{"id":7613,"date":"2013-03-03T23:22:24","date_gmt":"2013-03-04T04:22:24","guid":{"rendered":"http:\/\/insidesurgery.com\/?p=7613"},"modified":"2013-03-04T23:05:01","modified_gmt":"2013-03-05T04:05:01","slug":"osteogenesis-imperfecta","status":"publish","type":"post","link":"https:\/\/insidesurgery.com\/2013\/03\/osteogenesis-imperfecta\/","title":{"rendered":"Osteogenesis Imperfecta"},"content":{"rendered":"
1) osteogenesis imperfecta is a congenital disorder of Type I collagen with expression chiefly as brittle bones
\n2) sometimes can affect joints, ligaments, sclerae, teeth, ears, and skin
\n3) four clinical types have been described<\/p>\n
<\/p>\n
1) multiple fractures are hallmarks of the disease
\n2) highly variable clinically, even among family members<\/p>\n
Type 1<\/p>\n
3) normal-appearing newborn with multiple fractures apparent in first year of life, flat feet, joint laxity, and blue sclerae<\/p>\n
4) hearing loss as middle ear bones fracture and fuse
\n5) bluish yellow teeth that have misshapen contours
\n6) fracture frequency decreases as child ages
\n7) patient can reach normal stature and have normal life span<\/p>\n
Type 2<\/p>\n
8) fatal in utero or neonatal period owing to respiratory failure<\/p>\n
Type 3<\/p>\n
9) short stature at birth owing to fractures occurring in utero, abnormal dentition, hearing loss, blue sclera at birth that later become white<\/p>\n
Type 4<\/p>\n
10) similar to Type 1 but with normal sclerae and more variable symptoms<\/p>\n
Radiology<\/em><\/p>\n bones in osteogenesis imperfecta are thin and curvy<\/p>\n