Document Detail


Sclerosing bone dysplasias: review and differentiation from other causes of osteosclerosis.
MedLine Citation:
PMID:  22084176     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Sclerosing bone dysplasias are skeletal abnormalities of varying severity with a wide range of radiologic, clinical, and genetic features. Hereditary sclerosing bone dysplasias result from some disturbance in the pathways involved in osteoblast or osteoclast regulation, leading to abnormal accumulation of bone. Several genes have been discovered that, when disrupted, result in specific types of hereditary sclerosing bone dysplasia (osteopetrosis, pyknodysostosis, osteopoikilosis, osteopathia striata, progressive diaphyseal dysplasia, hereditary multiple diaphyseal sclerosis, hyperostosis corticalis generalisata), many of which exhibit similar pathologic mechanisms involving endochondral or intramembranous ossification and some of which share similar underlying genetic defects. Nonhereditary dysplasias include intramedullary osteosclerosis, melorheostosis, and overlap syndromes, whereas acquired syndromes with increased bone density, which may simulate sclerosing bone dysplasias, include osteoblastic metastases, Paget disease of bone, Erdheim-Chester disease, myelofibrosis, and sickle cell disease. Knowledge of the radiologic appearances, distribution, and associated clinical findings of hereditary and nonhereditary sclerosing bone dysplasias and acquired syndromes with increased bone density is crucial for accurate diagnosis. © RSNA, 2011.
Authors:
Lauren L Ihde; Deborah M Forrester; Christopher J Gottsegen; Sulabha Masih; Dakshesh B Patel; Linda A Vachon; Eric A White; George R Matcuk
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Radiographics : a review publication of the Radiological Society of North America, Inc     Volume:  31     ISSN:  1527-1323     ISO Abbreviation:  Radiographics     Publication Date:  2011 Nov 
Date Detail:
Created Date:  2011-11-15     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8302501     Medline TA:  Radiographics     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1865-82     Citation Subset:  IM    
Affiliation:
Department of Radiology, Keck School of Medicine, University of Southern California, 1500 San Pablo St, 2nd Floor Imaging, Los Angeles, CA 90033-5313.
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