ESPE Abstracts (2016) 86 P-P1-31

Growth of Children with Congenital Adrenal Hyperplasia (CAH) During the First 2 years of Life - Data from the Duth Longitudinal Registry

Annelieke van der Lindea, Nel Roelevelda, Erika vd Akkerb, Mirjam van Albadac, Sabine Hannemad, Gea Hoorweg-Nijmane, Hetty vd Kampf, Martijn Finkeng, Roelof Odinkh, Paul van Trotsenburgi, Paul Verkerkj & Hedi Claahsena


aRadboud University Medical Centre, Nijmegen, The Netherlands; bErasmus Medical Centre, Rotterdam, The Netherlands; cUniversity Medical Centre Groningen, Groningen, The Netherlands; dLeiden University Medical Centre, Leiden, The Netherlands; eSt Anthonius Hospital, Nieuwegein, The Netherlands; fUniversity Medical Centre Utrecht, Utrecht, The Netherlands; gVU Medical Centre, Amsterdam, The Netherlands; hCatharina Hospital, Eindhoven, The Netherlands; iAmsterdam Medical Centre, Amsterdam, The Netherlands; jTNO, Leiden, The Netherlands.


Background: A national database has been developed to register longitudinal data from all CAH children detected through neonatal screening from 2002 onwards. So far, data from 105 children have been registered (65% of Dutch CAH patients) to evaluate treatment and long-term effects in CAH.

Aims: To evaluate height and weight in relation to medication used in the first 2 years of life.

Methods: Biometric data and medication dosage were available for 68 children at 0, 6, 12 and 24 months. Mixed model analyses with manual backward selection were performed for height, expressed as height standard deviation score (SDS) corrected for target height SDS (HSDS-THSDS), and for SDS weight-for-height.

Results: For HSDS-THSDS, a decrease of −0.019 SDS/month (95%CI −0.031 – −0.006; P 0.004) was identified, which amounts to −0.456 SDS at 2 years of age. HSDS-THSDS growth velocity was positively associated with hydrocortisone (HC) and fludrocortisone (FC) dosage at t=2 years only. SDS weight-for-height showed a decline of −0.027/month (95%CI −0.046 – −0.009; P 0.04) with HC and FC dosages in the first 6 months being negatively associated with the outcome (HC −0.176 SDS/month (95%CI −0.361 – 0.009; P 0.062) and FC −0.011 SDS/month (95%CI −0.017 – −0.004; P 0.002)). At t=2 years, FC was positively associated with SDS weight-for-height. Birth weight and parental height seemed to have positive and negative effects on growth, respectively.

Conclusions: These preliminary results showed decreasing HSDS-THSDS and SDS weight-for-height values in the first 2 years of life among CAH children. The positive associations with HC and FC dosages at t=2 years probably reflect higher dosages due to growth, rather than a realistic positive effect on growth, whereas medication seemed to have a negative effect on SDS weight-for-height in the first 6 months. More detailed exploration of the data may reveal how dosage regimen effects growth.