caa a22 4500 469094583 CHVBK 20180323133027.0 cr unu---uuuuu 170328e19920801xx s 000 0 eng 10.1007/BF00241817 doi (NATIONALLICENCE)springer-10.1007/BF00241817 The skeleton in congenital, generalized lipodystrophy: evaluation using whole-body radiographic surveys, magnetic resonance imaging and technetium-99m bone scintigraphy [Elektronische Daten] [James Fleckenstein, A. Garg, F. Bonte, M. Vuitch, R. Peshock] Congenital generalized lipodystrophy (CGL) is a rare genetic disease characterized by the absence of body fat from birth. Focal bone lesions have also been reported, but their pathophysiology is poorly understood. To characterize skeletal abnormalities further in 3 patients with CGL, we employed whole-body radiographic skeletal surveys, magnetic resonance imaging (MRI, including gadolinium enhancement), and triple phase technetium-99m methylene diphosphonate bone scintigraphy. Radiographs showed numerous focal lesions within the long bones, as described in other reports. MRI showed that the entire marrow space of the long bones was abnormal and was characterized, at least in part, by the absence of marrow fat. Prolonged T1 and T2 times and marked gadolinium enhancement were observed in radiographically normal-appearing long bone. Radiographically lytic lesions occasionally demonstrated fluid-fluid levels on MRI and enhanced peripherally after gadolinium infusion. Bone scintigraphy findings such as periarticular hyperemia were relative subtle. We conclude that the appendicular skeleton of patients with CGL is diffusely abnormal and is predisposed to focal osteolysis and cyst formation. International Skeletal Society, 1992 Magnetic resonance imaging nationallicence Lipodystrophy nationallicence Adipose tissue nationallicence Diabetes mellitus nationallicence Scintigraphy nationallicence Bone marrow nationallicence Gd-DTPA nationallicence Fleckenstein James Department of Radiology, University of Texas Southwestern Medical Center at Dallas, Dallas, Texas, USA aut Garg A. Department of Internal Medicine, University of Texas Southwestern Medical Center at Dallas, Dallas, Texas, USA aut Bonte F. Department of Radiology, University of Texas Southwestern Medical Center at Dallas, Dallas, Texas, USA aut Vuitch M. Department of Pathology, University of Texas Southwestern Medical Center at Dallas, Dallas, Texas, USA aut Peshock R. Department of Radiology, University of Texas Southwestern Medical Center at Dallas, Dallas, Texas, USA aut Skeletal Radiology Springer-Verlag 21/6(1992-08-01), 381-386 0364-2348 21:6<381 1992 21 256 https://doi.org/10.1007/BF00241817 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF00241817 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Fleckenstein James Department of Radiology, University of Texas Southwestern Medical Center at Dallas, Dallas, Texas, USA aut NATIONALLICENCE 700 1- Garg A. Department of Internal Medicine, University of Texas Southwestern Medical Center at Dallas, Dallas, Texas, USA aut NATIONALLICENCE 700 1- Bonte F. Department of Radiology, University of Texas Southwestern Medical Center at Dallas, Dallas, Texas, USA aut NATIONALLICENCE 700 1- Vuitch M. Department of Pathology, University of Texas Southwestern Medical Center at Dallas, Dallas, Texas, USA aut NATIONALLICENCE 700 1- Peshock R. Department of Radiology, University of Texas Southwestern Medical Center at Dallas, Dallas, Texas, USA aut NATIONALLICENCE 773 0- Skeletal Radiology Springer-Verlag 21/6(1992-08-01), 381-386 0364-2348 21:6<381 1992 21 256 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer