KNIEST DYSPLASIA - HISTOCHEMICAL-STUDY OF THE GROWTH PLATE

被引:21
作者
HORTON, WA
RIMOIN, DL
机构
[1] Division of Medical Genetics, Harbor-UCLA Medical Center, Torrance, CA
关键词
D O I
10.1203/00006450-197911000-00012
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
Chondro-osseous tissue from four patients with the Kniest dysplasia was studied histochemically using a new plastic embedding technique. Extensive vacuolar changes were observed p—1 throughout the endochondral growth plate and adjacent resting cartilage. These changes occurred within the cartilage matrix and also in the lacunae of degenerating chrondrocytes. The septa of the lesions contained chondroitin sulfate, but little keratan sulfate or collagen. Resting cartilage not adjacent to the growth plate stained irregularly and showed few of the vacuolar lesions, and chondrocytes were enlarged and contained cytoplasic inclusions, but no vacuolar material. Thus, there appears to be a sequence of events initiated by cellular accumulation of a substance and progressing to cellular and matrix degeneration. Speculation: The findings suggest that there may be a defect in the synthesis, structure, or secretion of a major cartilage matrix component (e.g., proteoglycan, collagen) which leads to its accumulation in the chondrocyte rough endoplasmic reticulum. Both cellular and matrix degeneration subsequently occur either due to a toxic effect of this material or to the absence of the normal molecule. © 1979 International Pediatric Research Foundation, Inc.
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页码:1266 / 1270
页数:5
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