This Article  • References  • Full text PDF  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citing articles on HighWire  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Social Bookmarking   What's this?

III. Progressive Diaphyseal Dysplasia

W. P. U. JACKSON, M.D.; JOSEPH HANELIN, M.D.; FULLER ALBRIGHT, M.D.

AMA Arch Intern Med. 1954;94(6):902-910.

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

PROGRESSIVE diaphyseal dysplasia, so-named by Neuhauser, Schwachman, Wittenborg, and Cohen, in 1948,⁸ is also known as Engelmann's disease.* Fairbank ⁶ believes that Cockayne ⁴ described the first case in 1920 and mentions the other few reports of the syndrome.

Affected patients are weak and thin and are shorter than normal in stature. Their muscular development is poor; their gait, waddling. Some complain of pain in the legs. The diagnosis is made in the first decade of life on the basis of roentgenographic changes in the long bones. In the early stages symmetrical fusiform expansion occurs at the midshafts. Sometimes two separate diaphyseal foci of bone expansion are seen in the femora or in the tibiae, with the intervening shaft less intensely involved or almost totally spared. The outlines of the abnormal sections are usually smooth, but the bone pattern is amorphous in texture and of irregular density, with consequent . . . [Full Text PDF of this Article]

Author Affiliations
CAPE TOWN, UNION OF SOUTH AFRICA; BOSTON

From the Department of Medicine of the Harvard Medical School and the Medical Service and the Department of Radiology of the Massachusetts General Hospital.

Footnotes
Eli Lilly Research Fellow, 1953; lately Assistant in Medicine at the Massachusetts General Hospital and Harvard Medical School; present address, Department of Medicine, University of Cape Town (Dr. Jackson).

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?