Inherited Lipodystrophies and the Metabolic Syndrome

Houshang Monajemi; Erik Stroes; Robert A. Hegele; Eric Fliers


Clin Endocrinol. 2007;67(4):479-484. 

In This Article

Concluding Remarks

Generalized lipodystrophies are easily detected clinically and are usually diagnosed by paediatricians because of the characteristic features from birth onwards. Partial lipodystrophies, on the other hand, are more difficult to recognize, only causing metabolic abnormalities later in life ( Table 2 ). As many of the metabolic features of partial lipodystrophy resemble those of the metabolic syndrome and/or type 2 diabetes mellitus, patients with FPLD are often misdiagnosed. The only clinical signs that are unique to such patients are lipoatrophy and the onset of severe metabolic abnormalities. Because of the marked difference in natural courses of the metabolic derangements in lipodystrophic patients vs. metabolic syndrome subjects, more attention needs to be paid to improving correct identification of lipodystrophic subjects.

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