BSPED2013 Poster Presentations (1) (89 abstracts)
Design and validation of a severity scale for use in congenital hyperinsulinism
Jessica Most 1 , Zainab Mohamed 1 , Hima Bindu Avatapalle 1 , Sarah Ehtisham 1 , Peter Foster 2 , Adam Stevens 3 , Karen E Cosgrove 4 , Mark J Dunne 4 , Indraneel Banerjee 1 & Peter E Clayton 1
1Department of Paediatric Endrocrinology, Royal Manchester Children’s Hospital, Manchester, UK; 2School of Mathematics, University of Manchester, Manchester, UK; 3Faculty of Medical and Human Sciences, University of Manchester, Manchester, UK; 4Faculty of Life Sciences, University of Manchester, Manchester, UK.
Introduction: Congenital hyperinsulinism (CHI) is an important cause of hypoglycaemia in infancy requiring intensive medical and surgical support. Carbohydrate requirement (CHO) represents a simple index of severity but does not predict the failure of medical treatment and hence the requirement for pancreatectomy.
Aims: To design and validate a severity tool for use in early onset CHI patients.
Methods: To design the Manchester CHI severity score (M-CHISS), frequency of blood glucose levels <3.5 mmol/l over 72 h (hypo), maximum dose of glucagon (max-glucagon) (μg/kg per h) and maximum dose of diazoxide (max-diazoxide) (mg/kg per day) as putative markers of severity, were tested for correlation with CHO (mg/kg per min) in a cohort (n=9) with early onset CHI. M-CHISS was validated in an independent cohort (n=29) with 4 years follow-up data, in whom 13 (45%) children had CHI-related mutations and 10 (35%) children underwent pancreatic surgery.
Results: Max-glucagon (standardised coefficient 0.9, R2=0.8, <0.001) but not hypo or max-diazoxide correlated positively with CHO in the design cohort. To derive the M-CHISS tool, CHO and max-glucagon were each categorised into scores of one, two and three, and added together to form a composite score. In the validation cohort, M-CHISS, ranging between one and six, correlated positively with the need for surgery, indicating a severe outcome (odds ratio (95% CI) 3.7 (1.3; 10.6), R2=0.6, P<0.001) in a logistic regression model controlling for age, gender and prematurity. For M-CHISS=6, the probability for requiring surgery was 80% (with a 75% probability of not needing surgery if M-CHISS<6). In contrast, for M-CHISS<3, the probability of not requiring surgery was 100% (with a 53% probability of requiring surgery for M-CHISS≥3).
Conclusions: M-CHISS is a simple and practical tool to assess the severity of hypoglycaemia in early presenting CHI. Further validation is required in prospective cohorts to test the prognostic value of M-CHISS.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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