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August 20, 1938

La maladie de Lobstein: Le syndrome des Sclérotiques bleues dans ses rapports avec la fragilité osseuse et l'hérédo-syphilis

JAMA. 1938;111(8):746-747. doi:10.1001/jama.1938.02790340082037

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Abstract

This is a monograph on the syndrome of blue sclerotics with osteopsathyrosis, or fragilitas ossium, called Lobstein's disease after Lobstein's report in 1933. The authors divide the ocular syndrome with blue sclerotics into (1) the osseous syndrome with psathyrotic deformities and developmental changes of bone; (2) the auricular syndrome, namely deafness; (3) the articular syndrome, and (4) the endocrine syndrome. The most constant observations are the blue sclerotics, which are of diagnostic significance if they are familial and associated with auditory symptoms. The authors distinguish between complete and incomplete forms of Lobstein's disease. In discussing the osseous symptoms they mention also the vertical form of osteopsathyrosis. The craniofacial deformations are particularly frequent; the occluding temporal and frontal bones, the smallness of the face and the skull, the thinning of the cranial wall. The articular syndrome is merely excessive relaxation of the joints, which is inconstant. The auricular syndrome is particularly

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