Introduction: Optimizing glucocorticoid (GC) therapy in patients with congenital adrenal hyperplasia (CAH) remains a challenge. While overdosing may result in Cushings syndrome, underdosing is associated with female virilization and adrenal insufficiency. This study evaluated day-profiling of salivary 17-hydroxyprogesterone (17OHP) for the biochemical control of adolescents with CAH due to 21-hydroxylase deficiency.
Methods: Twelve patients (4 males; age 19.3±0.6; BMI 28.0±1.9) were enrolled after transfer from pediatric to adult care. Sampling was performed at 0700, 0930, 1200, 1500, 1800, 2200, and 2400 h. 43 healthy volunteers (22 males; age 37.8±1.8; BMI 25.7±0.7) served as a control group, and upper normal 17OHP cutoffs (mean+2S.D.) were calculated for each time point. Results are expressed as mean±S.E.M. 17OHP was measured by RIA (DPC Diagnostic Products Corp).
Results: Initial evaluation revealed elevated 17OHP baseline values in 64% of patients. Five patients were tested only once (1.8±0.6 sampling times elevated, mean HC-equivalent dose 33.3±10.3 mg), while 7 patients were repeatedly tested. For the latter, 6.0±0.4 sampling times were elevated in the initial day profiles (mean HC-equivalent dose 25.0±1.1 mg). In contrast, 2.9±1.0 elevated sampling times were found after adaption of medication, such as addition of longer-acting glucocorticoids (mean HC-equivalent dose 24.1±2.0 mg). With respect to all profiles, results of single morning evaluation and day-sampling were similar in 38%. Seventy eight percent of profiles with normal 17OHP levels at 0700 h demonstrated increased 17OHP throughout the day.
Conclusion: A relevant proportion of patients with CAH demonstrated insufficient biochemical control during adolescence. Salivary 17OHP day profiles led to changes in GC application with improved 17OHP suppression, while daily GC dose remained stable. Determination of single morning 17OHP levels did not allow for reliable control of androgen excess.
03 - 07 May 2008
European Society of Endocrinology