Endocrine Abstracts

JOINT2086

Introduction: Pubarche is a rare encounter during infancy that needs to be thoroughly investigated. Etiology ranges from normal variant to adrenal tumors.

Methods: An 8-month-old girl with pubarche was referred by her pediatrician after initial evaluation that revealed suppressed levels of cortisol and adrenal androgens. Parents noticed pubic hair onset about one week before referral. Her past medical history was remarkable for recurrent respiratory infections during the previous trimester and bronchiolitis for which she was treated with inhaled corticosteroids in high doses (250 micrograms of fluticasone propionate twice daily), with short intervals without treatment. The review of the growth chart revealed impaired growth velocity from the age of 6 months, as the infant’s length deviated from the 50th to the 10th percentile, while her weight followed the 50th percentile. On physical examination her length was 66 cm (10th %ile), her body weight (BW) 8.35 kg (50th %ile), Tanner stage: Axillary I, pubic hair II, breast II. Facial plethora was appreciated, while clitoral size was normal.

Results: Diagnostic work-up confirmed suppressed cortisol, adrenal androgens and inappropriately low ACTH (serum Cortisol 0.36µg/dl, ACTH 11.52pg/ml, DHEA-S 0.003µg/dl, 17-OH PRG 0.95ng/ml, FSH 3.88mIU/ml, LH<0.3mIU/ml, prolactin 473mIU/ml, estradiol 9.9 pg/ml). Tumor markers (βHCG, aFP) were negative. Abdominal sonogram was normal. The diagnosis of iatrogenic Cushing syndrome was established, based on the above medical history, physical examination and laboratory results. Inhaled corticosteroids were discontinued and hydrocortisone was administered at a replacement dose until the adrenal axis recovered. During follow up, two months later, her height velocity accelerated and laboratory results were normalized (serum Cortisol 11.3 µg/dl, ACTH 52.5pg/ml, DHEA-S 5.1µg/dl, 17-OH PRG 1.2ng/ml, FSH 4.92mIU/ml, LH<0.2mIU/ml, estradiol<6pg/ml). Hydrocortisone was discontinued. At the age of 12 months, the infant had complete regression of pubic hair and normal growth, (length 76 cm, 75th %ile, BW 10.8 kg, 50th-75th %ile), while premature thelarche persisted.

Conclusions: Inhaled steroids are commonly used in treatment of asthma and respiratory pediatric infections. They are considered safer compared to systemic steroids, however iatrogenic Cushing syndrome can be caused by high-dose inhaled regimens. That’s why children on steroid treatment should be followed up closely to prevent iatrogenic Cushing syndrome. The differential diagnosis of the syndrome includes malignant tumors, adenoma, and nodular hyperplasia of the adrenal glands, which are very rare in infants.