OBJECTIVES: To describe the phenotype of premature adrenarche (PA) patients, elucidate historical and physical correlates, and distinguish PA from late-onset congenital adrenal hyperplasia (LOCAH). STUDY DESIGN: Retrospective chart review of 122 patients (91 female and 31 male) with PA and 11 with LOCAH. RESULTS: In PA patients, birth weight was <2 standard deviation scores (SDSs) in 6%, and 14% were premature. Body mass index SDSs were high (2.00 ± 1.84). Bone age (BA) SDS was >2 in 31% and was greater in boys than in girls; PA subjects were taller, and the predicted height was at or above genetic potential. Dehydroepiandrosterone sulfate (DHEAS) was higher in boys. LOCAH patients had earlier pubic hair, more advanced BA, lower height SDSs, lower adult height prediction, and higher adrenal androgen levels. CONCLUSION: Patients with PA tend to be overweight with BA acceleration but normal height prediction. LOCAH is distinguished by earlier presentation and higher 17-hydrocyprogesterone and adrenal androgens.
OBJECTIVES: To describe the phenotype of premature adrenarche (PA) patients, elucidate historical and physical correlates, and distinguish PA from late-onset congenital adrenal hyperplasia (LOCAH). STUDY DESIGN: Retrospective chart review of 122 patients (91 female and 31 male) with PA and 11 with LOCAH. RESULTS: In PApatients, birth weight was <2 standard deviation scores (SDSs) in 6%, and 14% were premature. Body mass index SDSs were high (2.00 ± 1.84). Bone age (BA) SDS was >2 in 31% and was greater in boys than in girls; PA subjects were taller, and the predicted height was at or above genetic potential. Dehydroepiandrosterone sulfate (DHEAS) was higher in boys. LOCAH patients had earlier pubic hair, more advanced BA, lower height SDSs, lower adult height prediction, and higher adrenal androgen levels. CONCLUSION:Patients with PA tend to be overweight with BA acceleration but normal height prediction. LOCAH is distinguished by earlier presentation and higher 17-hydrocyprogesterone and adrenal androgens.
Authors: Astrid-Helene Ravn Jørgensen; Julie Aarestrup; Jennifer L Baker; Simon Francis Thomsen Journal: JAMA Dermatol Date: 2020-07-01 Impact factor: 10.282