Abstract
Transition medicine aims at the coordinated transfer of young patients with a
chronic disease from paediatric to adult care. The present study reflects 20
years of experience in transitioning patients with congenital adrenal
hyperplasia (CAH) in a single center setting. Our endocrine transition-clinic
was established in 2002 and offers joint paediatric and adult consultations.
Data were evaluated retrospectively from 2002 to 2005 and 2008 to present.
Fifty-nine patients (29 males) were transferred. Median age was 18.4 years
(17.6–23.6). Ninety percent of the patients presented with
21-hydroxlase-deficiency (21-OHD), 38 patients (23 m) with salt-wasting
(sw), 7 (1 m) with simple-virilising (sv) and 8 (3 m) with the
non-classic (nc) form. Rarer enzyme deficiencies were found in 6 cases:
17α-OHD (2 sisters), P450-oxidoreductase-deficiency (2 siblings),
3β-hydroxysteroid-dehydrogenase-deficiency (1 m) and
11β-OHD (1 female). Thirty-four patients (57.6%, 20 m)
are presently still attending the adult clinic, 1 patient (1.7%, m)
moved away and 24 (40.7%, 8 m) were lost to follow-up (13
sw-21-OHD, 6 sv-21-OHD, 5 nc-21-OHD). Thirty-seven patients (62.7%)
attended the adult clinic for >2 years after transfer, 17
(28.8%) for >10 years. In the lost to follow-up group, median
time of attendance was 16.3 months (0–195.2). Defining a successful
transfer as two or more visits in the adult department after initial
consultation in the transition clinic, transfer was efficient in 84.7%
of the cases. A seamless transfer to adult care is essential for adolescents
with CAH. It requires a continuous joint support during the transition period,
remains challenging, and necessitates adequate funding.
Key words
transition - congenital adrenal hyperplasia - single centre experience
