  Fig 2. Radioraph of A, left and B, right elbow, showing bowing defernity of the ulna and radial head dislocation bilaterally Past 6 years, where as in the past the factures were commonplace, occurring every few months. His prognosis is somewhat guarded because of predisposition to fractures. Discussion Osteogensis imperfecta is inheritied mutation is two genetic loci coding for type I collagen-COLIA1 and COLIA2. Type I collagen is normally faund within bone, tendons, ligaments, teeth, and the sclera of the eye. There are 4 major cliniical considerations when diagnosing osteogensis imperfecta. The criteria are ostoporosis with an abnormally fragile skeleton, blue sclera, blue-gray to yellowish-brown opalescent (denetinogenosis imperfecta), and otosclerosis leading to presenile hearing loss. At least 2 of these fatures must be present for a positive diagnosis. Other common features incude ligamentous laxity, abnormal temperature regulation with episodic diaphoresis, easybruisbility of the skin, constipation premature vasclur calcification, and imppropriate euphoriat 5,8 system, leading to comp lactions of acrtic valve incompetence and dilation of the aortic root.6 Traditionally, this disorder has been subbivided into 2 types (congenita and tarda). The congenita form isassociated with the development of severe osseous fractures and defornity in utero. The skull is paper thin and soft. This type |