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

Abstract

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.

Proposed Screening for Congenital Hyperinsulinism in Newborns: Perspective from a Neonatal–Perinatal Medicine Group

“…The clinical presentation of patients with CHI is heterogeneous. A majority of patients present in the neonatal period [20][21][22][23], and the most severe cases can manifest with profound hypoglycemia with or without signs within hours of birth. Signs can range from nonspecific and subtle (jitteriness and poor feeding) to severe (apnea, reduced consciousness, seizures, coma, and/or death).…”