ESPE Abstracts

Introduction: Transition medicine aims at the coordinated transfer of young patients with a chronic disease from paediatric to adult specialist care. The present study reflects 20 years of experience in transitioning patients with congenital adrenal hyperplasia (CAH) in a single centre university setting.

Methods: The endocrine transition clinic was established in 2002 and offers joint consultations with a paediatric and an adult endocrinologist for patients and their families. Data obtained at the transition clinic were evaluated retrospectively from 2002 to 2005 and 2008 to present (2006 and 2007 not considered due to incomplete data acquisition).

Results: 59 patients (29 m) with CAH were transferred from the paediatric to the adult endocrine department during the stated time period. Median age was 18.4 years (17.6 -23.6). The main underlying enzyme defect was 21-hydroxlase deficiency (21-OHD, 90% of cases). 38 patients (23 m) presented with classic 21-OHD with salt wasting (sw), 7 (1 m) with the simple virilising (sv) form and 8 (3 m) with non-classic (nc) 21-OHD. Rarer enzyme deficiencies were found in 6 cases: 2 sisters with 17α-OHD, 2 siblings with P450-oxidoreductase-deficiency, 1 male patient with 3ß-hydroxsteroid-dehydrogenase-deficiency and 1 female patient with 11ß-OHD. At present 34 patients (57.6%, 20 m) are still seen at the adult clinic, 1 patient (1.7%, m) moved abroad and 24 patients (40.7%, 8 m) were lost to follow-up (13 sw 21-OHD, 6 sv 21-OHD, 5 nc 21-OHD). 37 patients (62.7%) attended the adult clinic for > 2 years after transfer, 17 patients (28.8%) for > 10 years. 6 patients (10.2%) were transferred within the last two years. In the group of patients lost to follow-up, median time of continued visits at the adult clinic was 16.3 months (0 – 195.2). Defining a successful transfer as 2 or more visits in the adult endocrine department after initial consultation in the transition clinic, transfer was efficient in 85 % of the cases.

Conclusion: A seamless transfer from paediatric to adult medical care is essential for adolescents with CAH. It requires continuous joint support from both sides during the transition period. Successful transition remains challenging and necessitates adequate funding. Further investigations are needed to establish predictive parameters for a sustainable transition process.