BSPED2014 Oral Communications Oral Communications 4 (9 abstracts)
Neurodevelopmental phenotypes in children with early and late presenting congenital hyperinsulinism
Zainaba Mohamed 2 , Maria Michaelidou 1 , Jacqueline Nicholson 4 , Thomas Butler 4 , Lindsey Rigby 4 , Raja Narendra Rao Padidela 4 , Karen E Cosgrove 2 , Mark J Dunne 2 , Peter Clayton 3 & Indraneel Banerjee 4
1School of Medicine, University of Manchester, Manchester, UK; 2Faculty of Life Sciences, University of Manchester, Manchester, UK; 3Faculty of Medical and Human Sciences, University of Manchester, Manchester, UK; 4Department of Paediatric Endocrinology, Royal Manchester Children’s Hospital, Manchester, UK.
Introduction: Adverse neurodevelopmental outcomes have been recognised in children with hypoglycaemia due to early and late presenting congenital hyperinsulinism (CHI). The Vineland Adaptive Behaviour Scales II (VABS-II) is a standardised measure used to assess parent reported adaptive behaviour. The test measures five domains; motor, communication, daily living skills (DLS), socialisation, and maladaptive behaviour. We have used VABS-II to identify specific neurodevelopmental phenotypes in both early and late CHI.
Methods: A cohort of 42 children (29 males, 69%) with CHI treated either medically or surgically, was selected at age >1.5 years for VABS-II testing. Total VABS-II and sub-domain scores except behaviour were converted to SDS using normative data. The Social Communication Questionnaire (SCQ) was used to screen for neurodevelopmental difficulties associated with an autism spectrum disorder (ASD). Those presenting before age 1 month were early-CHI and those after 1 month were late-CHI.
Results: In our cohort, 27 (64%) children presented early, while 15 (36%) presented later (median (range) age 1.0 (0.3; 3.5) years). VABS-II–SDS was low in the whole cohort (−1.0 (−3.6; +2.3)) and was negatively correlated with age (R=−0.3, P=0.05). While all VABS-II domain SDS showed declining trends with increasing age at presentation, DLS–SDS showed the strongest correlation (P=0.04, R2=0.2), when adjusted for gender. In contrast, behaviour scores tended to be higher in early than late CHI (18 (13; 24) vs 16 (10; 22), P=0.1), particularly for internalising behaviours (P=0.02), suggesting maladaptive behaviour in early presenters. SCQ scores were also higher in early CHI (5.5 (1; 31) vs 3.0 (0; 18), P=0.04), although there was no difference in ASD-at risk scores (4 (20%) vs 1 (9%), P=0.4). ASD was diagnosed in one child, each from early and late CHI.
Conclusion: VABS-II psychometric testing shows differential neurodevelopmental phenotypes in early and late CHI, suggesting that the pattern of hypoglycaemic neuronal injury is dependent on the age at presentation.
Volume 36
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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