Linked Life Data

1422977

Source:http://linkedlifedata.com/resource/pubmed/id/1422977

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
4
pubmed:dateCreated
1992-11-27
pubmed:abstractText
Light and electron microscopic studies of diastrophic dysplasia iliac crest growth cartilage performed on five occasions in two patients from 1 to 10 years of age reveal extensive cell and matrix abnormalities at each time period. Light microscopy shows atypical chondrocytes with extreme variation in size and shape, and premature cytoplasmic degeneration, and formation of target ghost cells. Prominent, densely staining fibrotic foci are present throughout the cartilage. Ultrastructure reveals some structurally intact chondrocytes with a single large fat inclusion, slightly dilated rough endoplasmic reticulum, and abundant glycogen. As early as 1 year of age cystic degeneration of chondrocyte cytoplasm is evident with indistinct organelles seen. The cartilage matrix demonstrates a general increase in fibrous tissue as well as the fibrotic foci. The collagen in these foci is remarkably abnormal. It is composed of short, extremely broad fibrils ranging from 150 to 950 nm in width which are separated at their terminal ends but fused to each other centrally in random fashion. On cross-section there are very few round fibrils but rather a marked irregularity in shape giving the appearance of having fibrils randomly added to others to form enlarged nonuniform fibril aggregates. On longitudinal sectioning, regular cross-banding across the entire fibril width is seen but fibril splitting and aggregation are highly irregular.(ABSTRACT TRUNCATED AT 250 WORDS)
pubmed:grant
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:status
MEDLINE
pubmed:month
Oct
pubmed:issn
0171-967X
pubmed:author
pubmed:issnType
Print
pubmed:volume
51
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
324-31
pubmed:dateRevised
2007-11-14
pubmed:meshHeading
pubmed:year
1992
pubmed:articleTitle
Light and electron microscopic abnormalities in diastrophic dysplasia growth cartilage.
pubmed:affiliation
Department of Orthopaedic Surgery, Children's Hospital, Harvard Medical School, Boston, Massachusetts 02115.
pubmed:publicationType
Journal Article, Research Support, U.S. Gov't, P.H.S.