Late imperfect osteogenesis in obstetrics and gynecology practice
F. A. Miftakhova , E. R. Osipova , D. P. Ignatieva
Kazan medical journal ›› 1987, Vol. 68 ›› Issue (5) : 389 -390.
Late imperfect osteogenesis in obstetrics and gynecology practice
Imperfect osteogenesis (Lobstein disease, Wrolick disease, etc.) is a hereditary disease transmitted both autosomal dominant and autosomal recessive and characterized by impaired bone formation. It is clinically manifested by blue sclerae, discolored tooth enamel ("amber" teeth), loose joints, muscle atrophy, and increased bone fragility. Subcostal fractures with little local pain and swelling are predominantly seen in this disease. They can be complete and incomplete, often fused at an angle, which leads to deformities of limbs and thorax in the form of disproportionately short arms and legs while the head, trunk, and feet are normal in size. The phalanges of the fingers are never broken. The configuration of the skull is characterized by a broad forehead bulging forward and steeply upward, the temporal bones overhanging the ear canals; the ears are protruding downward and outward.
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