Abstract
Eleven patients with salt losing CAH (21 OH deficiency), aged 5-19 years were treated with cortisone 20-55 mg/m2/24 h in 3 divided doses and supplementary salt. Normal (< 60 m.i.u./1) and moderately raised (133, 140 m.i.u./1) PRC values in 4 patients were compatible with good control - normal excretion of pregnanetriol (Ptriol). High PRC values (153-522 m.i.u./1) were found in connection with high values of Ptriol (6.5-34.8 μmol/24 h). Plasma Aldosterone (PA) was 3-11 (control values <18) ng/100 ml. Normal ACTH values (11-82 pg/ml) were found in all patients - in one pt. with high PRC (522 m.i.u./1) ACTH was 99 pg/ml.
During maintenance of cortisone therapy, mineralocorticoid (Florinef(R) was added to the treatment. The dose was gradually increased over a period (median 15 months) up to 1-4 μg/kg/24 h (2 doses) and normal PRC values were established. PA decreased significantly to values below 4 ng/100 ml. Ptriol excretion decreased markedly (to 0.4 -69 μmol/24 h). ACTH values were still in the normal range. During the observation period no change in growth velocity could be demonstrated.
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Petersen, K., Winslow, I. & Nielsen, M. Normal ACTH levels in saltlosing congenital adrenal hyperplasia (CAH) with elevated plasma renin concentration (PRC). The effect of mineralocorticoid treatment. Pediatr Res 18, 1226 (1984). https://doi.org/10.1203/00006450-198411000-00154
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DOI: https://doi.org/10.1203/00006450-198411000-00154