Skip to main content
PMC home page
Archives of Disease in Childhood logoLink to Archives of Disease in Childhood
. 1997 Sep;77(3):214–218. doi: 10.1136/adc.77.3.214

Randomised controlled trial of growth effect of hydrocortisone in congenital adrenal hyperplasia

I N Silva 1, C E Kater 1, C d Cunha 1, M B Viana 1
PMCID: PMC1717313  PMID: 9370898

Abstract





The influence of 15 or 25 mg/m2 of daily oral hydrocortisone with fludrocortisone 0.1 mg/day on growth and laboratory findings was evaluated in a prospective randomised crossover trial over 12 months in 26 children with 21-hydroxylase deficiency. Nine non-salt losers had fludrocortisone stopped for a further six month period. Height velocity was significantly decreased during treatment with 25 mg/m2 as compared with 15 mg/m2. This was the most sensitive indicator of corticosteroid treatment excess. A dose dependent effect upon plasma concentrations of 17-hydroxyprogesterone, testosterone, and androstenedione was found but increased values were still detected in more than half of the determinations made during the 25 mg/m2 period. Height velocity and 17-hydroxyprogesterone concentrations were positively correlated. Growth hormone response to clonidine stimulation and insulin-like growth factor-1 concentrations were both within reference values and there was no difference between treatment periods. Withdrawal of fludrocortisone did not result in any difference for the non-salt losers. It was concluded that 25 mg/m2 of hydrocortisone depressed growth in children with congenital adrenal hyperplasia, and that full suppression, or even normalisation, of plasma concentrations of 17-hydroxyprogesterone and androgens should not be considered a treatment goal, but instead an indication of corticosteroid treatment excess.



Full Text

The Full Text of this article is available as a PDF (103.8 KB).

