Vineland adaptive behavior scales to identify neurodevelopmental problems in children with Congenital Hyperinsulinism (CHI)

Orphanet Journal of Rare Diseases
Maria Salomon-EstebanezJacqueline Nicholson

Abstract

Congenital Hyperinsulinism (CHI) is a disease of severe hypoglycaemia caused by excess insulin secretion and associated with adverse neurodevelopment in a third of children. The Vineland Adaptive Behavior Scales Second Edition (VABS-II) is a parent report measure of adaptive functioning that could be used as a developmental screening tool in patients with CHI. We have investigated the performance of VABS-II as a screening tool to identify developmental delay in a relatively large cohort of children with CHI. VABS-II questionnaires testing communication, daily living skills, social skills, motor skills and behaviour domains were completed by parents of 64 children with CHI, presenting both in the early neonatal period (Early-CHI, n = 48) and later in infancy (Late-CHI, n = 16). Individual and adaptive composite (Total) domain scores were converted to standard deviation scores (SDS). VABS-II scores were tested for correlation with objective developmental assessment reported separately by developmental paediatricians, clinical and educational psychologists. VABS-II scores were also investigated for correlation with the timing of hypoglycaemia, gender and phenotype of CHI. Median (range) total VABS-II SDS was low in CHI [-0.48 (-3....Continue Reading

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Citations

Sep 25, 2018·Diabetic Medicine : a Journal of the British Diabetic Association·I BanerjeeM J Dunne
Jan 22, 2021·The Journal of Clinical Endocrinology and Metabolism·Jonna M E MännistöHanna Huopio

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Methods Mentioned

BETA
pancreatectomy

Software Mentioned

VABS
SPSS

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