PubMed Health⌕ Search

SEARCH · PubMed Health

Results for “FIBROUS DYSPLASIA OF BONE”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 847 records · Page 47Linked to original sources
PubMed1976

Benign bone tumors.

There is little information in the literature concerning the role of bone scanning in benign bone neoplasms except for sporadic reports. Since the advent of 99mTc-polyphosphate, bone imaging has proven feasible and useful in locating the cause of bone pain, such as in osteoid osteomas, which are not always radiologically apparent, and in evaluating whether or not a radiologic lesion is indeed benign and solitary. Blood-pool images are particularly important in neoplastic disease, since the absence of hyperemia in the immediate postinjection period favors the diagnosis of a benign neoplasm, as does low-grade uptake on the delayed study. The scan, including pinhole magnification images, is especially valuable in diagnosing lesions in the spine and pelvis, which are poorly seen radiologically. We have studied various types of benign bone tumors, including simple and aneurysmal bone cysts, fibrous cortical defects, and nonossifying fibromas, all of which had minimal or no increased uptake of the radiopharmaceutical, unless traumatized. Although osteochondromas and enchondromas showed varied accumulation of activity, the scan was useful in differentiating these from sarcomatous lesions. All osteoid osteomas demonstrated marked activity, and could be accurately located preoperatively, as could the extent of fibrous dysplasia. The bone scan in the reticuloses also showed abnormal accumulation of activity, and aided in arriving at the prognosis and treatment of histiocytic bone lesions.

Bone Cysts↗
PubMed1989

Contraceptive methods in the McCune-Albright syndrome.

We describe the case of a woman with McCune-Albright syndrome who had a pathological bone fracture while being treated with an oral contraceptive. In this syndrome the bone lesions contain estrogen and progesterone receptors. The possibility of progression of the bone lesions during pregnancy is well-known. We judge the use of oral contraceptives to be dangerous in this syndrome; the affected women must be orientated towards alternative contraceptive methods.

Adult↗
PubMed2001

Cellular and molecular basis of fibrous dysplasia.

Recent advances have been made in the cellular and molecular mechanisms involved in monostotic and polyostotic fibrous dysplasia, a rare nonmalignant disease causing bone deformations and fractures. The molecular basis of fibrous dysplasia has been clarified when mutations affecting the stimulatory alpha subunit of G protein (Gs) have been found in dysplastic bone lesions. The histological analysis of dysplastic lesions revealed that the mutations in Gsalpha caused abnormalities in cells of the osteoblastic lineage and therefore in the bone matrix. Further in vitro analyses of bone cells from mutant dysplastic bone lesions revealed that the abnormal deposition of immature bone matrix in fibrous dysplasia results from decreased differentiation and increased proliferation of osteoblastic cells. Finally, the signaling pathway involved in these osteoblastic abnormalities has been identified. It is now apparent that the constitutive elevation in cAMP level induced by the Gsalpha mutations leads to alterations in the expression of several target genes whose promoters contain cAMP-responsive elements, such as c-fos, c-jun, Il-6 and Il-11. This in turn affects the transcription and expression of downstream genes and results in the alterations of osteoblast recruitment and function in dysplastic bone lesions. These mechanisms provide a cellular and molecular basis for the alterations in bone cells and bone matrix in fibrous dysplasia.

Adenylyl Cyclases↗
PubMed1989

Cystic degeneration of fibrous dysplasia masquerading as sarcoma.

An area of fibrous dysplasia of bone may undergo rapid enlargement which may be due to either cystic degeneration or malignant transformation. These complications may be clinically and radiologically indistinguishable and, unless both are borne in mind, incorrect management may follow. Magnetic resonance imaging was used in one of our cases and was the only imaging modality to demonstrate the true nature of the condition.

Adult↗
PubMed2003

Tamoxifen treatment for precocious puberty in McCune-Albright syndrome: a multicenter trial.

