Morphological changes in growth-plate cartilage in osteogenesis imperfecta

• Published in: Journal of bone and joint surgery. British volume Vol. 72; no. 3; pp. 475 - 479
• Main Authors: SANGUINETTI, C, GRECO, F, DE PALMA, L, SPECCHIA, N, FALCIGLIA, F
• Format: Journal Article
• Language: English
• Published: London British Editorial Society of Bone and Joint Surgery 01.05.1990
• Subjects: Biological and medical sciences; Diseases of the osteoarticular system; Growth Plate - pathology; Humans; Malformations and congenital and or hereditary diseases involving bones. Joint deformations; Medical sciences; Osteogenesis Imperfecta - pathology; Human; Pathology; Epiphyseal cartilage; Pathogenesis; Collagen; Diseases of the osteoarticular system; Osteogenesis imperfecta; Glycosaminoglycan; Histology; Osteochondrodysplasia
• ISSN: 0301-620X; 2044-5377
• DOI: 10.1302/0301-620x.72b3.2187879

We obtained specimens of growth-plate cartilage from four patients with osteogenesis imperfecta. Light microscopy showed structural changes in the tissue and morphological changes in chondrocytes and matrix, particularly in the hypertrophic zone. There were changes in the process of calcification in the primary mineralisation zone of the cartilage. We also found histochemical changes in the matrix glycosaminoglycans (GAGs) in the zones where physiological mineralisation was disturbed and where the trabeculae were interrupted and poorly mineralised. In addition to the known molecular defects in collagen, changes in GAGs and non-collagenous proteins are important factors in the pathogenesis of the disease.