CORTICOSTEROID PRODUCTION IN ABETALIPOPROTEINEMIA - EVIDENCE FOR AN IMPAIRED RESPONSE TO ACTH

  • 1 January 1982
    • journal article
    • research article
    • Vol. 100  (1) , 115-126
Abstract
The concept that an inherent absence of plasma LDL [low density lipoprotein] may be associated with a reduced synthesis of steroid hormones was evaluated in 2 patients with ABL [abetalipoproteinemia]. Basal production of adrenal corticosteroids, assessed by the concentrations of serum cortisol and the rates of excretion of urinary 170HCS [hydroxycorticosteroid] and 17KS [ketosteroid] and urine free cortisol, was normal in both patients with ABL. Prolonged stimulation (24-36 h) with ACTH, however, disclosed an impairment in adrenal corticosteroid production in both patients with ABL (as compared to normolipidemic controls), which was manifest by lower serum cortisol levels, reduced rates of urinary excretion of 170HCS and 17KS and 17KS and a marked decrease in the excretion of urine free cortisol. Plasma LDL apparently serve as an important source of cholesterol for adrenal corticosteroid synthesis during prolonged stimulation with ACTH but a total absence of LDL does not does not impair adrenal steroidogenesis in the basal state.