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Revista Española de Cirugía Oral y Maxilofacial

On-line version ISSN 2173-9161Print version ISSN 1130-0558


CREO MARTINEZ, T. et al. Treatment for fibrous dysplasia when associated with hemophilia C: A case report. Rev Esp Cirug Oral y Maxilofac [online]. 2007, vol.29, n.6, pp.375-380. ISSN 2173-9161.

Fibrous Dysplasia is a benign bone disease that changes normal bone tissue for a proliferation of connective fibrous tissue. It is thought that an alteration of the Gsa gene is the main cause of the disease. Hemophilia C is a rare inherited blood disease leading to abnormal hemorrhages in affected patients. They have a factor XI deficiency. It is the least frequent of all hemophilias. It is a recessive autosomal disease, affecting both men and women. A 13 year-old patient developed fibrous dysplasia in right upper maxilla. The patient started with pain on chewing hard food. She had vestibular swelling and reddening of the right side of the palate. She had a discrete factor XI deficiency (heterozygotic). She needed special preparation before the lesion could be removed because of her deficiency. It has been discovered that the mutation of gene Gsa (GNAS1) is the reason for fibrous dysplasia. This gene is in chromosome 20q. The cause of hemophilia C is a factor XI deficiency due to a mutation in the FXI gene in chromosome 4. Perhaps these two rare diseases are related, because both are unusual diseases and both are in the same patient.

Keywords : Fibrous dysplasia; Hemophilia C; Gene Gsa; Pamidronate; FXI gene.

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