STUDIES IN CONGENITAL GENERALIZED LIPODYSTROPHY

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Abstract
Plasma from 3 subjects with congenital generalized lipodystrophy was filtered on Sephadex G-50, and the fractions obtained were analyzed for immunoreactive insulin (IRI). In one patient (I.T.) plasma was obtained in the course of an iv glucose tolerance test, during which there was a substantial increase of IRI. In the fasting state IRI was recovered as a single peak with an elution volume corresponding to crystalline insulin. Twenty min after the glucose load the insulin immunoreactivity appeared in two peaks, a major peak corresponding to crystalline insulin ("little" insulin) and a small peak corresponding to beef pro-insulin ("big" insulin). The relative amount of "big" insulin remained small during the glucose tolerance test. Twenty min after the glucose load the amount of "big" insulin was 4 per cent, whereas 6 per cent was found after 170 min. In a second patient (L.S.N.) with a substantially, lower IRI-activity "big" insulin was not detected after a glucagon load. Likewise, in a third patient (A.E.) with frank diabetes, studied after the age of puberty, a tolbutamide tolerance test failed to reveal any significant amount of "big" insulin.

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