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At least 595 records · Page 33Linked to original sources
PubMed1981

McCune-Albright's syndrome.

A case of polyostotic fibrous dysplasia, cutaneous pigmentation, and a bifid ureter is presented. The need for urinary tract investigation in McCune-Albright's syndrome is stressed.

Child↗
PubMed2000

External cholesteaoma and fibrous dysplasia of temporal bone.

Cholesteatoma is a disease that involves almost exclusively the middle ear structures and the mastoid bone. In rare cases it involves the external auditory canal. The author would like to present case report of a patient affected by external ear canal cholesteatoma associated to fibrous dysplasia of the temporal bone. The problems related to the pathogenesis and the diagnosis of the disease are presented and discussed.

Adult↗
PubMed1995

Increased expression of the c-fos proto-oncogene in bone from patients with fibrous dysplasia.

BACKGROUND: Fibrous dysplasia is characterized by intense marrow fibrosis and increased rates of bone turnover. The lesions of fibrous dysplasia resemble those described in the long bones of transgenic mice overexpressing the c-fos proto-oncogene. Activating mutations in the alpha subunit of the stimulatory guanine-nucleotide-binding protein (GS alpha) linked to adenylate cyclase have recently been described in bone cells from patients with the McCune-Albright syndrome and fibrous dysplasia. METHODS: We used in situ hybridization to determine the level of expression of c-fos in bone-biopsy specimens from two normal subjects, eight patients with fibrous dysplasia, and six patients with other bone disorders characterized by high rates of bone turnover. The probe used corresponded to the fourth exon of the c-fos gene. RESULTS: High levels of c-fos expression were detected in the bone lesions from all eight patients with fibrous dysplasia. No expression of c-fos was detected in bone specimens from the normal subjects or from specimens of normal bone obtained from patients with fibrous dysplasia. The cells that expressed c-fos in the dysplastic lesions were fibroblastic and populated the marrow space. A very low level of c-fos expression was detected in the biopsy specimens from the patients with other bone diseases. One patient with polyostotic fibrous dysplasia and one patient with the McCune-Albright syndrome were tested for the previously described GS alpha gene mutations and were found to express these mutations in bone. CONCLUSIONS: Increased expression of the c-fos proto-oncogene, presumably a consequence of increased adenylate cyclase activity, may be important in the pathogenesis of the bone lesions in patients with fibrous dysplasia.

Adolescent↗
PubMed1987

Polyostotic fibrous dysplasia and chronic osteomyelitis in a 12-year-old boy. Diagnostic difficulties in double bone pathology.

A twelve-year-old boy with double bone pathology (polyostotic fibrous dysplasia and chronic monofocal osteomyelitis), causing radiologic diagnostic difficulties is reported. The nuclear scan which was interpreted with disregard of the patient's history and physical examination lead to erroneous conclusions. Basic clinical signs and symptoms should always be taken into account when more sophisticated examinations are performed.

Child↗
PubMed1982

A case of monostotic fibrous dysplasia of the temporal bone associated with epileptic seizure.

An 11-year-old male with monostotic fibrous dysplasia of the left temporal bone was reported. At the age of seven years, the patient began having epileptic attacks, and a bony swelling of the left temporal region was noticed by his mother. Roentgenologically, there were almost thorough osseous obstruction and osseous proliferation of the external auditory canal and pars petrosa, respectively. Audiologic examinations indicated gradual functional disturbance based on the affected internal ear. A total of 20 cases with monostotic fibrous dysplasia of the temporal bone reported between 1946 and 1980 was analyzed, and the association of fibrous dysplasia and epilepsy was discussed.

Child↗
PubMed1998

[Effect of pamidronate on clinical symptoms and bone metabolism in fibrous dysplasia and McCune-Albright syndrome].

BACKGROUND: Patients with fibrous dysplasia and the McCune-Albright syndrome which is characterized by additional endocrine dysfunctions, such as pubertas precox, suffer from a regional impairment in the differentiation of osteoblasts that is acquired during early embryogenesis and results in fibrous bone lesions. These lesions may cause bone deformities, fractures and chronical pain in the affected skeletal regions. We here report about our experience with a systemic treatment of the bisphosphonate pamidronate. PATIENTS: We treated 3 patients with fibrous dysplasia and 5 patients with McCune-Albright syndrome over a cumulative period of 37 patient-years. RESULTS: In all patients who suffered from painful lesions, intravenous infusions of 60 mg pamidronate resulted in an improvement of pain that lasted up to 6 months. In 2 patients we also observed a reduction in the size of some of the osteolytic lesions. Side effects were limited to asymptomatical fever and a small decline in total serum calcium within the physiological limits during the infusions. CONCLUSION: Pamidronate appears to be an effective and well tolerable treatment option for patients with fibrous dysplasia.

Adolescent↗
PubMedDate not supplied

Fibrous dysplasia associated with an idiopathic bone cavity.

A patient with fibrous dysplasia of the maxilla had an associated idiopathic bone cavity (traumatic bone cyst). The idiopathic bone cavity was localized over the roots of the right maxillary second molar and mimicked periapical pathosis. The patient was inappropriately treated with root canal therapy.

Adult↗
PubMed1988

Fibrous dysplasia of the temporal bone.

A 38-year-old woman with fibrous dysplasia of the entire right half of the mandible and the right temporal bone is presented. She had a unilateral progressive conductive hearing loss and stenosis of the auditory canal complicated by a chronic external otitis, a hidden cholesteatoma, destruction of the ossicles and an impending facial palsy. Surgery was performed to create a new auditory canal, to eradicate the cholesteatoma and to decompress the facial nerve from the surrounding fibrous dysplasia.

Adult↗
PubMed1995

Fibrous dysplasia of the temporal bone: imaging findings.

Lichtenstein [1] in 1938 coined the term fibrous dysplasia to describe a disorder characterized by the progressive replacement of normal bone elements by fibrous tissue. Histopathologically, these lesions consist of an abnormal proliferation of fibrous elements intermixed with haphazardly arranged trabeculae of woven bone. The disease can involve any bone in the body. In the head and neck, the skull and facial bones are involved in 10-25% of cases of monostotic fibrous dysplasia and in 50% of the polyostotic variety. Involvement of the temporal bone, however, is relatively rare, and only 53 cases have been reported. The three major radiographic classifications of fibrous dysplasia are pagetoid, sclerotic, and cystic. Any of these types may involve the temporal bone and related structures, including the external canal, middle ear, jugular foramen, or, rarely, the otic capsule. In this essay, we illustrate the radiographic features of the disease based on our experience with seven cases, seen at our institution since 1977, of fibrous dysplasia involving the temporal bone.

Adult↗