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April 1965

Lipoatrophic Diabetes: II. Metabolic Studies Concerning the Mechanism of Lipemia

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 Institutes of Arthritis and Metabolic Diseases training grant No. 2T1-AM-5190 (Dr. Ruvalcaba).

Am J Dis Child. 1965;109(4):287-294. doi:10.1001/archpedi.1965.02090020289004

IN THE preceding article1 a detailed case report of a 15-month-old Negro female with the prediabetic phase of lipoatrophic diabetes has been presented. Results of a variety of studies concerning her endocrine function and carbohydrate metabolism were presented and discussed.

A characteristic feature of lipoatrophic diabetes is the disturbance in lipid metabolism which results in a generalized absence, or virtual absence, of subcutaneous adipose tissue. This lipoatrophy has been present in all reported patients with lipoatrophic diabetes2-14 and is essential to establishing this diagnosis. The lipoatrophy has been reported to appear insidiously during the first year of life,3 from 12 to 14 years before the onset of the diabetes,2,5,8 and even after the appearance of the diabetes.4

Hyperlipemia has been present in all but two of the reported patients with this syndrome2,10; the mechanism of the lipemia is not understood although various theories have

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