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Both Low Blood Glucose and Insufficient Treatment Confer Risk of Neurodevelopmental Impairment in Congenital Hyperinsulinism: A Multinational Cohort Study
- Source :
- Frontiers in Endocrinology, Vol 8 (2017), Rasmussen, A H, Melikyan, M, Globa, E, Shcherderkina, I, Poertner, F, Larsen, A-M, Filipsen, K, Brusgaard, K, Dahl Christiansen, C, Hansen, L K & Christesen, H B T 2017, ' Both Low Blood Glucose and Insufficient Treatment Confer Risk of Neurodevelopmental Impairment in Congenital Hyperinsulinism : A Multinational Cohort Study ', Frontiers in Endocrinology, vol. 8, no. JUL, 156 . https://doi.org/10.3389/fendo.2017.00156, Frontiers in Endocrinology
- Publication Year :
- 2017
- Publisher :
- Frontiers Media SA, 2017.
-
Abstract
- BACKGROUND/AIMS: Congenital hyperinsulinism (CHI) is a heterogeneous disease most frequently caused by KATP-channel (ABCC8 and KCNJ11) mutations, with neonatal or later onset, variable severity, and with focal or diffuse pancreatic involvement as the two major histological types. CHI confers a high risk of neurological impairment; however, sparsely studied in larger patient series. We assessed the neurodevelopmental outcome in children with CHI at follow-up in a mixed international cohort.METHODS: In two hyperinsulinism expert centers, 75 CHI patients were included (Russian, n = 33, referred non-Scandinavian, treated in Denmark n = 27, Scandinavian, n = 15). Hospital files were reviewed. At follow-up, neurodevelopmental impairment and neurodevelopmental, cognitive and motor function scores were assessed.RESULTS: Median (range) age at follow-up was 3.7 years (3.3 months-18.2 years). Neurodevelopmental impairment was seen in 35 (47%). Impairment was associated with abnormal brain magnetic resonance imaging (MRI); odds ratio (OR) (95% CI) 15.0 (3.0-74.3), p = 0.001; lowest recorded blood glucose ≤1 mmol/L; OR 3.8 (1.3-11.3), p = 0.015, being non-Scandinavian patient, OR 3.8 (1.2-11.9), p = 0.023; and treatment delay from first symptom to expert center >5 days; OR 4.0 (1.0-16.6), trend p = 0.05. In multivariate analysis (n = 31) for early predictors with exclusion of brain MRI, treatment delay from first symptom to expert center >5 days conferred a significantly increased risk of neurodevelopment impairment, adjusted OR (aOR) 15.6 (1.6-146.7), p = 0.016, while lowest blood glucose ≤1 mmol/L had a trend toward increased risk, aOR 3.5 (1.1-14.3), p = 0.058. No associations for early vs. late disease onset, KATP-channel mutations, disease severity, focal vs. diffuse disease, or age at follow-up were seen in uni- or multivariate analysis.CONCLUSION: Not only very low blood glucose, but also insufficient treatment as expressed by delay until expert center hospitalization, increased the risk of neurodevelopmental impairment. This novel finding calls for improvements in spread of knowledge about CHI among health-care personnel and rapid contact with an expert CHI center on suspicion of CHI.
- Subjects :
- Pediatrics
medicine.medical_specialty
Multivariate analysis
Endocrinology, Diabetes and Metabolism
030209 endocrinology & metabolism
Disease
Hypoglycemia
lcsh:Diseases of the endocrine glands. Clinical endocrinology
ABCC8
03 medical and health sciences
congenital hyperinsulinism
0302 clinical medicine
Endocrinology
030225 pediatrics
treatment delay
medicine
neurological outcome
Original Research
neurodevelopmental impairment
lcsh:RC648-665
biology
business.industry
Congenital hyperinsulinism
Treatment delay
medicine.disease
Neurological outcome
Neurodevelopmental impairment
hypoglycemia
Cohort
biology.protein
business
Hyperinsulinism
Cohort study
Subjects
Details
- Language :
- English
- ISSN :
- 16642392
- Volume :
- 8
- Database :
- OpenAIRE
- Journal :
- Frontiers in Endocrinology
- Accession number :
- edsair.doi.dedup.....910b1f7d4675d7be74b04b0321f1667a
- Full Text :
- https://doi.org/10.3389/fendo.2017.00156