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At least 739 records · Page 41Linked to original sources
PubMed2005

Pathologic fractures in children.

Fractures through bone tumors are often difficult to treat. We reviewed our combined experience with this problem in children, as well as the existing literature, to formulate management guidelines. For this study, prospective databases (1987 to 2002) from three referral centers were screened for pathologic fractures occurring under the age of 14 years. One hundred five patients presented with fracture through unicameral bone cyst, nonossifying fibroma, fibrous dysplasia, aneurysmal bone cyst and osteosarcoma. Seventeen patients were excluded. The most common primary locations were the proximal humerus and proximal femur. Pathologic fracture through nonossifying fibroma had the best outcome; union occurred with nonsurgical treatment in all cases. Unicameral bone cyst required surgical treatment to avoid persistence of the cyst and refracture. However fracture healing was predictable without surgical treatment. Proximal femoral lesions tended to heal in malunion if not fixed surgically. Aneurysmal bone cyst required surgical treatment for the lesion to heal and to allow the fracture to heal as well. Percutaneous sclerotherapy may be the treatment of choice for many of these lesions. Fibrous dysplasia allows fracture healing with nonoperative therapy. Progressive deformity requires followup and surgical correction. Malignant lesions presenting a pathologic fracture are best managed by initial nonoperative therapy during investigation and neoadjuvant therapy when possible, followed by definitive treatment.

Adolescent↗
PubMed1996

Activating mutations of Gs protein in monostotic fibrous lesions of bone.

Activating mutations of the alpha chain of the heterotrimeric signal transducer Gs disrupt the inherent guanosine triphosphatase activity of the alpha chain, stimulate adenylyl cyclase, and can result in independent cell proliferation. Such mutations are identified in a number of endocrine disorders, including McCune-Albright syndrome, which is a triad of endocrinopathy, café au lait spots, and polyostotic fibrous dysplasia. The mutation in this syndrome is a missense point mutation in exon 8 that results in the substitution of either histidine or cysteine for arginine at position 201. Monostotic fibrous dysplasia is a nonhereditary isolated bone lesion. Other isolated bone lesions that share some cytologic and clinical similarities to fibrous dysplasia are osteofibrous dysplasia and aggressive fibromatosis involving bone. Four cases of monostotic fibrous dysplasia, four cases of aggressive fibromatosis involving bone, and one case of osteofibrous dysplasia were studied to determine if a mutation was present in exon 8 of the alpha chain of Gs. A missense mutation was present in all of the fibrous dysplasias. The other fibrous lesions and uninvolved tissue did not contain a mutation. Somatic activating mutations of Gs differentiate fibrous dysplasia from the other lesions and may be responsible for the loss of control of local proliferation and growth factor expression.

Adolescent↗
PubMed1984

Pituitary and ovarian abnormalities demonstrated by CT and ultrasound in children with features of the McCune-Albright syndrome.

In a random series of 97 children referred to the National Institutes of Health with a presumptive diagnosis of precocious puberty, eight girls were found to have features of the McCune-Albright syndrome, including fibrous dysplasia of bone and/or skin lesions resembling café au lait spots. Two girls had the classic triad. Radiographic evaluation of these patients included computed tomography of the head and pelvic ultrasound. The pituitary glands were suspicious for abnormality in five of the eight girls. Seven girls underwent pelvic ultrasound, and in all of them the ovaries were considered to be abnormal for their chronological age; in addition, two had functional ovarian cysts. The role of diagnostic radiological studies in the diagnosis of this syndrome is discussed.

Child↗
PubMed1981

[Cutaneous manifestations of McCune-Albright syndrome: report of a case (author's transl)].

An 11-year-old boy with fibrous dysplasia of bone of the McCune-Albright syndrome type led to a search of the literature for reports of cutaneous signs other than hyperpigmented macules. We have found descriptions of patchy alopecia, verrucous nevi, soft tissue tumours and other cutaneous abnormalities associated with the McCune-Albright syndrome. Our patient presented in addition pili torti, dermal cysts and a pre-auricular appendix which are all signs not previously described in this syndrome. Our observation is in keeping with the hypothesis that there is a congenital ectodermal and mesodermal mal development to account for this polydysplasia.

Alopecia↗
PubMed1986

The McCune-Albright syndrome.

The presence of polyostotic fibrous dysplasia of bone, hyperpigmented skin macules, and precocious sexual development in children is known as the McCune-Albright syndrome. To date, a complex combination of multiple endocrinopathies including goiter, hyperthyroidism, acromegaly, Cushing syndrome, hyperprolactinemia, sexual precocity, hyperparathyroidism, and hypophosphatemic hyperphosphaturic rickets have been described in association with this syndrome. Even though the pathogenetic mechanisms involved in the development of the endocrinopathies is unknown, it was assumed for many years that hypothalamic dysfunction was the cause in most cases. The overwhelming amount of data now permits the development of an alternate hypothesis; one of hyperfunctioning endocrine organs working with relative autonomy from hypothalamic control.

