title: Abnormal growth hormone secretory dynamics in children with familial hypercholesterolemia.
abstract: Growth hormone secretory dynamics and plasma somatomedin C concentrations were assessed in four prepubertal patients with defects in the low-density lipoprotein (LDL) receptor pathway before and after 2 months of treatment with mevinolin, an HMG-CoA reductase inhibitor that reduces intracellular cholesterol. Pre- and posttreatment mean 24-hour growth hormone levels and pulse amplitude were similar and tended to be higher than in age-matched prepubertal controls. Pre- and posttreatment somatomedin C levels were also similar and lower than in age-matched prepubertal controls. All patients responded to growth hormone provocative testing with a peak response of greater than 7 ng/ml, independent of treatment status. Growth velocity was not significantly altered in any patient following 2 months of treatment with mevinolin, and was within the normal range for age. Thus, children with defects in the LDL receptor pathway express abnormalities in growth hormone secretion and somatomedin C generation comparable to those seen in other chronic diseases. Treatment with mevinolin has no apparent effect on these biochemical abnormalities, suggesting that it may not have long-term effects on growth. Regardless of mevinolin therapy, children with defects in the LDL receptor pathway may manifest a degree of growth retardation and, hence, growth rate and skeletal maturation should be closely monitored.
authors.affiliation: Developmental Endocrinology Branch, National Institute of Child Health Human Development, Bethesda, Md.
authors.forename: L
authors.initials: L
authors.lastname: Laue
authors.forename: J M
authors.initials: JM
authors.lastname: Hoeg
authors.forename: K
authors.initials: K
authors.lastname: Barnes
authors.forename: J L
authors.initials: JL
authors.lastname: Ross
authors.forename: D L
authors.initials: DL
authors.lastname: Loriaux
authors.forename: G P
authors.initials: GP
authors.lastname: Chrousos
mesh_terms: D002648:Child; D002675:Child, Preschool; D005260:Female; D013006:Growth Hormone / Q000097:blood / Q000378:metabolism*; D006801:Humans; D006938:Hyperlipoproteinemia Type II / Q000097:blood / Q000378:metabolism*; D007334:Insulin-Like Growth Factor I / Q000032:analysis*; D008055:Lipids / Q000097:blood; D008074:Lipoproteins / Q000097:blood; D008297:Male; D011973:Receptors, LDL / Q000502:physiology; D013002:Somatomedins / Q000032:analysis*
chemical_list: D008055:Lipids; D008074:Lipoproteins; D011973:Receptors, LDL; D013002:Somatomedins; D007334:Insulin-Like Growth Factor I; D013006:Growth Hormone