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At least 361 records · Page 20Linked to original sources
PubMed2003

Effect of intravenous pamidronate on bone markers and local bone mineral density in fibrous dysplasia.

Bisphosphonates have proven to be effective in patients with fibrous dysplasia of the bone (FD) as shown by their effect on bone pain, markers of bone turnover, or radiological changes. The aim of this study was to evaluate the usefulness of measuring bone mineral density (BMD) of affected bones to assess the efficacy of bisphosphonate treatment. Seven patients (mean age 26 years) received courses of 180 mg intravenous infusion of pamidronate every 6 months (60 mg/day during 3 days). Clinical symptoms, serum alkaline phosphatase, and urinary C-terminal cross-linking telopeptide of type I collagen were assessed every 3 months. BMD of total skeleton and X-rays of FD areas (FDa) were performed at baseline and at 12 months. BMD of FDa was compared with the contralateral side (CL) using the region of interest program on the total skeleton scan. BMD of total skeleton was normal at baseline. Average BMD of FDa was -11.4% compared with CL, a significantly greater difference than that observed between the left and right sides in healthy controls, -0.7% (P < 0.02). At 12 months bone pain diminished in all patients. Bone turnover markers decreased. Mean total skeleton BMD increased 3.3% (P < 0.02). Subregions of the total skeleton scan presenting FD lesions augmented: arms +9.6% (P < 0.02), legs +4.2%, and pelvis +3.5% (P < 0.05). The increase in mean BMD of FDa was +6.8% compared with +2.6% in CL. No changes were observed on the X-ray. These results indicate that simultaneous determination of markers of bone turnover and BMD of FDa is useful in short-term follow-up to determine the efficacy of intravenous pamidronate.

Adolescent↗
PubMed1990

Fluid-fluid level: a nonspecific finding in tumors of bone and soft tissue.

Fluid-fluid levels have commonly been reported to occur in aneurysmal bone cysts but have also been seen in telangiectatic osteosarcoma, chondroblastoma, and giant cell tumor of bone. The authors reviewed their experience with nine bone and three soft-tissue tumors that showed fluid-fluid levels on computed tomographic or magnetic resonance images. The bone tumors included fibrous dysplasia, simple bone cyst, recurrent malignant fibrous histiocytoma of bone, two classical osteosarcomas, and four aneurysmal bone cysts. The soft-tissue tumors included soft-tissue hemangioma and two synovial sarcomas. Except for aneurysmal bone cysts, these types of tumors have not been reported to be associated with fluid-fluid levels. Radiologic-pathologic correlation was available in seven patients; in all seven, the fluid-fluid levels indicated prior hemorrhage. The authors conclude that the presence of fluid-fluid levels in bone or soft-tissue tumors cannot be considered diagnostic of any particular tumor.

Adolescent↗
PubMed1978

[The aid of bone scans in diagnosis of fibrous dysplasia (author's transl)].

Fibrous dysplasia of the bone does not only occur in childhood as commonly believed, but may also be diagnosed during adult life as a stepwise progressing disease. Bone scans are useful to differentiate active from non-active disease. Suspicious accumulations of the radio-pharmaceutical which may be detected by total body scanning, should be confirmed by roentgen examination as a more specific method. Bone scan is helpful in early detection of focal disease and may visualize lesions in wide-spread disease for further radiological workup.

Age Factors↗
PubMed1993

Polyostotic coexisting fibrous dysplasia and aneurysmal bone cyst of the chest wall.

A case of a 39-year-old woman with a palpable mass in the right hemithorax is presented. The mass had been growing during the last 16 years. Radiographs and computed tomography showed two lesions in the right thoracic wall: the greater was in the anterior and lateral portion of the 7th rib, the minor lesion in the 6th rib costovertebral joint. Both lesions were surgically removed. Histological examination demonstrated the association of fibrous dysplasia and aneurysmal bone cyst in the two lesions. The coexistence of these two lesions supports the theory that aneurysmal bone cyst may represent a secondary change due to haemodynamic alterations of the vascular bed caused by fibrous dysplasia.

