Skip to main content
PMC home page
Archives of Disease in Childhood logoLink to Archives of Disease in Childhood
. 1981 Mar;56(3):208–213. doi: 10.1136/adc.56.3.208

Circadian patterns of plasma cortisol, 17-hydroxyprogesterone, and testosterone in congenital adrenal hyperplasia.

H Frisch, K Parth, E Schober, W Swoboda
PMCID: PMC1627169  PMID: 7212759

Abstract

In 11 children aged between 2 and 17 years with (nonsalt-losing) congenital adrenal hyperplasia (21-hydroxylase deficiency) blood was drawn at 90-minute intervals during a 24-hour period and levels of 17-hydroxyprogesterone, testosterone, and cortisol were measured. Levels of 17-ketosteroids and pregnanetriol were measured too in 24-hour urine samples. These measurements were taken under different regimens of treatment and after interruption of treatment. Cortisol level rose and fell rapidly after administered corticosteroid, and reached unphysiologically high levels. Testosterone levels showed pronounced variations but stayed in the normal range for most of the time even in untreated patients; thus testosterone provides a poor control parameter. Levels of 17-hydroxyprogesterone showed extreme fluctuations and very high peak levels in untreated patients; standard treatment with two or three daily doses of corticosteroids did not prevent a pronounced rise in its level after midnight. After the first morning dose of hydrocortisone a very steep fall was observed. The 24-hour pregnanetriol excretion correlated well with the corresponding total integrated 17-hydroxyprogesterone area. It is concluded that single 17-hydroxyprogesterone values are unlikely to give adequate information about the quality of treatment.

Full text

PDF
210

Selected References

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

  1. Atherden S. M., Barnes N. D., Grant D. B. Circadian variation in plasma 17-hydroxyprogesterone in patients with congenital adrenal hyperplasia. Arch Dis Child. 1972 Aug;47(254):602–604. doi: 10.1136/adc.47.254.602. [DOI] [PMC free article] [PubMed] [Google Scholar]
  2. Barnes N. D., Atherden S. M. Diagnosis of congenital adrenal hyperplasia by measurement of plasma 17-hydroxyprogesterone. Arch Dis Child. 1972 Feb;47(251):62–65. doi: 10.1136/adc.47.251.62. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Golden M. P., Lippe B. M., Kaplan S. A., Lavin N., Slavin J. Management of congenital adrenal hyperplasia using serum dehydroepiandrosterone sulfate and 17-hydroxyprogesterone concentrations. Pediatrics. 1978 Jun;61(6):867–871. [PubMed] [Google Scholar]
  4. Hughes I. A., Winter J. S. The application of a serum 17OH-progesterone radioimmunoassay to the diagnosis and management of congenital adrenal hyperplasia. J Pediatr. 1976 May;88(5):766–773. doi: 10.1016/s0022-3476(76)81112-2. [DOI] [PubMed] [Google Scholar]
  5. Hughes I. A., Winter J. S. The relationships between serum concentrations of 17OH-progesterone and other serum and urinary steroids in patients with congenital adrenal hyperplasia. J Clin Endocrinol Metab. 1978 Jan;46(1):98–104. doi: 10.1210/jcem-46-1-98. [DOI] [PubMed] [Google Scholar]
  6. Huseman C. A., Varma M. M., Blizzard R. M., Johanson A. Treatment of congenital virilizing adrenal hyperplasia patients with single and multiple daily doses of prednisone. J Pediatr. 1977 Apr;90(4):538–542. doi: 10.1016/s0022-3476(77)80362-4. [DOI] [PubMed] [Google Scholar]
  7. 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]
  8. Lippe B. M., LaFranchi S. H., Lavin N., Parlow A., Coyotupa J., Kaplan S. A. Serum 17-alpha-hydroxyprogesterone, progesterone, estradiol, and testosterone in the diagnosis and management of congenital adrenal hyperplasia. J Pediatr. 1974 Dec;85(6):782–787. doi: 10.1016/s0022-3476(74)80340-9. [DOI] [PubMed] [Google Scholar]
  9. McKenna T. J., Jennings A. S., Liddle G. W., Burr I. M. Pregnenolone, 17-OH-pregnenolone, and testosterone in plasma of patients with congenital adrenal hyperplasia. J Clin Endocrinol Metab. 1976 May;42(5):918–925. doi: 10.1210/jcem-42-5-918. [DOI] [PubMed] [Google Scholar]
  10. Parth K., Zimprich H., Swoboda W., Brunel R., Bohrn E. Congenital adrenal hyperplasia: simultaneous determination of plasma aldosterone and 17-hydroxyprogesterone. Acta Endocrinol (Copenh) 1978 Jan;87(1):148–157. doi: 10.1530/acta.0.0870148. [DOI] [PubMed] [Google Scholar]
  11. Pham-Huu-Trung M. T., Gourmelen M., Girard F. The simultaneous assay of cortisol and 17alpha-hydroxyprogesterone in the plasma of patients with congenital adrenal hyperplasia. Acta Endocrinol (Copenh) 1973 Oct;74(2):316–330. doi: 10.1530/acta.0.0740316. [DOI] [PubMed] [Google Scholar]
  12. Solomon I. L., Schoen E. J., Donelan L., Brandt-Erichsen D. Blood testosterone values in patients with congenital virilizing adrenal hyperplasia. J Clin Endocrinol Metab. 1975 Mar;40(3):355–362. doi: 10.1210/jcem-40-3-355. [DOI] [PubMed] [Google Scholar]
  13. Strott C. A., Yoshimi T., Lipsett M. B. Plasma progesterone and 17-hydroxyprogesterone in normal men and children with congenital adrenal hyperplasia. J Clin Invest. 1969 May;48(5):930–939. doi: 10.1172/JCI106052. [DOI] [PMC free article] [PubMed] [Google Scholar]
  14. von Schnakenburg K., Bidingmaier F., Knorr D. 17-Hhydroxyprogesteron, Testosteron und Androstenedion beim behandelten congenitalen adrenogenitalen syndrom. Monatsschr Kinderheilkd. 1977 May;125(5):579–580. [PubMed] [Google Scholar]

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

RESOURCES