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      Effect of Glucagon on Glucose Production, Lipolysis, and Gluconeogenesis in Familial Hyperinsulinism

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          We present an infant with severe familial hyperinsulinism in whom glucose production rate, lipolysis, and gluconeogenesis from glycerol were measured by use of glucose and glycerol labelled with stable isotopes. Administration of a single dose of glucagon (0.1 mg/kg) caused an increase in glucose production rate by near 140% from 4.2 to 10.1 mg·kg<sup>–1</sup>·min<sup>–1</sup>. The rate of appearance of glycerol, reflecting the rate of lipolysis, decreased from 15.1 to 12.6 µmol· kg<sup>–1</sup>·min<sup>–1</sup>. The amount of glycerol converted to glucose by gluconeogenesis was 9.1 µmol·kg<sup>–1</sup>·min<sup>–1</sup> before and 10.5 µmol·kg<sup>–1</sup>·min<sup>–1</sup> after glucagon administration. We conclude that the marked rise in glucose production rate was mainly the result of increased glycogenolysis. Following the trial, the child was started on long-acting (zinc-protamine) glucagon which made it possible to discontinue intravenous treatment with glucose.

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          Familial hyperinsulinism maps to chromosome 11p14-15.1, 30 cM centromeric to the insulin gene.

          Familial hyperinsulinism (HI) is the most common cause of persistent neonatal hyperinsulinaemic hypoglycemia. Linkage analysis in 15 families (12 Ashkenazi Jewish, 2 consanguineous Arab, 1 non-Jewish Caucasian) mapped HI to chromosome 11p14-15.1 (lod score = 9.5, theta = 0 at D11S921). Recombinants localized the disease locus to the 6.6 cM interval between D11S926 and D11S928. In Jewish families, association (p = 0.003) with specific D11S921/D11S419 haplotypes suggested a founder effect. This locus, which is important for normal glucose-regulated insulin secretion, represents a candidate gene for studies of other diseases of beta-cell dysfunction including non-insulin-dependent diabetes mellitus (NIDDM).

            Author and article information

            Horm Res Paediatr
            Hormone Research in Paediatrics
            S. Karger AG
            August 1998
            17 August 1998
            : 50
            : 2
            : 94-98
            Uppsala University Children’s Hospital, Uppsala, Sweden
            23242 Horm Res 1998;50:94–98
            © 1998 S. Karger AG, Basel

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            Pages: 5
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