Serum Cortisol and 17-Hydroxyprogesterone Concentrations in Children with Classic Congenital Adrenal Hyperplasia

Robert L. Rosenfield

Section of Pediatric Endocrinology, The University of Chicago Children’s Hospital Chicago, Illinois 60637

Address correspondence to: Robert L. Rosenfield, M.D., University of Chicago, Department of Pediatric Endocrinology, Pritzker School of Medicine, 5841 South Maryland (M/C 5053), Chicago, Illinois 60637-1470. E-mail: . jmoore{at}peds.bsd uchicago.edu

To the editor:

Charmandari et al. (1) reported a detailed delineation of the 24-h 17-hydroxyprogesterone and cortisol patterns in patients with classic 21-hydroxylase deficiency. Their analysis of these patterns and the known pharmacokinetics of cortisol led them to conclude that "these findings indicated that hydrocortisone should be administered during the period of increased hypothalamic-pituitary-adrenal axis activity, between 0400 and 1600 h, with the biggest dose given in the morning."

The problem with their conclusion is that they did not actually test whether their recommended therapeutic regime led to the expected result of improved control of the abnormal adrenal secretion. Had they done so, they would have found out that their deduction was incorrect, and to follow their suggestion would compromise the growth of these children.

We likewise performed intensive sampling in patients whose steroid patterns could not be controlled by standard hydrocortisone and cortisone treatment some time ago, and we also tested the effect of alternative therapeutic regimens (2). We did not try administering cortisol at 0400 h to nip the early morning rise of ACTH in the bud, because, like them, we recognized that this is impractical. However, after testing multiple other regimens, including one much like what they suggest, we came to a conclusion that is opposite to their recommendation; we demonstrated that optimal control of such patients involves giving the largest fraction of corticosteroid at bedtime, particularly with a longer acting form of steroid such as dexamethasone. Over the subsequent years, we have come to find that prednisone yields results similar to dexamethasone and with less side effects because the dosage can more readily be fine-tuned.

Received February 19, 2002.

References

  1. Charmandari E, Matthews DR, Johnston A, Brook CG, Hindmarsh PC 2001 Serum cortisol and 17-hydroxyprogesterone interrelation in classic 21-hydroxylase deficiency: is current replacement therapy satisfactory? J Clin Endocrinol Metab 86:4679–4685[Abstract/Free Full Text]
  2. Rosenfield RL, Bickel S, Razdan AK 1980 Amenorrhea related to progestin excess in congenital adrenal hyperplasia. Obstet Gynecol 56:208–215[Abstract]




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