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Precocious Pubarche: Distinguishing Late-Onset Congenital Adrenal Hyperplasia from Premature Adrenarche

Université Paris Descartes (J.-B.A., R.B., M.C.), 75006 Paris, France; Assistance Publique-Hôpitaux de Paris (AP-HP) (J.-B.A., M.-L.C., R.B.), Hôpital Bicêtre, Unité d’Endocrinologie Pédiatrique, 94275 Le Kremlin-Bicêtre, France; Institut National de la Santé et de la Recherche Médicale U953 (J.-B.A., G.B., M.C.), 75014 Paris, France; AP-HP (C.T.), Hôpital Necker–Enfants Malades, Service d’Explorations Fonctionnelles, 75743 Paris, France; Groupement Hospitalier Est (V.T.), Centre de Biologie et Pathologie Est, 69677 Bron, France; and AP-HP (M.C.), Hôpital Saint Vincent de Paul, Service de Pédiatrie Générale, 75674 Paris, France

Address all correspondence and requests for reprints to: Dr. M. Chalumeau, Department of Pediatrics, Hôpital Saint Vincent de Paul, 82, avenue Denfert-Rochereau, 75674 Paris Cedex 14, France. E-mail: martin.chalumeau{at}svp.aphp.fr.

Context: Because precocious pubarche (PP) reveals late-onset congenital adrenal hyperplasia (LO-CAH) in 5 to 20% of cases, an adrenal stimulation test is recommended in all patients presenting with it. This test is stressful and expensive, and results are normal in more than 80% of cases.

Objective: Our objective was to identify clinical and plasma predictors of LO-CAH among patients presenting with PP.

Design, Setting, and Patients: We conducted a retrospective cohort study that included all patients seen for PP at our hospital between 1999 and 2006 (n = 238). All had undergone an ACTH test.

Main Outcome Measure: LO-CAH was defined by a post-ACTH 17-hydroxyprogesterone (17-OHP) plasma level greater than 10 ng/ml and confirmed by mutational analysis of the CYP21 gene. The association of standard clinical and laboratory indicators with LO-CAH was assessed.

Results: Ten (4%) of 238 patients had LO-CAH. Basal 17-OHP, 4-androstenedione, and testosterone plasma levels were significantly higher in these patients. A 2-ng/ml threshold for basal 17-OHP plasma levels offered 100% (95% CI, 69–100) sensitivity for the diagnosis of LO-CAH and 99% (95% CI, 96–100) specificity.

Conclusion: We identified three plasma predictors of LO-CAH in patients presenting with PP. A selective strategy based on a 2-ng/ml basal 17-OHP plasma level threshold would have safely avoided 99% of the unnecessary ACTH tests among our patients.

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				Simpler Method to Differentiate Between Premature Adrenarche and Late-Onset Congenital Adrenal Hyperplasia Journal Watch Pediatrics and Adolescent Medicine, September 9, 2009; 2009(909): 2 - 2. [Full Text]