BSPED2012 Oral Communications Oral Communications 2 (10 abstracts)
Abnormal neurological and developmental outcomes in children with persistent and spontaneously resolving congenital hyperinsulinism
Bindu Avatapalle 1 , Sajni Shah 1 , Megan Pryce 1 , Jacqueline Nicholson 1 , Lindsey Rigby 1 , Louise Caine 1 , Mohammed Didi 3 , Sarah Ehtisham 1 , Leena Patel 2 , Mars Skae 1 , Raja Padidela 1 , Indraneel Banerjee 2 & Peter Clayton 2
1Department of Paediatric Endocrinology, Manchester Children’s Hospital, Manchester, UK; 2Department of Paediatric Endocrinology, Manchester Academic Health Sciences Centre and Manchester Children’s Hospital, University of Manchester, Manchester, UK; 3Department of Paediatric Endocrinology, Alderhey Children’s Hospital, Liverpool, UK.
Introduction: Neuroglycopaenia is recognised with abnormal neurology and development (Ab Dev) in 26–44% of children with persistent congenital hyperinsulinism (P-CHI). The prevalence of Ab Dev in spontaneously resolving CHI (R-CHI) is not known. We aimed to investigate Ab Dev in R-CHI and P-CHI children in a contemporary cohort.
Methods: All children (n=67) were assessed for Ab Dev in the domains of speech, language, motor and vision, and categorised as mild or severe. All children (>2.5 years at assessment) were classified into 3 groups. i) P-CHI, who had undergone surgery or remained on medical therapy, ii) R-CHI, who were off all treatment and, iii) CHI patients with no clinical concern about development.
Results: The median (range) age at diagnosis of CHI was 1.0 (1; 630) days, while age at follow up was 3.6 (1.8; 13.0) years. Ab Dev was present in 26 (39%) children, of whom 18 (69%) were severe. While spontaneous resolution was achieved in 33 (49%), the prevalence of Ab Dev was similar between R-CHI and P-CHI (30 v 47%, P=0.16). Severe speech, motor and vision abnormalities were present in 61, 50, and 27% respectively with limb weakness being present in 8 (31%) of Ab Dev children. Seizures occurred in 12 (46%) children, including infantile spasms in four children. When variables at diagnosis including prematurity, gender and presence of mutations were tested for correlation with Ab Dev in stepwise backward logistic regression, diazoxide dose (odds ratio (95% confidence intervals) 1.3 (1.1; 1.6), P=0.03) indicating CHI severity, and presentation within one week (5.9 (1.3; 27.9), P=0.02), indicating early diagnosis, were most significant.
Conclusions: Ab Dev was present in a third of children with both R-CHI and P-CHI. The association of Ab Dev with an early presentation and severe disease indicates that early aggressive treatment of hypoglycaemia is important to improve long-term prognosis.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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