Linked Life Data

2803859

Source:http://linkedlifedata.com/resource/pubmed/id/2803859

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
4
pubmed:dateCreated
1989-12-21
pubmed:abstractText
The osteoid of a patient with Fibrogenesis Imperfecta Ossium is described. Three iliac crest biopsies were taken; firstly before treatment, secondly after calcitriol therapy and finally after successful treatment with melphalan and prednisolone. In the pretreatment biopsy the osteoid was greatly enlarged, showed complete absence of the birefringence characteristic of oriented collagen fibers, and at ultrastructural level was shown to be composed of abnormal collagen fibrils. The fibrils were often curved and were extremely variable in thickness. Calcification within the osteoid took the form of calcospherites and spread of calcification from these to collagen fibrils was greatly delayed. In the second biopsy two aspects of osteoid ultrastructure were noted; some samples resembled the first biopsy, but others had a different organization. The osteoid of these samples had two regions: an inner region containing abnormal collagen fibrils and an outer region composed of moderately electron-dense amorphous material. The osteoblasts associated with this region were clearly highly biosynthetically active. The third biopsy, after treatment with Melphalan and prednisolone, showed a reversion to more normal bone ultrastructure with uniform, oriented collagen fibrils and prompt mineralization resulting in narrow osteoid seams. Remnants of the original abnormal osteoid were present in the marrow space as calcified debris. Reasons for the success of this therapeutic regime are unclear; however, some speculation is made as to the possible roles of the cytotoxic drug and the glucocorticoid in the regression of this condition.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:issn
8756-3282
pubmed:author
pubmed:issnType
Print
pubmed:volume
10
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
243-9
pubmed:dateRevised
2004-11-17
pubmed:meshHeading
pubmed:year
1989
pubmed:articleTitle
Ultrastructural features of the osteoid of patients with fibrogenesis imperfecta ossium.
pubmed:affiliation
Institute of Orthopaedics (University of London), Royal National Orthopaedic Hospital, Stanmore, Middlesex, England.
pubmed:publicationType
Journal Article, Case Reports