ESPE Abstracts (2018) 89 P-P2-004

Adrenals and HPA Axis P2

Hydrocortisone (HC) Dose in Children with Congenital Adrenal Hyperplasia (CAH)

Heike Hoyer-Kuhna, Angela Huebnerb, Annette Richter-Unruhc, Rudolf Oeverinkd, Markus Bettendorfe, Tilman Rohrerf, Klaus Kapelarig, Friedrich-Wilhelm Roehlh, Reinhard Holli, Joachim Woelflej & on behalf of the German CAH registry k


aChildren’s Hospital, University of Cologne, Cologne, Germany; bChildren’s Hospital, University of Dresden, Dresden, Germany; cChildren’s Hospital, University of Muenster, Muenster, Germany; dPediatric Endocrinology Practice, Oldenburg, Germany; eChildren’s Hospital, University of Heidelberg, Heidelberg, Germany; fChildren’s Hospital, University of Homburg, Homburg, Germany; gChildren’s Hospital, University of Innsbruck, Innsbruck, Austria; hInstitute of Statistics, University of Magdeburg, Magdeburg, Germany; iInstitute of Epidemiology and Medical Biometry, Ulm, Germany; jChildren’s Hospital, University of Bonn, Bonn, Germany; kGermany

Background: Recommendations for initial and maintenance dose of HC in CAH differ widely. However, treatment of CAH in young children is complicated by the lack of a suitable low-dose HC preparations.

Methods: The German Society for Paediatric Endocrinology and Diabetology (DGKED) initiated a registry for patients with classical CAH (German CAH registry). Anonymized data are transferred for central analysis, including a validation step and a benchmarking report, twice yearly. Until March 2018, the DGKED-CAH database included 26818 patient visits from 1480 patients (654 males). 43 centres from Germany and 3 centres from Austria contributed longitudinal data. Parameters were selected based on current treatment guidelines. A custom-made electronic health record software is used at participating centres for standardized documentation and anonymous transfer of patient records. Linear regression model were implemented with SAS 9.4.

Results: Daily hydrocortisone (HC) dose per m2 body surface area (BSA) was 20.03±0.35 mg (mean ±SE) for patients younger than 3 months of age (n=309), 15.10±0.3 mg/m2 for the age-group 4–12 months (n=442), 14.31±0.24 for patients 1–5 years (n=707) and 14.54±0.20 for patients 6–18 years (n=969). Overall, there was no significant gender difference regarding dose. In patients on fludrocortisone (presumable salt-losing CAH), HC dose was lower in the age-group 0–3 months (19.6 versus 24.4 mg/m2, P<0.05) and in the age-group 1–5 years (14.1 versus 15.1 mg/m2, P<0.05), while in patients older than 6 years a higher HC dose was recorded in patients on fludrocortisone (15.1 versus 13.5 mg/m2, P<0.0001). ANOVA regression modeling revealed a significant interaction between age-group and fludrocortisone use, as well as between gender and fludrocortisone use (both P<0.001).

On average, the largest HC dose was administered in the morning. Median absolute doses morning– midday – evening were 2.0 [Q1-Q3: 2.0–2.5] – 1 [1.0–1.4] – 1.0 [1.0–1.5] mg for children <3 months of age, and 2.5 [2.0–3.0] – 1.1 [1.0–1.8] – 1.3 [1.0–2.0] in the age-group 4–12 months. For children 1–5 years of age, the respective median doses were 4.2 [3.0–5.3], 2.2 [1.7–2.6] and 2.3 [1.8–2.9] mg.

Conclusion: This large, multicentre database provides comprehensive information on prescribed hydrocortisone substitution doses in children with CAH. Low absolute doses required in children younger than 6 years of age are difficult to achieve, considering that currently only a 10 mg tablet formulation is available in Austria and Germany.

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