Abstract
A 14 year old female had the onset of pubic hair, breast development and mild acne at 6 to 7 years of age. The bone age was 3 1/2 years advanced. She had menarche at 9 years of age and has had monthly periods since. Early evaluation disclosed no abnormalities. The development of cystic acne at 10 years of age prompted additional evaluation. External genitalia were normal with no fusion of the labia and normal to small clitoris. There was no hirsutism. CT scan of the brain, ultrasound of pelvis, long bones, T4,TSH, FSH (9.1 mIU/ml), LH (6.1),estradiol (51 and 148 pg/ml) were normal.DHEA-SO4 (225 μg/dl). Elevated 17 OH-Progesterone (3028 ng/dl), androstenedione (350 ng/dl), testosterone (121 ng/dl) 24 hr urine 17 KS (14.9 mg),pregnanetriol (2.7 mg) normalized with Dexamethasone suppression and daily prednisone (5 mg) afterwards, to testosterone (34), 17 OH Progesterone (141 and 50), urinary 17 KS (5.3) and pregnanetriol (0.2). Serum 17 OH Progesterone in both parents and a brother were normal.
The findings are consistent with nonclassical CAH presenting with isosexual central precocious puberty probably as a result of increased biological maturity (as manifested by bone age) induced by CAH. CAH should be considered in the differential diagnosis of isosexual precocity in females.
Supported by John W. Champion.
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Sotos, J., Ibrahim, M. 111 CAH (21-HYDROXYLASE DEFICIENCY) PRESENTING AS CENTRAL PRECOCIOUS PUBERTY. Pediatr Res 19, 622 (1985). https://doi.org/10.1203/00006450-198506000-00131
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DOI: https://doi.org/10.1203/00006450-198506000-00131