BSPED2015 ORAL COMMUNICATIONS Oral Communications 5 (10 abstracts)
White matter integrity and neurocognitive deficits in children with hyperinsulinemic hypoglycaemia and ketotic hypoglycaemia: a comparison study
Anitha Kumaran 1 , Jemima Bullock 2 , Kiran Seunarine 3 , Kling Chong 4 , Ritika Kapoor 5 , Fareneh Vargha-Khadem 6 , Chris Clark 3 & Khalid Hussain 5
1Department of Endocrinology, Great Ormond Street Hospital NHS Trust, London, UK; 2Department of Psychology, Great Ormond Street NHS Foundation Trust, London, UK; 3Imaging and Biophysics Unit, UCL Institute of Child Health, London, UK; 4Department of Radiology, Great Ormond Street Hospital NHS Trust, London, UK; 5Genetics and Epigenetics in Health and Disease, Institute of Child Health, University College London, London, UK; 6Development Cognitive Neuroscience Unit, UCL Institute of Child Health, London, UK.
Background: Children with hyperinsulinaemic hypoglycaemia (HH) are at a high risk of brain injury, while children with ketotic hypoglycaemia (KH) are believed to be neurologically normal, due to the absence and presence respectively of ketone bodies that act as an alternate fuel during hypoglycaemia. Our objective was to ascertain if children with HH sustain greater white matter (WM) injury in comparison to children with KH.
Methods: Neurologically normal children between 5 and 16 years of age with HH and KH were recruited from the endocrine and metabolic outpatient clinic database from 2009 to 2012. Wechsler Intelligence Scale for Children Fourth edition (HH, n=21 and KH, n=14), conventional neuroradiological assessments (HH, n=21 and KH, n=14), and diffusion tensor imaging (HH, n=15 and KH, n=12) were performed. Fractional anisotropy (FA) images that reflects white matter integrity were aligned and voxelwise statistical analysis was performed using Tract-Based Spatial Statistics.
Results: On conventional neuroimaging reduced white matter was seen in 7/21 (33%) with HH and 4/14 (28.5%) with KH. Perceptual reasoning (HH 91.9 vs KH 105.8, P=0.006) and Full scale IQ (HH 89.3 vs KH 100.5, P=0.026) was significantly lower in HH group. Significantly low FA values were seen in global white matter (P=0.018) especially in the genu (P=0.021), splenium (P=0.043), and body of corpus callosum (P=0.022) in HH group. In children with HH mean WM FA correlated significantly with full scale IQ (r=0.586, P=0.035) and perceptual reasoning index (r=0.691, P=0.009). FA values of body of corpus callosum correlated positively to full scale IQ (r=0.675, P=0.011).
Conclusion: Children with HH manifest abnormalities in global white matter and corpus callosum that correlates with cognitive deficits. Future longitudinal studies are required to confirm this and delineate the pattern of deficits at key developmental stages.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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