Congenital adrenal hyperplasia owing to 21-hydroxylase deficiency

C. J. Migeon, A. B. Wisniewski

Research output: Contribution to journalArticle

Abstract

In the absence of long-term results of experimental therapies, a common sense approach toward dealing with the growth of patients who have CAH is desirable. First, an effort can be made to decrease the replacement cortisol dose during the first year of life. Doubling, rather than tripling, the basal dose at times of stress could be helpful. The use of adjunctive therapy for infections could result in fewer fevers. After 1 year of age, mean parental height could be used to establish at which centile the child should theoretically grow. The dose of cortisol could be adjusted to maintain the bone age between ± 1 SD. Plasma androstenedione levels should not rise above 50 ng/dL, and 17-hydroxy-progesterone should not be totally suppressed but be maintained between 500 and 1000 ng/dL. Compliance with therapy should be encouraged, particularly for adolescent patients. In the final analysis, a realistic expectation for patients would be a height between the 50th and third percentile of the normal growth curve and, in some cases, slightly below the third percentile when the genetic potential is slight.

Original languageEnglish (US)
Pages (from-to)193-206
Number of pages14
JournalEndocrinology and metabolism clinics of North America
Volume30
Issue number1
DOIs
StatePublished - Jan 1 2001

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Endocrinology

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