Standardised practices in the networked management of congenital hyperinsulinism: a UK national collaborative consensus.

Congenital hyperinsulinism (CHI) is a condition characterised by severe and recurrent hypoglycaemia in infants and young children caused by inappropriate insulin over-secretion. CHI is of heterogeneous aetiology with a significant genetic component and is often unresponsive to standard medical therapy options. The treatment of CHI can be multifaceted and complex, requiring multidisciplinary input. It is important to manage hypoglycaemia in CHI promptly as the risk of long-term neurodisability arising from neuroglycopaenia is high. The UK CHI consensus on the practice and management of CHI was developed to optimise and harmonise clinical management of patients in centres specialising in CHI as well as in non-specialist centres engaged in collaborative, networked models of care. Using current best practice and a consensus approach, it provides guidance and practical advice in the domains of diagnosis, clinical assessment and treatment to mitigate hypoglycaemia risk and improve long term outcomes for health and well-being.

Delayed Resolution of Feeding Problems in Patients With Congenital Hyperinsulinism.

Background: Congenital Hyperinsulinism (CHI) is the most common cause of recurrent and severe hypoglycaemia in childhood. Feeding problems occur frequently in severe CHI but long-term persistence and rates of resolution have not been described. Methods: All patients with CHI admitted to a specialist center during 2015-2016 were assessed for feeding problems at hospital admission and for three years following discharge, through a combination of specialist speech and language therapy review and parent-report at clinical contact. Results: Twenty-five patients (18% of all patients admitted) with CHI were prospectively identified to have feeding problems related to sucking (n = 6), swallowing (n = 2), vomiting (n = 20), and feed aversion (n = 17) at the time of diagnosis. Sixteen (64%) patients required feeding support by nasogastric/gastrostomy tubes at diagnosis; tube feeding reduced to 4 (16%) patients by one year and 3 (12%) patients by three years. Feed aversion resolved slowly with mean time to resolution of 240 days after discharge; in 15 patients followed up for three years, 6 (24%) continued to report aversion. The mean time (days) to resolution of feeding problems was lower in those who underwent lesionectomy (n = 4) than in those who did not (30 vs. 590, p = 0.009) and significance persisted after adjustment for associated factors (p = 0.015). Conclusion: Feeding problems, particularly feed aversion, are frequent in patients with CHI and require support over several years. By contrast, feeding problems resolve rapidly in patients with focal CHI undergoing curative lesionectomy, suggesting the association of feeding problems with hyperinsulinism.