Background: Premature pubarche is a diagnosis of exclusion, and it is a benign period. Non-classic congenital adrenal hyperplasia (NC-CAH) is one of the most important causes in the differential diagnosis of premature pubarche.
Objective and hypotheses: In this study it was aimed to evaluate the clinical and laboratory data, of 75 cases (five male, 70 female) diagnosed as premature pubarche. Additionally basal 17-hydroxyprogesterone levels determined as NC-CAH criteria according to ACTH stimulation test results were revaluated.
Method: Premature pubarche patients who were admitted to Trakya University Medical Faculty Pediatric Department, Pediatric Endocrinology Outpatient Clinic were evaluated.
Results: The mean diagnosis age was 7.2±0.7 years and the female/male ratio was 14/1. Seven percent of the cases were born small for gestational age and 57% of the cases were overweight. The cases were divided into three groups on the basis of 17-hydroxyprogesterone response to ACTH stimulation test and the groups were compared for the clinical and laboratory findings (stimulated 17-hydroxyprogesterone <4.9 ng/ml, 59.9 ng/ml and <10 ng/ml, respectively). Three of the cases (4%) were diagnosed with NC-CAH. No differences were found in anthropometric findings, basal DHEAS, 17-hydroxyprogesterone, androstenedione and cortisol levels among the groups. Body mass index SDS was directly correlated with height SDS and bone age SDS. Two NC-CAH cases had a basal 17-hydroxyprogesterone level less than 1.5 ng/ml. Cortisol responses to ACTH stimulation test were significantly lower in NC-CAH cases compared with the other cases.
Conclusion: According to the results of study, premature pubarche is the problem of girls and being overweight is a risk factor. Basal 17-hydroxyprogesterone level >2 ng/ml should be reconsidered in the use of differential diagnosis of NC-CAH. Due to the suboptimal cortisol response to ACTH stimulation test, also there is a need for trials to define approach to NC-CAH cases with clinical signs in severe trauma and stress.
10 - 12 Sep 2016
European Society for Paediatric Endocrinology