Abstract
9 unrelated children (2 F, 7 M) with glucocorticoid deficiency form adrenal origin were studied. They were 19 days to 8 years at diagnosis. The disease was revealed in 7 cases by hypoglycemia and/or seazures and suspected in the 2 others because achalasia. Sibling from 4 families died suddenly in childhood. Biological findings showed : very low cortisol levels without any response to synacthen. DAH and Δ4 did not react to synacthen too. Extremely high ACTH levels {x = 1270 (range 328-3095), N = < 90 pg/ml}; low basal aldosterone values (x = 16.4, range < 10-31, N = 10-60 pg/ml) with an impaired response to synacthen (x = 27.2, range < 10-80, N = 60-310 pg/ml) as observed in complete adrenal failure. But evidence for intact mineralocorticoid function was provided by following date : 1) renin activity in each patient was in the normal range for age before and on exclusive glucocorticoid treatment (4 years 1/2 follow up); 2) Salt deprivation (.3 mEq/kg/5 days) performed in 5 cases was well tolerated and accompanied by a rise in renin and aldosterone levels (mean 5 and 12 fold respectively); 3) when in 4 cases DXM (2 mg/lm273 × 48 h), succeded in lowering ACTH level to the normal range, then a significant response of aldosterone to the synacthen test was observed in 3 (reaching 102, 134 and 215 pg/ml) contrasting with the absence of any rise in androgen levels. These findings may suggest that a large amount of endogenous ACTH was occupying all the receptors sites of the intact zone glomerulosa, while the fasciculata-reticularis remains unresponsive to ACTH.
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Cabrol, S., Gourmelen, M., Tubiana, N. et al. APPARENT IMPAIRMENT OF ALDOSTEROME SECRETION IN ISOLATED PRIMITIVE GLUCOCORTICOID DEFICIENCY. Pediatr Res 20, 1203 (1986). https://doi.org/10.1203/00006450-198611000-00179
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DOI: https://doi.org/10.1203/00006450-198611000-00179