LAUSR

Introduction Children with classical congenital adrenal hyperplasia (CAH) have an impaired steroid synthesis due to 21-hydroxylase dysfunction and require glucocorticoid replacement. Therapy management in children and adolescent is based on auxological, clinical and laboratory monitoring. The measurement of steroid precursors in saliva is particularly suitable for patients in pediatric endocrinology. Methods In this retrospective and longitudinal study of 22 patients with CAH, we analyzed 546 saliva samples for 17-hydroxyprogesterone (s17-OHP) in prepubertal/pubertal patients. Additionally, we correlated them with auxological parameters such as delta-height standard deviation score (dHSDS). Results We analysed a median observation period of 5.5 years per patient. No precocious pubertal development, abnormal vital signs or Addison crises occurred. 57.1 % of the samples were collected in prepubertal children. 72.5 % of s17-OHP values were attributed to normal auxological development. In the total cohort the median values for s17-OHP were 67.8 pg/ml (morning), 42.5 pg/ml (noon) and 25.0 pg/ml (evening). The difference in values between the group of normal/abnormal growing patients and between prepubertal/pubertal patients was not significant. Discussion/ Conclusion. The measurement of s17-OHP is an important sub-aspect in the overall assessment of treatment response in CAH. It can provide an indication of over-/undertreatment and allows the assessment of day profiles especially in phases of changing (e.g. puberty) steroid requirements. We present here observational data from a larger cohort with longitudinal multiple measurements of s17-OHP. The values do not allow a significant differentiation between normal and abnormal growth or pubertal status. Thus, relying solely on s17-OHP is not advisable.