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
RUVALCABA RHA, SAMOLS E, KELLEY VC. Lipoatrophic Diabetes: I. Studies Concerning Endocrine Function and Carbohydrate Metabolism. Am J Dis Child. 1965;109(4):279–286. doi:10.1001/archpedi.1965.02090020281003
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