Plasma adrenocorticotrophic hormone in congenital adrenal hyperplasia. Importance in long-term management

In a prospective study, assays of plasma adrenocorticotrophic hormone (ACTH) were compared with established criteria to evaluate the determination's usefulness in monitoring the control of congenital adrenal hyperplasia (CAH). In 22 infants and children with 21-hydroxylase deficiency, the plasma ACTH value correctly identified the status of control in 51 of 73 (70%) patient visits. Plasma ACTH concentrations were significantly higher in patients whose conditions were out of control when compared with patients whose conditions were under control, although there was an overlap between the two groups. Plasma ACTH concentrations were significantly higher in patients with sodium-losing CAH than in patients with non-sodium-losing CAH. These findings support the concepts that patients with the sodium-losing condition have a more severe enzyme deficiency and that ACTH stimulation may be affected by sodium balance. Although plasma ACTH determinations are a useful adjunct in the long-term management of CAH, they cannot be relied on as the sole criterion of control.