism/genetics , Adolescent , Cherubism/diagnosis , Cherubism/surgery , Cherubism/therapy , Child , Esthetics , Female , Follow-Up Studies , Humans , Karyotyping , Male , Osteotomy , PedigreeABSTRACT
A case of rare facial dysplasia due to renal osteodystrophy of end stage renal failure is described in a 19-year old boy treated with chronic haemodialyses. The favourable effects of surgical correction are shown.
