Journal article

"Baby rattle" pelvis dysplasia

V Cormier-Daire, R Savarirayan, RS Lachman, JA Neidich, K Grace, DL Rimoin, WR Wilcox

American Journal of Medical Genetics | WILEY-LISS | Published : 2001

Abstract

We report an apparently previously undescribed lethal skeletal dysplasia, clinically resembling achondrogenesis, but with distinct radiologic and chondro-osseous morphologic features. These comprise bifid distal ends of the long bones of the limbs, absent vertebral body ossification, a unique "baby rattle" pelvic configuration with tall and broad ilia, absent endochondral ossification, regions of mesenchymal cells within the resting cartilage, and abnormal mesenchymal ossification. © 2001 Wiley-Liss, Inc.

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