Etiological diversity of connective tissue dysplasia 

S.L. Pervykh, E.I. Karpova 

Pirogov Russian National Research Medical University, Moscow 

Morphological abnormalities in all types of connective tissue dysplasia (CTD) result in various clinical manifestations. This paper reviews the histological basis for changes in the matrix, fibers, and cells of connective tissue and their interpretation for clinical practice. The mechanism of extracellular formation of various fibrous matrix components and their involvement in CTD development is discussed. The role of ions of the matrix and activity of tissue enzymes in the development of clinical manifestations of some CTD types are addressed. Interstitial processes of the generation of fibrous matrix and gel-like substance of connective tissue containing proteoglycans are controlled and regulated by a complex, branched, multilevel system. Despite the leading role of genetic factors, epigenetic factors contribute greatly to the clinical manifestations of CTD. In particular, the effect of polylactic acid-based substrates is now poorly understood. This paper also analyzes the mechanisms involved both in the development of clinical manifestations and potential prevention of CTD.

Keywords: connective tissue dysplasia, matrix, collagen, fibroblast, polylactic acid, lactate.

For citation: Pervykh S.L., Karpova E.I. Etiological diversity of connective tissue dysplasia. RMJ. 2023;6:13–15.