Congenital adrenal hyperplasia owing to 21-hydroxylase deficiency. Growth, development, and therapeutic considerations.
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-hydroxyprogesterone 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.
Migeon
C J
CJ
Department of Pediatrics, Division of Pediatric Endocrinology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA. cmigeon@jhmi.edu
Wisniewski
A B
AB
D000312:Adrenal Hyperplasia, Congenital / Q000201:enzymology / Q000503:physiopathology* / Q000628:therapy*; D000315:Adrenalectomy; D001827:Body Height; D005260:Female; D005315:Fetal Diseases / Q000188:drug therapy; D005938:Glucocorticoids / Q000008:administration & dosage / Q000627:therapeutic use; D020249:Hormone Replacement Therapy; D006801:Humans; D008297:Male; D008901:Mineralocorticoids / Q000008:administration & dosage / Q000627:therapeutic use; D011247:Pregnancy
D005938:Glucocorticoids; D008901:Mineralocorticoids