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A REPORT OF AN AFFLICTED FAMILY

JASON E. FARBER, M.D.; AARON E. MARGULIS, M.D.

Arch Intern Med. 1943;71(5):658-665.

	Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

For centuries physicians have witnessed the tragic instance of a child born with multiple fractures of the bones. Lobstein,¹ in 1833, designated this condition as Ostepsathyrosis, a descriptive term derived from Greek, meaning friable bones. In 1849, Vrolik reported a similar condition, which he named osteogenesis imperfecta, implying defective formation of bone as the cause. Spurway,² in 1896, reported the first instance in which the condition of fragile bones (fragilitas ossium) was associated with blue scleras. Bronson,³ in 1917, and van der Hoeve and de Kleyn,⁴ in 1918, added to the syndrome the third feature, deafness. Key,⁵ in 1926, referred to the syndrome as "hereditary hypoplasia of the mesenchyme" and called attention to the hypotonicity of the ligaments with hypermobile joints. In 1928 Bell⁶ collected 75 pedigrees including 489 cases of this abnormality. The condition is not so rare as is generally thought.

Recent literature⁷ has shown renewed . . . [Full Text PDF of this Article]

Author Affiliations
BUFFALO; NEW YORK

From the Edward J. Meyer Memorial Hospital and the University of Buffalo School of Medicine, Buffalo, and the Department of Pathology of the New York Post-Graduate Medical School and Hospital, Columbia University, New York.

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