Patients with Van Buchem Disease, an Osteosclerotic Genetic Disease, Have Elevated Bone Formation Markers, Higher Bone Density, and Greater Derived Polar Moment of Inertia than Normal

Abstract excerpt

Van Buchem disease is an autosomal recessive disease characterized by overgrowth of the skeleton. In a group of Dutch patients the disease is thought to be due to a 52-kb deletion that results in decreased expression of the SOST gene. To further characterize the disease, the morphology of the metacarpals of six adult subjects and two juveniles with Van Buchem disease were measured on hand x-rays along with nine...

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