Abstract
The increased production of adrenal androgens or adrenarche, characterized by a disproportional rise in DHEAS, is known to occur prior to pubertal maturation of the hypothalamo-pituitary-gonarche is observed in normal childre. However, the dissociation between adrenarche and gonarche is observed in some pathological conditions such as true precocious puberty. In the present study we report a newly developed by DHEAS and the late adrenarche in a female patient with PHP. An ELISA for DHEAs was developed by means of DHEA-3-hemisuccinate-BRP conjugate and antiserum against DHEA-3-hemisuccinate-BSA. The sensitivity of the assay was 12.5 pg/well and IC50 was about 400 pg/well. The intra- and inter-assay coefficients of variation were <8% and <10%, respectively. There was close correlation between the DHEAS measurements by this ELISA method and the conventional RIA method over a wide range of serum concentrations (r=0.98, n=52). Serum DHEAS levels were determined in 923 normal children aged 5 days through 18 years and the normal range for each age was established. A 13-year-old female patient presented with short stature. She was obese and had round face, short neck, and brachydactyly with with short metacarpal bones. She had low serum calcium and high serum phosphorus concentrtions with normal renal function. She did not respond to exogenous PTII in terms of urinary cAMP and phosphorus secretion and was diagnosed as PHP. Although she had had menarche at the age of 12 years and regular menstrual cycles and her breast development was at Tanner's stage IV, she had no axillary and puble hair. Urinary 17-KS secretion and serum DHEAS level were low, and serum DHEAS levels remained to be lower than the age-matched normal range up to the age of 15 years even after normalization of serum calcium and phosphorus levels. In conclusion. (1) this new ELISA should provide a useful tool be a newly recognized complication of PHP, suggesting resistence of adrenal cortex to the factor(s) which is responsible for adrenarche in PHP.
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Kaisumnta, N., Asakura, Y., Maesaka, H. et al. LATE ADRENARCHE IN A PATIENT WITH PSEUDORYPOPARATHYROIDISM (PHP): APPLICATION OF OF A NEWLY DEVELOPED ELISA FOR DUEAS. Pediatr Res 33 (Suppl 5), S14 (1993). https://doi.org/10.1203/00006450-199305001-00064
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DOI: https://doi.org/10.1203/00006450-199305001-00064