Paget's disease and fibrous dysplasia. 1991

S Wallach
University of South Florida College of Medicine, Tampa.

Many papers were published on both Paget's disease and fibrous dysplasia during the past year. In Paget's disease, evidence for a generalized, probably viral disorder of the skeleton has been adduced, although focal radiologic features dominate the clinical picture. Unusual clinical manifestations were highlighted in several clinical reports. A search for biochemical abnormalities other than increased serum alkaline phosphatase and urinary hydroxyproline levels yielded evidence for secondary hyperparathyroidism in many cases, and also, a confusing array of abnormalities in vitamin D metabolite levels. The application of newer imaging techniques such as computed tomography, MR imaging, bone marrow scintigraphy, and thermography was reported. The year's reports particularly highlighted new forms of effective therapy, including intranasal calcitonin, second- and third-generation bisphosphonates, and gallium nitrate. Finally, the feasibility of joint replacement in arthritic joints secondary to Paget's disease was again documented. Fibrous dysplasia continued to be an enigmatic disorder with no new insights as to etiology. Reports of unusual clinical features, imaging characteristics, bony distribution, and an array of endocrine linkages were prominent. A highlight of the year's reports was the discovery of an increased female sex steroid receptor number of dysplastic cells, and the possibility that sex steroids linked to their receptors may be responsible for the bony overgrowth. Concern was again expressed as to the possibility of malignant transformation of dysplastic lesions and the possible contribution of radiotherapy treatment to sarcoma development.

UI MeSH Term Description Entries
D010001 Osteitis Deformans A disease marked by repeated episodes of increased bone resorption followed by excessive attempts at repair, resulting in weakened, deformed bones of increased mass. The resultant architecture of the bone assumes a mosaic pattern in which the fibers take on a haphazard pattern instead of the normal parallel symmetry. Paget's Disease of Bone,Osseous Paget's Disease,Paget Disease of Bone,Paget Disease, Bone,Pagets Disease, Bone
D002116 Calcitonin A peptide hormone that lowers calcium concentration in the blood. In humans, it is released by thyroid cells and acts to decrease the formation and absorptive activity of osteoclasts. Its role in regulating plasma calcium is much greater in children and in certain diseases than in normal adults. Thyrocalcitonin,Calcitonin(1-32),Calcitrin,Ciba 47175-BA,Eel Calcitonin,Calcitonin, Eel,Ciba 47175 BA,Ciba 47175BA
D004700 Endocrine System Diseases Pathological processes of the ENDOCRINE GLANDS, and diseases resulting from abnormal level of available HORMONES. Endocrine Diseases,Diseases of Endocrine System,Disease, Endocrine,Disease, Endocrine System,Diseases, Endocrine,Diseases, Endocrine System,Endocrine Disease,Endocrine System Disease,System Disease, Endocrine,System Diseases, Endocrine
D005357 Fibrous Dysplasia of Bone A disease of bone marked by thinning of the cortex by fibrous tissue containing bony spicules, producing pain, disability, and gradually increasing deformity. Only one bone may be involved (FIBROUS DYSPLASIA, MONOSTOTIC) or several (FIBROUS DYSPLASIA, POLYOSTOTIC). Osteitis Fibrosa Disseminata,Fibrocartilaginous Dysplasia of Bone,Fibrocystic Dysplasia of Bone,Jaffe-Lichtenstein Disease,Bone Fibrocartilaginous Dysplasia,Bone Fibrocartilaginous Dysplasias,Bone Fibrocystic Dysplasia,Bone Fibrocystic Dysplasias,Bone Fibrous Dysplasia,Bone Fibrous Dysplasias,Jaffe Lichtenstein Disease
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D014807 Vitamin D A vitamin that includes both CHOLECALCIFEROLS and ERGOCALCIFEROLS, which have the common effect of preventing or curing RICKETS in animals. It can also be viewed as a hormone since it can be formed in SKIN by action of ULTRAVIOLET RAYS upon the precursors, 7-dehydrocholesterol and ERGOSTEROL, and acts on VITAMIN D RECEPTORS to regulate CALCIUM in opposition to PARATHYROID HORMONE.

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