ESPE Abstracts (2016) 86 P-P1-558

Cerebral Outcome of Children with Congenital Hyperinsulism

Annett Helleskov Rasmussena, Maria Melikianb, Fani Pørtnera, Anna-marie Larsend, Inna Scherderkinab, E Globac, Karen Filipsend, Klaus Brusgaarde & Henrik Christesena,f

aHans Christian Andersen Children’s Hospital, Odense University Hospital, Odense, Denmark; bEndocrinology Scientific Centre, Russian Academy of Science, Moscow, Russia; cDepartment of Children and Adolescent Endocrinology, Ukrainian Center of Endocrine Surgery, Endocrine Organs and Tissue Transplantation, Moscow, Ukraine; dPhysiotherapist. Odense University Hospital, Odense, Denmark; eDepartment of Clinical Genetics, Odense University Hospital, Odense, Denmark; fInstitute of Clinical Research, University of Southern Denmark, Odense, Denmark

Background: Congenital hyperinsulinism (CHI) is a rare condition characterized by unregulated secretion of insulin from pancreatic islet β cells. The primary treatment goal is to obtain normoglycemia, since hypoglycemia in the neonatal period can have severe impact on cerebral development.

Objective and hypotheses: To assess the cerebral function in children with CHI at follow up.

Method: From an international cohort, 40 children diagnosed with CHI (Russia/Kazakhstan/Belarus, n=21, Ukraine, n=4, Sweden/Denmark, n=15) were examined to assess neurodevelopment. The children underwent a standardized assessment program including Bayley Scale of Infant and Toddler Development, 3rd Edition (age 0–2 years=10), DP-3 Developmental Profile (age >2 years=20), or Wechsler’s Intelligent Scale for Children, 4rd edition (age ≥6 years, n=5), and Movement ABC to assess motor function (age 3–17 years, n=10). Exclusion criteria were gestational age <32 weeks.

Results: Median (range) age at follow up was 3.4 (102 d–18.5 y) years. The mean (S.D.) lowest recorded blood glucose was 0.9 (0.6) mmol/l, birth weight 3604 (735) g, Apgar score (5 and 10 min) 8.1 (1.2), and 8.6 (0.8). The majority (n=21, 52%) had the first recorded hypoglycaemic episode <3 days after birth. Epilepsy was found in 8 (21%). By Bayley III, mean (S.D.) motor function composite score was 76.7±28.6 (26th percentile); cognitive score 73.6±24 (20th percentile); language score 63.4±4 (13th percentile), social-emotion score 75.6±23 (23th percentile) and adaptive score 0.6±28 (15th percentile). Twenty children assessed by DP-3 achieved a mean standard score of 68 (normal score 85–115). Ten children achieved an average Movement ABC score equalling 39th percentile, and five Danish children assessed with WISC-IV had a normal cognitive development with a mean (S.D.) IQ score of 95±12.4 (40th percentile).

Conclusion: A high degree of neurodevelopmental delay still occurs in children with CHI, dependent on treatment.

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