ICCBH2017 Poster Presentations (1) (209 abstracts)
1Developmental Endocrinology Research Group, University of Glasgow, Glasgow, UK; 2Paediatric Neurosciences Research Group, Department of Paediatric Neurology, Royal Hospital for Children, Glasgow, UK; 3Scottish Muscle Network, Queen Elizabeth University Hospital, Glasgow, UK; 4Department of Paediatrics, Forth Valley Royal Hospital, Stirling, UK; 5Department of Paediatric Neurology, Royal Hospital for Sick Children, Edinburgh, UK; 6Department of Paediatrucs, Ninewells Hospital, Dundee, UK; 7Department of Paediatrics, Royal Aberdeen Childrens Hospital, Aberdeen, UK.
Background: Previous studies with small number of boys with Duchenne Muscular Dystrophy (DMD) suggest that growth failure occurs in glucocorticoid naïve (GC) boys.
Objective: To evaluate height and longitudinal growth in boys with DMD prior to GC.
Method: Retrospective evaluation in boys with DMD with height measurements obtained for clinical purposes. Out of the 91 boys currently managed in Scotland, 51 had at least one height (Ht) measurement prior to GC; 36 had two height measurements prior to GC and 18 had three height measurements prior to GC. Ht and BMI were converted to SDS according to the 1990 UK growth standards. Results reported as median (range).
Results: At median age of 3.5 years (0.1, 7.0), median Ht SDS was −1.0 (−2.8, 1.0) with 11/51 boys (22%) with Ht SDS <−2.0. Median BMI SDS was +0.8 (−3.3, 3.4) with 2/51(4%) boys with BMI SDS < −2.0. Ht SDS was not associated with age (r=0.03, P=0.81) and BMI SDS (r=0.19, P=0.18). For the 36 boys with two height measurements prior to GC, median Ht SDS at baseline was −1.2 (−2.8, 1.0) at median age of 2.8 years (0.1, 6.6). Median Ht SDS at follow-up was −1.1 (−3.4, 0.2) (P=0.60 vs baseline) at median age of 4.6 years (2.4, 6.9). For the 18 boys with three height measurements prior to GC, median HT SDS at baseline was −0.9 (−2.7, 1.0) at median age of 1.6 years (0.2, 6.6). Median Ht SDS at first follow-up was −1.3 (−2.8, 0.7) (P=0.09 vs baseline) at median age of 3.8 years (1.3, 6.9). Median Ht SDS at second follow-up was −1.0 (−3.2, 0.6) (P=0.19 vs baseline) at median age of 5.3 years (3.1, 8.6). Ht SDS <−2.0 was observed in 5/18 (28%), 6/18 (33%) and 6/18 (33%) at baseline, first and second follow-up. Height velocity at first and second follow-up were 7.9 cm/year (2.7, 11.2) and 6.6 cm/year (0, 10.1) (P=0.15).
Conclusion: Our results suggest that short stature occurs in GC naïve boys with DMD but severe growth failure is however not frequently encountered.
Disclosure: The authors declared no competing interests.