Predictive factors of health status of patients with congenital adrenal hyperplasia due to 21-hydroxylase diagnosed during childhood

Program: Abstracts - Orals, Featured Poster Presentations, and Posters
Session: SUN 29-49-Congenital Adrenal Hyperplasia & Ectopic Cushing's
Clinical
Sunday, June 16, 2013: 1:45 PM-3:45 PM
Expo Halls ABC (Moscone Center)

Poster Board SUN-29
Anne Bachelot*1, Jérôme Dulon1, Jean-Louis Golmard2, Christiane Coussieu2, Juliane Leger3, Sylvie Cabrol4, Claire Bouvattier5, Michel Polak6 and Philippe A Touraine1
1Pitié-Salpêtrière Hospital, Paris, France, 2Pitié-Salpêtrière Hospital, 3Hosp Robert Debre, Paris, France, 4Hopital Armand Trousseau, Paris Cedex 12, France, 5Hosp Saint-Vincent-De-Paul, Paris Cedex 14, France, 6Hopital Necker Enfants Malades, Paris Cedex 15, France
Adult patients with congenital adrenal hyperplasia (CAH) often suffer from complications as obesity, osteoporosis and perturbation of the reproductive axis leading to infertility. Nevertheless, these complications did not concern all CAH patients, and predictive or causal factors responsible for such complications are not well established yet. We therefore proposed to studied adult CAH patients status in term of body mass index (BMI), bone mineral density (BMD), adrenal CT-scan and reproductive parameters and to determined factors that could account for such complications. Hundred and four patients (71 women, 33 men, 52 with salt-wasting form, 17 with virilizing form and 35 with non classical form of CAH) were studied. Mean age was 27.9 years (16-52). Among the 71 women, 52% have a BMI>25 kg/m², 50% irregular menstrual cycles, 35% hirsutism, 48% abnormal BMD (osteopenia or osteoporosis) and 54% adrenal hyperplasia on CT-scan. Among the 33 men, 40% have a BMI>25 kg/m², 36% adrenal rest tumors, 78% abnormal BMD and 57% adrenal hyperplasia on CT-scan. In univariate analysis, predictive factors for BMI>25 kg/m², abnormal CT-scan, presence of hirsutism or irregular menstrual cycles in women were all related to hormonal control of CAH, including 17OHprogesterone, androstenedione or ACTH levels (p<0.05). Predictive factors of abnormal BMD were weight and LH levels (p<0.05). Predictive factor of the presence of adrenal rest tumors was the severity of the disease, i.e. the presence of classical form of CAH (p=0.002). Total cumulative (TCG) glucocorticoid doses were calculated from pediatric and adult files in 71 patients and were significantly associated to BMI (p=0.01) and abnormal CT-scan (p=0.05).

This study confirms the high prevalence of complications in adult CAH patients and highlights the predominant role of hormonal control and of treatment in the development of these complications. Such data are of importance in improving the management of patients with CAH and in acquiring further knowledge for use in the design of novel therapeutic interventions that aim to improve patient outcome. Finally, it highlights the importance of long term follow up of these patients and of transitional care between childhoods to adult life.

Nothing to Disclose: AB, JD, JLG, CC, JL, SC, CB, MP, PAT

*Please take note of The Endocrine Society's News Embargo Policy at http://www.endo-society.org/endo2013/media.cfm

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