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April 1965

Lipoatrophic Diabetes: I. Studies Concerning Endocrine Function and Carbohydrate Metabolism

Author Affiliations

From the Division of Endocrinology and Metabolism, Department of Pediatrics, University of Washington. This work was done during the tenure of a traineeship in Pediatric Endocrinology and Metabolic Disease, National Institute for Arthritis and Metabolic Diseases training grant No. 2T1-AM-5190 (Dr. Ruvalcaba).

Am J Dis Child. 1965;109(4):279-286. doi:10.1001/archpedi.1965.02090020281003

LAWRENCE1 suggested the term "lipoatrophic diabetes" to designate the syndrome which he earlier2 had characterized as having the following features: (1) lack of adipose tissue, (2) accentuated muscularity, (3) hepatomegaly with cirrhosis, (4) diabetes which is resistant to insulin and without ketosis, and (5) elevated metabolic rate without hyperthyroidism. The first recorded example of this syndrome was a patient reported by Ziegler3 in 1928 as a case of "lipodystrophy" in a 27-year-old woman in whom the subcutaneous tissue had disappeared early in the second decade of life and who had muscles of "masculine appearance," hepatomegaly, a high metabolic rate, and diabetes that appeared early in the third decade of life. In 1944, McQuarrie4 described a 4-year-old boy with similar features; in 1946, Lawrence and Aberd2 described one additional case; and at least 14 more cases have been reported.5-15 In all but two of

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