title: Study on growth hormone and insulin secretion in myotonic dystrophy.
abstract: Growth hormone (GH) levels were measured in 12 patients with myotonic dystrophy (MD; 7 men and 5 women, aged 21-49 years) and 14 volunteers after administration of 100 micrograms GH-releasing hormone (GHRH; 1-29). A 75-g oral glucose tolerance test was carried out to determine glucose, insulin, plasma C-peptide, and urinary C-peptide. The GH level in six MD patients responded normally to GHRH (group I), with a peak of 17.1 +/- 1.46 micrograms/l, compared with controls (27.8 +/- 19.6 micrograms/l, NS), and that in the other six patients responded subnormally, with a peak of 3.15 +/- 1.46 micrograms/l, lower than in controls and in group I patients (P < 0.001). In group I the insulin response to the glucose tolerance test showed hyperinsulinism and was lower than that in group II patients; stimulated C-peptide was also higher in group II than in group I and in controls; urinary C-peptide levels were parallel to those in previous data. In all MD patients there were a negative correlation between absolute values of GH response to GHRH and insulin response to glucose tolerance test (r = -0.79, P < 0.001). Our data suggest that the failure in GH release and peripheral insulin action is due to a generalized defect in cellular membrane function in MD patients.
authors.affiliation: Endocrine Service, Hormonal Laboratory, Ciutat Sanitària i Universitària de Bellvitge, Barcelona, Spain.
authors.forename: J M
authors.initials: JM
authors.lastname: Gómez Sáez
authors.forename: J M
authors.initials: JM
authors.lastname: Fernández Real
authors.forename: M
authors.initials: M
authors.lastname: Fernández Castañer
authors.forename: M A
authors.initials: MA
authors.lastname: Navarro Moreno
authors.forename: J A
authors.initials: JA
authors.lastname: Martínez Matos
authors.forename: J
authors.initials: J
authors.lastname: Soler Ramón
mesh_terms: D000328:Adult; D002096:C-Peptide / Q000378:metabolism; D005069:Evaluation Studies as Topic; D005260:Female; D005951:Glucose Tolerance Test; D013006:Growth Hormone / Q000378:metabolism*; D006801:Humans; D007328:Insulin / Q000378:metabolism*; D000078790:Insulin Secretion; D008297:Male; D008875:Middle Aged; D009223:Myotonic Dystrophy / Q000503:physiopathology*
chemical_list: D002096:C-Peptide; D007328:Insulin; D013006:Growth Hormone