Kniest dysplasia. A histochemical study of the growth plate.
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.
D000293:Adolescent; D000328:Adult; D001848:Bone Diseases, Developmental / Q000378:metabolism* / Q000473:pathology; D001842:Bone and Bones / Q000378:metabolism*; D002356:Cartilage / Q000378:metabolism* / Q000648:ultrastructure; D002357:Cartilage Diseases / Q000378:metabolism* / Q000473:pathology; D002648:Child; D002809:Chondroitin Sulfates / Q000378:metabolism; D003094:Collagen / Q000378:metabolism; D005260:Female; D006801:Humans; D006984:Hypertrophy; D007632:Keratan Sulfate / Q000378:metabolism; D011509:Proteoglycans / Q000378:metabolism; D013577:Syndrome
D011509:Proteoglycans; D002809:Chondroitin Sulfates; D003094:Collagen; D007632:Keratan Sulfate