From Big Medical Encyclopedia

CHONDROGENESIS IMPERFECT (chondrogenesis imperfecta; Greek chondros a cartilage - f genesis origin) — the inborn disturbance of a skeletogeny caused by the wrong formation of cartilaginous tissue. At the same time it is observed patol. development of bones only of a cartilaginous origin. Growth of bones of a desmalny origin, napr, bones of a skull, is not broken.

The etiology is not found out. According to a number of researchers, at the heart of X. the N, as well as an imperfect desmogenez and bone formation (see Desmogenez imperfect, Bone formation imperfect), lies the general genetic defect of a mesenchyma (see).

At morfol. a research in it is excessive the developed cartilaginous tissue (see) the abundance of vascular channels is noted that, according to A. V. Rusakova^ causes strengthening of metabolism and growth of cartilaginous tissue leading to death of cartilaginous cells and interstitial substance. The strengthened growth and bystry aging of cartilaginous substance are followed by excess replenishment its insufficiently mature cartilaginous tkanyo. The excess amount and at the same time softness of pathological cartilaginous substance lead to disturbance of development of a bone tissue and deformation of bones.

See also Chondrodysplasia.

G. I. Lavrshtseva.