L. Butani et al., AN UNUSUAL RENAL COMPLICATION IN A PATIENT WITH OSTEOGENESIS IMPERFECTA, American journal of kidney diseases, 25(3), 1995, pp. 489-491
Osteogenesis imperfecta (OI) is a common 'rare' disorder with a report
ed incidence of 1/15,000 to 1/20,000 in newborns. Skeletal abnormaliti
es in this condition are so striking that its equally important and di
verse extraskeletal manifestations frequently remain unnoticed, Sensor
ineural deafness, blue sclerae, myopia, easy bruisability, dental anom
alies, and floppy mitral valves are some of its more frequently report
ed extraskeletal features, Other less common features include pulmonar
y hypoplasia, joint contractures, hydrocephalus, and osteogenic sarcom
a.(1-3). Our patient, a 16-year-old boy, developed a previously unrepo
rted complication: chronic renal failure, which on subsequent evaluati
on was attributed to obstructive uropathy secondary to bony pelvic out
let deformities. (C) 1995 by the National Kidney Foundation, Inc.