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Horm Metab Res 2024; 56(01): 45-50
DOI: 10.1055/a-2201-6548
Original Article: Endocrine Care

Transition from Paediatric to Adult Care in CAH: 20 Years of Experience at a Tertiary Referral Center

Cordula Kiewert
1   Division of Paediatric Endocrinology and Diabetes, Member of ENDO-ERN, University Hospital Essen, University of Duisburg-Essen, Germany
,
Julia Jedanowski
2   Department of Endocrinology, Diabetes and Metabolism, Member of ENDO-ERN, University Hospital Essen, University of Duisburg-Essen, Germany
,
Berthold P. Hauffa
1   Division of Paediatric Endocrinology and Diabetes, Member of ENDO-ERN, University Hospital Essen, University of Duisburg-Essen, Germany
,
Stephan Petersenn
3   ENDOC Center for Endocrine Tumors, Hamburg, Germany, and University of Duisburg-Essen, Germany
,
Klaus Mann
2   Department of Endocrinology, Diabetes and Metabolism, Member of ENDO-ERN, University Hospital Essen, University of Duisburg-Essen, Germany
,
Dagmar Führer
2   Department of Endocrinology, Diabetes and Metabolism, Member of ENDO-ERN, University Hospital Essen, University of Duisburg-Essen, Germany
,
Nicole Unger
2   Department of Endocrinology, Diabetes and Metabolism, Member of ENDO-ERN, University Hospital Essen, University of Duisburg-Essen, Germany
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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


Publication History

Received: 30 May 2023

Accepted after revision: 27 October 2023

Article published online:
03 January 2024

© 2024. Thieme. All rights reserved.

Georg Thieme Verlag
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

  • References

  • 1 Arlt W, Willis DS, Wild SH. et al. Health status of adults with congenital adrenal hyperplasia: a cohort study of 203 patients. J Clin Endocrinol Metab 2010; 95: 5110-5121
    CrossrefPubMedGoogle Scholar
  • 2 Kruse B, Riepe FG, Krone N. et al. Congenital adrenal hyperplasia - how to improve the transition from adolescence to adult life. Exp Clin Endocrinol Diabetes 2004; 112: 343-355
    Article in Thieme ConnectPubMedGoogle Scholar
  • 3 Speiser PW. Congenital adrenal hyperplasia: transition from chil dhood to adulthood. J Endocrinol Invest 2001; 24: 681-691
    CrossrefPubMedGoogle Scholar
  • 4 Reisch N, Arlt W, Krone N. Health problems in congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Horm Res Paediatr 2011; 76: 73-85
    CrossrefPubMedGoogle Scholar
  • 5 Conway GS. Congenital adrenal hyperplasia: adolescence and transition. Horm Res 2007; 68 (Suppl. 05) 155-157
    PubMedGoogle Scholar
  • 6 Bachelot A. Transition of care from childhood to adulthood: congenital adrenal hyperplasia. Endocr Dev 2018; 33: 17-33
    CrossrefPubMedGoogle Scholar
  • 7 Bachelot A, Vialon M, Baptiste A. et al. Impact of transition on quality of life in patients with congenital adrenal hyperplasia diagnosed during childhood. Endocr Connect 2017; 6: 422-429
    CrossrefPubMedGoogle Scholar
  • 8 Takasawa K, Kashimada K. Toward improving the transition of patients with congenital adrenal hyperplasia from pediatrics to adult healthcare in Japan. Front Pediatr 2022; 10: 936944
    CrossrefPubMedGoogle Scholar
  • 9 Gleeson H, Davis J, Jones J. et al. The challenge of delivering endocrine care and successful transition to adult services in adolescents with congenital adrenal hyperplasia: experience in a single centre over 18 years. Clin Endocrinol (Oxf) 2013; 78: 23-28
    CrossrefPubMedGoogle Scholar
  • 10 Reisch N, Willige M, Kohn D. et al. Frequency and causes of adrenal crises over lifetime in patients with 21-hydroxylase deficiency. Eur J Endocrinol 2012; 167: 35-42
    CrossrefPubMedGoogle Scholar
  • 11 Chrisp GL, Quartararo M, Torpy DJ. et al. Trends in hospital admissions for adrenal insufficiency in adolescents and young adults in the 21(st) century. Front Endocrinol (Lausanne) 2022; 13: 986342
    CrossrefPubMedGoogle Scholar
  • 12 Rushworth RL, Chrisp GL, Bownes S. et al. Adrenal crises in adolescents and young adults. Endocrine 2022; 77: 1-10
    CrossrefPubMedGoogle Scholar
  • 13 Godbout A, Tejedor I, Malivoir S. et al. Transition from pediatric to adult healthcare: assessment of specific needs of patients with chronic endocrine conditions. Horm Res Paediatr 2012; 78: 247-255
    CrossrefPubMedGoogle Scholar
  • 14 Speiser PW, Arlt W, Auchus RJ. et al. Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an endocrine society clinical practice guideline. J Clin Endocrinol Metab 2018; 103: 4043-4088
    CrossrefPubMedGoogle Scholar
  • 15 Pasquini S, Rinaldi E, Da Prato G. et al. Growing up with type 1 diabetes mellitus: data from the Verona Diabetes Transition Project. Diabet Med 2022; 39: e14719
    CrossrefPubMedGoogle Scholar
  • 16 Ekbom K, Lajic S, Falhammar H. et al. Transition readiness in adolescents and young adults living with congenital adrenal hyperplasia. Endocr Pract 2023; 29: 266-271
    CrossrefPubMedGoogle Scholar
  • 17 Auchus RJ, Arlt W. Approach to the patient: the adult with congenital adrenal hyperplasia. J Clin Endocrinol Metab 2013; 98: 2645-2655
    CrossrefPubMedGoogle Scholar
  • 18 Merke DP, Poppas DP. Management of adolescents with congenital adrenal hyperplasia. Lancet Diabetes Endocrinol 2013; 1: 341-352
    CrossrefPubMedGoogle Scholar
  • 19 Gray WN, Schaefer MR, Resmini-Rawlinson A. et al. Barriers to transition from pediatric to adult care: a systematic review. J Pediatr Psychol 2018; 43: 488-502
    CrossrefPubMedGoogle Scholar
  • 20 Engberg H, Moller A, Hagenfeldt K. et al. Identity, sexuality, and parenthood in women with congenital adrenal hyperplasia. J Pediatr Adolesc Gynecol 2020; 33: 470-476
    CrossrefPubMedGoogle Scholar
  • 21 Nordenstrom A, Falhammar H. Management of endocrine disease: diagnosis and management of the patient with non-classic CAH due to 21-hydroxylase deficiency. Eur J Endocrinol 2019; 180: R127-R145
    CrossrefPubMedGoogle Scholar