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Craniofacial fibrous dysplasia associated with primary hyperparathyroidism.

Isolated presentation of fibrous dysplasia or primary hyperparathyroidism is a common finding. Only few cases of craniofacial dysplasia associated with hyperparathyroidism have been reported in the literature. A case of a patient with fibrous dysplasia of craniofacial bones associated with primary hyperparathyroidism without additional endocrinophaties or associated disorders, will be presented. Beside the facial dysmorphism caused by dysplasia, the only clinical symptoms were due to the primary hyperparathyroidism.

Adult↗

Pathologic fracture of the femoral neck in a female soccer player.

We report a case of a healthy, young, female soccer player who developed progressive pain in her right hip. A bone cystic lesion was found in the right femoral neck and proximal femur. The lesion was considered a benign bone cyst and the patient was treated with injections of autologous bone marrow and grafting into the femoral neck. However, the cystic lesion did not heal. Subsequently, the patient fell and developed a fracture of the femoral neck that required internal fixation. The bone biopsy showed characteristic histologic features of fibrous dysplasia. A bone scan showed other areas of suspected dysplasia. Pamidronate therapy was given, and a reduction of the increased uptake was seen on bone scans. Fibrous dysplasia must be considered in the differential diagnosis of any cystic bone lesion.

Journal Article↗

Polyostotic fibrous dysplasia with acromegaly.

A 26-year-old man was found to have incomplete polyostotic fibrous dysplasia of bone and elevated growth hormone and prolactin levels. There was roentgenographic evidence of a pituitary tumor. The natural history of fibrous dysplasia in relationship to excess growth hormone secretion since childhood is described.

Acromegaly↗

[Initial clinical experience with BAS O, a bioactive glass-ceramic material].

The authors describe a group of patients where the surgical operation involved among others filling of bone defects with glass-ceramic material--dense (as granules) or porous (as a block). Glass-ceramics BAS O were developed in the laboratories of LASAK Co. in Prague. Two defects were of traumatic origin and osteosynthesis was part of three operations. The remaining defects were juvenile bone cysts, fibrous dysplasia and benign bone tumours. The follow-up period after operation varied in the first 11 patients between 6 months and 2 years. In patients of the mentioned group no problems of healing of the surgical wound were recorded nor allergic and side-reactions. The incorporation of glass-ceramic material was followed up by X-ray after three-months intervals. In no instance lighter areas were found on the X-ray pictures suggesting a fibrous outer layer. On the contrary, the trabeculae reached gradually its close vicinity. Based on experience from experimental work and investigation of X-ray signs of healing, the patients were allowed to burden the operated extremity after three months. The basic laboratory examinations made in these patients were within the normal range. In particular calcium throughout the investigation period in the normal range, the phosphorus levels varied near the upper borderline, alkaline phosphatase levels were in many young patients elevated and acid phosphatases varied. In eight patients during the postoperative period eosinophilia was revealed in the haemogram.

Adolescent↗

[Computer-assisted surgery (CAS) for the optimal treatment of craniofacial fibrous dysplasia].

BACKGROUND AND OBJECTIVE: The rare bone disease craniofacial fibrous dysplasia is only treated in cases of visible deformities of the splanchnocranium and neurocranium, compression syndromes, and delocation of nasal and paranasal drainage. METHODS AND PATIENTS: In a retrospective quality assessment analysis of six patients with craniofacial monostotic fibrous dysplasia, the indications were analysed for situations in which computer-assisted surgery (CAS) might be helpful. RESULTS AND CONCLUSIONS: CAS serves as an intraoperative basis for the assessment of cosmetic-aesthetic corrections of visible bone deformities of the splanchnocranium and neurocranium, and allows the controlled removal of pathologically affected bone in contralateral symmetry. This seems to be important, especially at the anterior skull base, as deformities of the human face produce strong psychological suffering. Furthermore, CAS allows the configuration of implants for defect reconstruction after focal restorations with bony radical surgery. Implants of various material can be prefabricated from these data, which can then be exactly fitted to cover the defect.

Adult↗

Extra-skeletal effects of bisphosphonates.

Bisphosphonates are pharmacological agents which are currently used both in osteoporosis than in other pathological conditions characterised by an increased bone resorption, such as Paget's disease of bone, malign hypocalcaemia during myeloma, osteolytic bone metastasis and fibrous dysplasia of bone. The most important biological effect of bisphosphonates is the reduction of bone remodelling through the inhibition of osteoclastic activity, but there are many clinical and experimental evidences of extra-skeletal biological effects of bisphosphonates. It has been shown that bisphosphonates exert their effects not only on bone tissue cells, but also on those of the immune system with an "immuno-modulating" effect, influencing the production of pro- and anti-inflammatory cytokines and changing the molecular expression involved in the immune processes and anti-inflammatory response. Although the available data are conflicting, there are several reports concerning the beneficial effects of bisphosphonates in controlling the progression of chronic joint inflammatory diseases, suggesting a wider use for these therapeutic agents in clinical practice.

Animals↗

[Primary clinical study on self-setting calcium phosphate cement in bone defect repair of extremities].

OBJECTIVE: To investigate the clinical application of self-setting calcium phosphate cement (CPC) in bone defect repair of extremities. METHODS: From May 1998 to January 2000, 32 cases of bone defect, in 36 sites, were repaired and reviewed, aged from 4 to 59 years old (24.7 years old on average), with bone defect 2 to 125 cm2 in size (13.1 cm2 on average). The causes of the bone defect were fracture, bone cyst, iliac bone harvesting, fibrous dysplasia, enchondroma and bone tuberculosis, which involved femur, iliac, tibia, humerus, phalanx, fibula, calcaneus, talus and acetabulum. All of the cases were followed up for 1 to 23 months, 15.3 months on average, before radiographic examination. RESULTS: All operations were successful and no general response was observed in all of the cases. X-ray examination showed an integrity interface between CPC and bone. And CT showed no gap existed. There was no increase of serum calcium and phosphate levels. CONCLUSION: CPC is applicable in the low- or non-weight-bearing site of the extremities.

Adult↗