Dehydroepiandrosterone sulfate treatment for atrichia pubis.
OBJECTIVE
To evaluate the efficacy of oral dehydroepiandrosterone sulfate (DHEAS) treatment for atrichia pubis in female adolescents.


METHODS
Two XY female adolescents with 17-hydroxylase deficiency and 2 XX females with panhypopituitarism presenting with atrichia pubis were treated with a daily dosage of DHEAS 10 mg/m2 body surface in addition to their regular substitution therapy. The dosage was increased according to clinical response. Pubic hair stages, growth and serum DHEAS were evaluated and in 1 case also serum IGFs and IGFBPs.


RESULTS
A dosage of 10 mg/m2 for 1 year led to serum DHEAS levels at the lower limit of the normal range. 15 mg/m2 was needed to achieve pubic hair stage 4-5 and axillary hair in patients with 17-hydroxylase deficiency. In panhypopituitarism, pubic hair developed at a slower pace and reached stage 4 on a dosage of 25-30 mg/m2. Baseline serum IGF-I SDS was -0.67 and did not change on the initial dosage of DHEAS, in combination with submaximal estrogen substitution (10 microg ethinyl estradiol). On the combination of 15 mg/m2 DHEAS and full estrogen substitution, IGF-I SDS increased to an average of -0.15. IGFBP-3 SDS increased from 1.4 to a mean of 2.6 in the first year, and went back to 1.4 in the second year. IGFBP-6 SDS was low at baseline (-2.5) and rose to -1.9 and -1.7 IGF-II and IGFBP-1 showed an irregular pattern.


CONCLUSIONS
Oral administration of DHEAS in a dosage of 15 mg/m2 o.d. is an efficacious treatment for atrichia pubis. For females with a panhypopituitarism a higher dosage appears needed. Given this and other biological actions of DHEAS, substitution therapy with DHEAS or DHEA to females with adrenal androgen deficiency appears rational.
Wit
J M
JM
Department of Pediatrics, Leiden University Medical Center, Leiden, The Netherlands. jwit@lumc.nl
Langenhorst
V J
VJ
Jansen
M
M
Oostdijk
W A
WA
van Doorn
J
J
D000284:Administration, Oral; D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital; D019314:Dehydroepiandrosterone Sulfate / Q000008:administration & dosage / Q000097:blood / Q000627:therapeutic use*; D004305:Dose-Response Relationship, Drug; D005260:Female; D006201:Hair Diseases / Q000188:drug therapy* / Q000209:etiology; D006801:Humans; D007018:Hypopituitarism / Q000150:complications; D008659:Metabolic Diseases / Q000150:complications; D010502:Perineum; D011627:Puberty
D019314:Dehydroepiandrosterone Sulfate