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Appan S., Hindmarsh P. C., Brook C. G. Monitoring treatment in congenital adrenal hyperplasia. Arch Dis Child. 1989 Sep;64(9):1235–1239. doi: 10.1136/adc.64.9.1235. [DOI] [PMC free article] [PubMed] [Google Scholar]
  2. Cavallo A., Corn C., Bryan G. T., Meyer W. J., 3rd The use of plasma androstenedione in monitoring therapy of patients with congenital adrenal hyperplasia. J Pediatr. 1979 Jul;95(1):33–37. doi: 10.1016/s0022-3476(79)80078-5. [DOI] [PubMed] [Google Scholar]
  3. DiMartino-Nardi J., Stoner E., O'Connell A., New M. I. The effect of treatment of final height in classical congenital adrenal hyperplasia (CAH). Acta Endocrinol Suppl (Copenh) 1986;279:305–314. doi: 10.1530/acta.0.112s305. [DOI] [PubMed] [Google Scholar]
  4. Esteban N. V., Yergey A. L. Cortisol production rates measured by liquid chromatography/mass spectrometry. Steroids. 1990 Apr;55(4):152–158. doi: 10.1016/0039-128x(90)90103-i. [DOI] [PubMed] [Google Scholar]
  5. Frisch H., Parth K., Schober E., Swoboda W. Circadian patterns of plasma cortisol, 17-hydroxyprogesterone, and testosterone in congenital adrenal hyperplasia. Arch Dis Child. 1981 Mar;56(3):208–213. doi: 10.1136/adc.56.3.208. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Hamill P. V., Drizd T. A., Johnson C. L., Reed R. B., Roche A. F., Moore W. M. Physical growth: National Center for Health Statistics percentiles. Am J Clin Nutr. 1979 Mar;32(3):607–629. doi: 10.1093/ajcn/32.3.607. [DOI] [PubMed] [Google Scholar]
  7. Hughes I. A. Management of congenital adrenal hyperplasia. Arch Dis Child. 1988 Nov;63(11):1399–1404. doi: 10.1136/adc.63.11.1399. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Kenny F. M., Preeyasombat C., Migeon C. J. Cortisol production rate. II. Normal infants, children, and adults. Pediatrics. 1966 Jan;37(1):34–42. [PubMed] [Google Scholar]
  9. Kerrigan J. R., Veldhuis J. D., Leyo S. A., Iranmanesh A., Rogol A. D. Estimation of daily cortisol production and clearance rates in normal pubertal males by deconvolution analysis. J Clin Endocrinol Metab. 1993 Jun;76(6):1505–1510. doi: 10.1210/jcem.76.6.8501158. [DOI] [PubMed] [Google Scholar]
  10. Klingensmith G. J., Garcia S. C., Jones H. W., Migeon C. J., Blizzard R. M. Glucocorticoid treatment of girls with congenital adrenal hyperplasia: effects on height, sexual maturation, and fertility. J Pediatr. 1977 Jun;90(6):996–1004. doi: 10.1016/s0022-3476(77)80581-7. [DOI] [PubMed] [Google Scholar]
  11. Korth-Schutz S., Virdis R., Saenger P., Chow D. M., Levine L. S., New M. I. Serum androgens as a continuing index of adequacy of treatment of congenital adrenal hyperplasia. J Clin Endocrinol Metab. 1978 Mar;46(3):452–458. doi: 10.1210/jcem-46-3-452. [DOI] [PubMed] [Google Scholar]
  12. Kuhnle U., Rösler A., Pareira J. A., Gunzcler P., Levine L. S., New M. I. The effects of long-term normalization of sodium balance on linear growth in disorders with aldosterone deficiency. Acta Endocrinol (Copenh) 1983 Apr;102(4):577–582. doi: 10.1530/acta.0.1020577. [DOI] [PubMed] [Google Scholar]
  13. Linder B. L., Esteban N. V., Yergey A. L., Winterer J. C., Loriaux D. L., Cassorla F. Cortisol production rate in childhood and adolescence. J Pediatr. 1990 Dec;117(6):892–896. doi: 10.1016/s0022-3476(05)80128-3. [DOI] [PubMed] [Google Scholar]
  14. Lox C. D., Christian C. D., Heine M. W. A simple radioimmunoassay for testosterone. Am J Obstet Gynecol. 1974 Jan 1;118(1):114–118. doi: 10.1016/s0002-9378(16)33654-7. [DOI] [PubMed] [Google Scholar]
  15. Maciel R. M., Spritzer N., Spritzer T. S., Abichequer M. B. Tratamento da hipertensão arterial resistente aos diuréticos - estudo comparativo cruzado entre verapamil e clonidina. Arq Bras Cardiol. 1981 Mar;36 (Suppl 1):47–50. [PubMed] [Google Scholar]
  16. Marshall W. A., Tanner J. M. Variations in pattern of pubertal changes in girls. Arch Dis Child. 1969 Jun;44(235):291–303. doi: 10.1136/adc.44.235.291. [DOI] [PMC free article] [PubMed] [Google Scholar]
  17. Migeon C. J., Donohoue P. A. Congenital adrenal hyperplasia caused by 21-hydroxylase deficiency. Its molecular basis and its remaining therapeutic problems. Endocrinol Metab Clin North Am. 1991 Jun;20(2):277–296. [PubMed] [Google Scholar]
  18. Miller W. L. Clinical review 54: Genetics, diagnosis, and management of 21-hydroxylase deficiency. J Clin Endocrinol Metab. 1994 Feb;78(2):241–246. doi: 10.1210/jcem.78.2.8106606. [DOI] [PubMed] [Google Scholar]
  19. New M. I. Female pseudohermaphroditism. Semin Perinatol. 1992 Oct;16(5):299–318. [PubMed] [Google Scholar]
  20. New M. I., Gertner J. M., Speiser P. W., del Balzo P. Growth and final height in classical and nonclassical 21-hydroxylase deficiency. Acta Paediatr Jpn. 1988;30 (Suppl):79–88. [PubMed] [Google Scholar]
  21. Rasat R., Espiner E. A., Abbott G. D. Growth patterns and outcomes in congenital adrenal hyperplasia; effect of chronic treatment regimens. N Z Med J. 1995 Aug 11;108(1005):311–314. [PubMed] [Google Scholar]
  22. Sandrini R., Jospe N., Migeon C. J. Temporal and individual variations in the dose of glucocorticoid used for the treatment of salt-losing congenital virilizing adrenal hyperplasia due to 21-hydroxylase deficiency. Acta Paediatr Suppl. 1993 Mar;388:56–61. doi: 10.1111/j.1651-2227.1993.tb12844.x. [DOI] [PubMed] [Google Scholar]
  23. Silva I. N., Oliveira-Júnior D. F., Simal C. J., Viana M. B., Chagas A. J. Morning steroid profile in children with congenital adrenal hyperplasia under different hydrocortisone schedules. Indian J Pediatr. 1994 Jul-Aug;61(4):341–346. doi: 10.1007/BF02751885. [DOI] [PubMed] [Google Scholar]
  24. Sperling M. A., Kenny F. M., Schutt-Aine J. C., Drash A. L. Linear growth and growth hormonal responsiveness in treated congenital adrenal hyperplasia. Am J Dis Child. 1971 Nov;122(5):408–413. doi: 10.1001/archpedi.1971.02110050078008. [DOI] [PubMed] [Google Scholar]
  25. Urban M. D., Lee P. A., Migeon C. J. Adult height and fertility in men with congenital virilizing adrenal hyperplasia. N Engl J Med. 1978 Dec 21;299(25):1392–1396. doi: 10.1056/NEJM197812212992505. [DOI] [PubMed] [Google Scholar]
  26. Vieira J. G., Lombardi M. T., Nishida S. K. Monoclonal antibody-based immunoenzymometric assay for serum human growth hormone. Braz J Med Biol Res. 1990;23(3-4):293–296. [PubMed] [Google Scholar]
  27. Young M. C., Ribeiro J., Hughes I. A. Growth and body proportions in congenital adrenal hyperplasia. Arch Dis Child. 1989 Nov;64(11):1554–1558. doi: 10.1136/adc.64.11.1554. [DOI] [PMC free article] [PubMed] [Google Scholar]
  28. van der Kamp H. J., Slijper F. M., Brandenburg H., de Muinck Keizer-Schrama S. M., Drop S. L., Molenaar J. C. Evaluation of young women with congenital adrenal hyperplasia: a pilot study. Horm Res. 1992;37 (Suppl 3):45–49. doi: 10.1159/000182400. [DOI] [PubMed] [Google Scholar]

Articles from Archives of Disease in Childhood are provided here courtesy of BMJ Publishing Group

RESOURCES