Adult↗
PubMed1987

Neurofibromatosis and Albright's syndrome.

For both NF and Albright's syndrome, the pathogenetic relationships between the various elements of the respective syndromes remain a mystery, and the importance of nontumorous endocrine abnormalities in neurofibromatosis has, in my opinion, been overstated. Nonetheless, the parallel between the two disorders is striking, and the consistent occurrence of skin and skeletal dyplasias in both suggests that a search for the fundamental defect in either can be advanced by focusing on what is common to these two types of tissue. For example, how does one reconcile a disturbance of the melanosome with skeletal aberrations? An answer to that question will put us on the right track. One final question must be asked. Are neurofibromatosis and Albright's syndrome alternate manifestations of the same disorder? In a trivial sense, the answer may be yes. That is, in at least some instances, one condition may have been mistaken for the other, and thus a factitious overlap or "sameness" misconstrued. Upon closer scrutiny, however, there are two important points that would cast doubt on a positive response to this question: Neurofibromatosis, or at least neurofibromatosis I, is heritable as an autosomal dominant trait, whereas heritability has not been documented for Albright's syndrome; and I am unaware of reported cases that describe both disorders, diagnosed by criteria beyond café au lait spots and bone dysplasia, in one and the same individual, although such a case has been shared with me by S. A. Sorensen, M.D., of the Genetics Institute of Copenhagen, Denmark. The full reporting of such a case would be of great interest.

Fibrous Dysplasia of Bone↗
PubMed2004

A novel technique based on a PNA hybridization probe and FRET principle for quantification of mutant genotype in fibrous dysplasia/McCune-Albright syndrome.

Somatic mutations are present in various proportions in numerous developmental pathologies. Somatic activating missense mutations of the GNAS gene encoding the Gs(alpha) protein have previously been shown to be the cause of fibrous dysplasia of bone (FD)/McCune-Albright syndrome (MAS). Because in MAS patients, tissues as diverse as melanocytes, gonads and bone are affected, it is generally accepted that the GNAS mutation in this disease must have occurred early in development. Interestingly, it has been shown that the development of an active FD lesion may require both normal and mutant cells. Studies of the somatic mosaic states of FD/MAS and many other somatic diseases need an accurate method to determine the ratio of mutant to normal cells in a given tissue. A new method for quantification of the mutant:normal ratio of cells using a PNA hybridization probe-based FRET technique was developed. This novel technique, with a linear sensitivity of 2.5% mutant alleles, was used to detect the percentage mutant cells in a number of tissue and cell culture samples derived from FD/MAS lesions and could easily be adapted for the quantification of mutations in a large spectrum of diseases including cancer.

Alleles↗
PubMed1986

Treatment of precocious puberty in the McCune-Albright syndrome with the aromatase inhibitor testolactone.

The McCune-Albright syndrome is characterized by café au lait spots, fibrous dysplasia of bones, and sexual precocity. Girls with precocious puberty due to this syndrome have episodic increases in serum estrogen levels together with the formation of large ovarian cysts. The serum gonadotropin levels are typically suppressed, and the precocious puberty has not responded to treatment with long-acting analogues of luteinizing hormone-releasing hormone (LHRH). Encouraged by our initial success in a pilot study of one patient, we have now treated five girls with the McCune-Albright syndrome with the aromatase inhibitor testolactone, which blocks the synthesis of estrogens. Testolactone decreased the levels of circulating estradiol (P less than 0.05) and the ovarian volume (P less than 0.05), and there was a return to pretreatment levels after testolactone was stopped. During treatment, the peak responses of luteinizing hormone and follicle-stimulating hormone to stimulation by LHRH rose above suppressed pretreatment levels--significantly above pretreatment levels for follicle-stimulating hormone (P less than 0.02)--and then returned to pretreatment levels after testolactone was discontinued. Growth rates fell in three patients during treatment but could not be assessed in the other two because of bone deformities. The mean rate of bone maturation decreased and menses stopped in three of the four girls who were menstruating regularly. We conclude that testolactone is an effective treatment of precocious puberty in the McCune-Albright syndrome.

Aromatase Inhibitors↗