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Berardinelli-Seip lipodystrophy

Berardinelli-Seip lipodystrophy Berardinelli-Seip lipodystrophy (BSCL) is a rare, but widely distributed, congenital disorder of metabolism. It is characterized by insulin-resistant diabetes mellitus and marked deficiency of adipose tissue. The clinical and imaging features of the syndrome are mostly due to fat deficiency, diabetes, or to manifestations of secondary hyperinsulinemia, which results from the failure of the tissues to respond to insulin. Absence of fat may be generalized, or depending upon the subtype of the disease, may not affect areas where fat plays a mechanical function, such as the palms and soles. Muscles appear hypertrophic. In addition, characteristic, but idiopathic, peri-articular lytic lesions may be seen in some individuals. The combination of imaging, clinical, and laboratory findings is characteristic and readily recognized once the components of the syndrome are known. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Skeletal Radiology Springer Journals

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References (24)

Publisher
Springer Journals
Copyright
Copyright © 2007 by ISS
Subject
Medicine & Public Health; Nuclear Medicine ; Pathology ; Orthopedics ; Imaging / Radiology
ISSN
0364-2348
eISSN
1432-2161
DOI
10.1007/s00256-007-0332-4
pmid
17554536
Publisher site
See Article on Publisher Site

Abstract

Berardinelli-Seip lipodystrophy (BSCL) is a rare, but widely distributed, congenital disorder of metabolism. It is characterized by insulin-resistant diabetes mellitus and marked deficiency of adipose tissue. The clinical and imaging features of the syndrome are mostly due to fat deficiency, diabetes, or to manifestations of secondary hyperinsulinemia, which results from the failure of the tissues to respond to insulin. Absence of fat may be generalized, or depending upon the subtype of the disease, may not affect areas where fat plays a mechanical function, such as the palms and soles. Muscles appear hypertrophic. In addition, characteristic, but idiopathic, peri-articular lytic lesions may be seen in some individuals. The combination of imaging, clinical, and laboratory findings is characteristic and readily recognized once the components of the syndrome are known.

Journal

Skeletal RadiologySpringer Journals

Published: Oct 1, 2007

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