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

• Published in: Archives of disease in childhood 2023-07, Vol.108 (Suppl 2), p.A262-A262
• Main Authors: Shaikh, Guftar, Worth, Chris, Noina Abid, Avatapalle, Bindu, Chesover, Alexander, Dastamani, Antonia, Mohammed Didi, Kapoor, Ritika, Salomon-Estebanez, Maria, Senniappan, Senthil, Shah, Pratik, Banerjee, Indraneel
• Format: Article
• Language: English
• Subjects: Carbohydrates; Collaboration; Dextrose; Endocrinology; Enteral feeding; Enteral nutrition; Evidence; Genetic screening; Glucagon; Glucose; Glucose monitoring; Hypertension; Hypoglycemia; Insulin; Long-term effects; Medical Services; Medical treatment; Octreotide; Patients; Pediatrics
• ISSN: 0003-9888; 1468-2044
• DOI: 10.1136/archdischild-2023-rcpch.416

ObjectivesCongenital hyperinsulinism (CHI) is a heterogeneous condition characterised by severe and recurrent hypoglycaemia caused by inappropriate insulin over-secretion and often unresponsive to standard medical therapy. Treatment is multifaceted and complex, requiring prompt multidisciplinary input to minimise the risk of neurodisability arising from neuroglycopaenia. The UK CHI consensus on the practice and management of CHI was developed to optimise and harmonise clinical management of patients in specialist and non-specialist centres engaged in collaborative, networked care.MethodsA UK consensus group, derived from multidisciplinary healthcare professionals with extensive experience of CHI met regularly to discuss available evidence and experience. Wherever possible, recommendations were based on high levels of evidence but when evidence was deemed insufficient or inadequate, authors discussed a consensus of experiences to frame a working recommendation.ResultsThe diagnosis of CHI is predicated on the finding of detectable serum insulin at the time of hypoglycaemia 8mg/kg/minute).Suspected or confirmed cases of CHI should be discussed with tertiary paediatric endocrinology centres with onward referral to a highly specialised centre if required. Initial management should focus on maintaining glucose ≥3.5mmol/L through escalating dextrose concentrations and glucagon infusions, ensuring fluid overload is avoided.Following glycaemic stabilisation, diazoxide is the first-line medical treatment and response must be carefully monitored, due to the risk of fluid retention and pulmonary hypertension. Poor response to diazoxide (persistent glycaemic instability with dose ≥10mg/kg/day) should prompt liaison with a specialised centre for consideration of second-line treatment e.g. octreotide, genetic testing and imaging, with surgery as per consensus management pathway (figure 1).Enteral feeding should be continued if possible and may require the addition of supplemental carbohydrates in discussion with a specialist dietician. Glucose monitoring should be regularly performed by glucometer and guidance with continuous glucose monitoring may be helpful under specialist supervision.At discharge, patients must be able to safely fast (glucose ≥3.5mmol/L) for 6 hours and have received training on glucose monitoring as well as the detection and treatment of hypoglycaemia. Follow-up