OBJECTIVE: We undertook a 1-year multicenter trial of tamoxifen treatment for precocious puberty in girls with McCune-Albright syndrome (MAS). STUDY DESIGN: Girls < or =10 years with classic or atypical MAS were recruited. Pretreatment history was collected for 6 months. Patients received 20 mg tamoxifen daily. Diaries were used to record bleeding. Evaluations included physical examination, bone age, pelvic ultrasound, hormone levels, and safety assessments. RESULTS: A total of 28 girls (2.9-10.9 years of age) were enrolled from 20 centers, of whom 25 completed 12 months of tamoxifen treatment. Compared with before the study, vaginal bleeding episodes decreased (3.42+/-3.36/year vs 1.17+/-1.41/year), growth velocity slowed (SDS 1.22+/-2.65 vs -0.59+/-3.06, P=.005), and rate of bone maturation decreased (1.21+/-0.78 vs 0.72+/-0.36, P=.02). Ovarian volumes were enlarged and asymmetric throughout the study, and uterine volumes were increased. No adverse events occurred. CONCLUSIONS: Tamoxifen treatment of precocious puberty in MAS results in a reduction of vaginal bleeding and significant improvements in growth velocity and rate of skeletal maturation.

Cafe-au-Lait Spots↗
PubMed1984

Absence of pubertal gonadotropin secretion in girls with McCune-Albright syndrome.

Precocious puberty in girls with McCune-Albright syndrome has been attributed in some cases to early activation of the hypothalamic-pituitary-gonadal axis and in other cases to sex steroid secretion by apparently autonomous ovarian cysts. We evaluated serum gonadotropins and sex steroids in six girls (aged 1-9 yr) with McCune-Albright syndrome. The children had Tanner stage II-IV pubertal development. In five patients, nocturnal gonadotropin concentrations and the gonadotropin response to LHRH were within the normal range for prepubertal children. Thus, the precocious puberty in these patients could not be explained by activation of the hypothalamic-pituitary-ovarian axis. One child had high amplitude nocturnal pulses of serum LH and a LH-predominant response to LHRH. She was the oldest of the six girls and had a bone age of 13.5 yr which is within the range in which hypothalamic-pituitary-ovarian activation normally occurs. The children all had ovarian enlargement and ovarian cysts determined by ultrasound. It appears that precocious puberty in McCune-Albright syndrome may result from ovarian estrogen secretion in the absence of normal pubertal activation of the hypothalamic-pituitary-ovarian axis.

Adolescent↗
PubMed1986

McCune-Albright syndrome. Long-term follow-up.

This article describes clinical follow-up of 15 patients--13 females and two males--with McCune-Albright syndrome. Osseous fractures occurred only during childhood, while hearing impairment due to temporal bone involvement occurred in four of six adults. Four females with precocious puberty had final heights that were not different from the mean for normal females; they eventually developed regular menses, and two had children. Persistent hyperthyroidism requiring ablative therapy occurred in three subjects, while hypophosphatemia occurred in three subjects. The protean manifestations of this disorder suggest that it results from a basic defect of cellular regulation. We postulate that its varied endocrine abnormalities result from altered regulation of intracellular cyclic adenosine monophosphate effects.

Acromegaly↗
PubMed1993

[Fibrous dysplasia of jaw and facial bones:A combined analysis of clinic,radiologic and histopathologic diagnosis of 117 cases]

A clinical-radiologic-histopathologic review of 106 cases of fibrous dysplasia involving the jaws is presented. Ninety eight patients onset in the first and second decades.Fifteen cases were polyostotic and the remaining cases were were monstotic.Radiologicaly,the lesions showed and extending growth pattern,poor definite border and slight pattern,poor definite border and slight involvement of teeth.The radiographic features were classified into four types:ground grass (52.0%),sclerotic(8.0%),cystic-like(8.0%) and mixed type (32.0%).Histologically the lesions were characterized by a cellular fibrous stroma that contain varying amounts of mineralized substance,represented by woven bone(63.0%),lamellar bone(30.0%) and cementum-like structure(7.0%).The final definitive diagnosis requires the combined evaluation of clinic,radiologic and histopathologic findings.

Journal Article↗