Adrenalectomy↗
PubMed1981

Electron microscopic studies of fibrous dysplasia.

Very few ultrastructural studies of fibrous dysplasia have been reported up to date. Eight cases of fibrous dysplasia (six cases of monostotic and two cases of polyostotic disease) were studied in detail by an electron microscope, and were compared with desmoplastic fibroma and fibrous cortical defect in order to clarify the fine structure of this disease. The fine structure of osteoblast or osteocyte-like cells, large cells including collagen fibers in the cytoplasm and so on was newly observed in this study. It was impossible to distinguish between monostotic and polyostotic forms even through an electron microscope.

Adolescent↗
PubMed1996

Cranio-facial fibro-osseous dysplasia. A case report and overview of the management of the condition.

Fibro-osseous dysplasia is a developmental non familial, benign anomaly of bone development occurring in single or multiple bones, characterised by the replacement of normal bone by fibro-osseous tissue. We describe the case of a fourteen year old boy, which illustrates many of the difficulties confronting surgeons dealing with this uncommon condition. Complete surgical excision was required, before a definitive histological diagnosis could be established. We report the clinical, radiological and pathological features of this unusual case. We review the pathology of the condition and discuss the approaches to surgical management.

Adolescent↗
PubMedDate not supplied

Common benign lesions of bone in children and adolescents.

Although benign bone neoplasms are relatively uncommon in children and adolescents, their discovery can produce great angst among providers and parents. In most but not all instances, imaging studies can indicate the likely benign nature of the tumor and allay fears. The clinical presentation, imaging, and contemporary management of five common types of tumor or tumor-like conditions of bone commonly seen in children and adolescents are reviewed: nonossifying fibroma, unicameral bone cyst, aneurysmal bone cyst, fibrous dysplasia, and exostosis.

Bone Cysts↗
PubMed1981

The Albright syndrome associated with acromegaly: report of a case and review of the literature.

The association of the Albright syndrome (polyostotic fibrous dysplasia of bone, hyperpigmented skin macules, and endocrine disorders) with acromegaly has been infrequently substantiated. The case of an 18-year-old girl with the classic Albright syndrome and acromegaly is described. The patient had a history of coarsening of acral and facial features, an insulin-resistant form of diabetes mellitus and elevated fasting growth hormone values. Neuro-endocrine studies demonstrated failure of growth hormone to suppress to less than 5 ng/ml during an oral glucose tolerance test, and the abnormal release of growth hormone upon injection of thyrotropin-releasing hormone. Although L-dopa failed to decrease growth hormone levels, bromocriptine produced a modest decline in growth hormone within two hours of ingestion. The patient had also experienced secondary amenorrhea with sub-normal follicle-stimulating-hormone (FSH) and luteinizing hormone (LH) levels, both of which demonstrated a prolonged sluggish response to an injection of gonadotropin-releasing hormone (GnRH); this response suggested hypogonadotropic hypogonadism, possibly on the basis of a tumor involving both pituitary and hypothalamus. Sellar polytomography demonstrated an enlarged sella with dorsal erosion and an asymmetric floor. Computerized tomography of the brain visualized a suprasellar mass extending into the hypothalamus. These findings suggest a hypersecretion of hypothalamic releasing factors, pituitary hormones, or both as an etiology for the endocrinopathy in this patient, and lend support to the theory that the endocrinopathies associated with the Albright syndrome result from over-production of hypothalamic-releasing hormones or autonomous secretion of pituitary hormones from an adenoma.

Acromegaly↗
PubMed2002

Bone scintigraphy as an adjunct for the diagnosis of oral diseases.

Bone scintigraphy is a very sensitive method for the detection of osteoblastic activity of the skeleton. The technique consists of imaging the uptake of bone-seeking radiopharmaceuticals, particularly technetium-99m labeled diphosphonates, in the mineral component of bone, which consists of hydroxyapatite crystals and calcium phosphate, as well as in the organic matrix such as collagen fibers. Plain radiographs, computed tomography, and magnetic resonance imaging are classified as structural imaging modalities, whereas bone scintigraphy is a functional method. In many cases, radionuclide imaging techniques are the only means by which early physiologic changes that are a direct result of biochemical alteration may be assessed, before significant bone mineral changes can be detected by other means. Since many oral diseases may cause metabolic changes in the oromaxillofacial complex, it would be of great value to use bone scintigraphy to evaluate more completely some conditions involving the bones in the region to formulate more appropriate treatment plans. Based upon the current literature, the authors discuss the possible applications of bone scintigraphy as a diagnostic and treatment planning adjunct for oral diseases. Bone scintigraphy has proven particularly useful in the study of malignant lesions and in the evaluation of vascularized bone grafts used for maxillofacial reconstructions.

Bone Diseases, Infectious↗