Skip to main content
NCBI home page
The Journal of Clinical Investigation logoLink to The Journal of Clinical Investigation
. 1980 Apr;65(4):829–835. doi: 10.1172/JCI109734

Familial combined hyperlipoproteinemia. Evidence for a role of growth hormone deficiency in effecting its manifestation.

T J Merimee
PMCID: PMC434469  PMID: 6987267

Abstract

Hyperlipidemia associated with an isolated deficiency of growth hormone was investigated in 10 subjects with hypercholesterolemia consistently present over a 10-yr period. 8 of these 10 had serum triglyceride concentrations greater than 185 mg/dl. 13 growth hormone-deficient patients with normal serum lipids, 28 age-matched controls, and 6 families possessing both growth hormone-deficient and hormonally normal members were also studied. Hyperlipidemia occurred with growth hormone deficiency only in families in which hormonally normal subjects likewise exhibited hyperlipidemia. However the elevation of serum lipids, particularly cholesterol, was invariably greater in the growth hormone-deficient members of these families. Studies were most consistent with the classification of this trait as familial combined hyperlipoproteinemia. Basal serum concentrations of insulin, glucose, and free fatty acids were similar in all groups. After oral glucose (1.5 g/kg of body wt) both hyperlipidemic and normolipidemic dwarfs exhibited a similar degree of glucose intolerance associated with insulinopenia. Sensitivity to insulin, assessed after the intravenous injection of insulin (0.05 U/kg of body wt), increased and was virtually identical in the two dwarf groups. Administration of 5 mg of human growth hormone twice a day for 1 wk to five subjects did not alter serum lipid patterns. The data provide no conclusive evidence concerning a direct effect of growth hormone deficiency on hyperlipoproteinemia. We postulate that in some individuals growth hormone deficiency may unmask an underlying defect in lipoprotein metabolism.

Full text

PDF
829

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Aloia J. F., Zanzi I., Cohn S. H. Absence of an effect of chronic administration of growth hormone on serum lipids. Metabolism. 1975 Jul;24(7):795–798. doi: 10.1016/0026-0495(75)90125-0. [DOI] [PubMed] [Google Scholar]
  2. Fisher W. R., Truitt D. H. The common hyperlipoproteinemias: an understanding of disease mechanisms and their control. Ann Intern Med. 1976 Oct;85(4):497–508. doi: 10.7326/0003-4819-85-4-497. [DOI] [PubMed] [Google Scholar]
  3. Goldstein J. L., Dana S. E., Brunschede G. Y., Brown M. S. Genetic heterogeneity in familial hypercholesterolemia: evidence for two different mutations affecting functions of low-density lipoprotein receptor. Proc Natl Acad Sci U S A. 1975 Mar;72(3):1092–1096. doi: 10.1073/pnas.72.3.1092. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Goldstein J. L., Schrott H. G., Hazzard W. R., Bierman E. L., Motulsky A. G. Hyperlipidemia in coronary heart disease. II. Genetic analysis of lipid levels in 176 families and delineation of a new inherited disorder, combined hyperlipidemia. J Clin Invest. 1973 Jul;52(7):1544–1568. doi: 10.1172/JCI107332. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. HAVEL R. J., EDER H. A., BRAGDON J. H. The distribution and chemical composition of ultracentrifugally separated lipoproteins in human serum. J Clin Invest. 1955 Sep;34(9):1345–1353. doi: 10.1172/JCI103182. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Herbert V., Lau K. S., Gottlieb C. W., Bleicher S. J. Coated charcoal immunoassay of insulin. J Clin Endocrinol Metab. 1965 Oct;25(10):1375–1384. doi: 10.1210/jcem-25-10-1375. [DOI] [PubMed] [Google Scholar]
  7. Merimee T. J., Fineberg S. E., Hollander W. Vascular disease in the chronic HGH-deficient state. Diabetes. 1973 Nov;22(11):813–819. doi: 10.2337/diab.22.11.813. [DOI] [PubMed] [Google Scholar]
  8. Merimee T. J., Fineberg S. E., McKusick V. A., Hall J. Diabetes mellitus and sexual ateliotic dwarfism: a comparative study. J Clin Invest. 1970 Jun;49(6):1096–1102. doi: 10.1172/JCI106325. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. Merimee T. J., Rabinowitz D., Rimoin D. L., McKusick V. A. Isolated human growth hormone deficiency. 3. Insulin secretion in sexual ateliotic dwarfism. Metabolism. 1968 Nov;17(11):1005–1011. doi: 10.1016/0026-0495(68)90006-1. [DOI] [PubMed] [Google Scholar]
  10. Merimee T. J., Siperstein M. D., Hall J. D., Fineberg S. E. Capillary basement membrane structure: a comparative study of diabetics and sexual ateliotic dwarfs. J Clin Invest. 1970 Dec;49(12):2161–2164. doi: 10.1172/JCI106434. [DOI] [PMC free article] [PubMed] [Google Scholar]
  11. Miettinen T. A., Penttilä I. M., Lampainen E. Familial occurrence of mild hyperlipoproteinaemias. Clin Genet. 1972;3(4):271–280. doi: 10.1111/j.1399-0004.1972.tb04276.x. [DOI] [PubMed] [Google Scholar]
  12. Nikkilä E. A., Aro A. Family study of serum lipids and lipoproteins in coronary heart-disease. Lancet. 1973 May 5;1(7810):954–959. doi: 10.1016/s0140-6736(73)91598-5. [DOI] [PubMed] [Google Scholar]
  13. Noble R. P. Electrophoretic separation of plasma lipoproteins in agarose gel. J Lipid Res. 1968 Nov;9(6):693–700. [PubMed] [Google Scholar]
  14. Rimoin D. L., Merimee T. J., McKusick V. A. Sexual ateliotic dwarfism: a recessively inherited isolated deficiency of growth hormone. Trans Assoc Am Physicians. 1966;79:297–311. [PubMed] [Google Scholar]
  15. Rose H. G., Kranz P., Weinstock M., Juliano J., Haft J. I. Inheritance of combined hyperlipoproteinemia: evidence for a new lipoprotein phenotype. Am J Med. 1973 Feb;54(2):148–160. doi: 10.1016/0002-9343(73)90218-0. [DOI] [PubMed] [Google Scholar]
  16. TROUT D. L., ESTES E. H., Jr, FRIEDBERG S. J. Titration of free fatty acids of plasma: a study of current methods and a new modification. J Lipid Res. 1960 Apr;1:199–202. [PubMed] [Google Scholar]
  17. YALOW R. S., BERSON S. A. Immunoassay of endogenous plasma insulin in man. J Clin Invest. 1960 Jul;39:1157–1175. doi: 10.1172/JCI104130. [DOI] [PMC free article] [PubMed] [Google Scholar]

Articles from Journal of Clinical Investigation are provided here courtesy of American Society for Clinical Investigation

RESOURCES