Hyperglycaemia with Ketosis does not always Equate to Diabetic Ketoacidosis

Background: Hyperglycaemic hyperosmolar syndrome (HHS) is a life threatening acute metabolic complication of diabetes now occurring more frequently in the paediatric age group than previously reported1. It is characterised by marked hyperglycaemia, hyper osmolality and severe dehydration without significant ketosis. It should be differentiated from diabetic ketoacidosis (DKA), a more common metabolic complication of diabetes in children which presents with hyperglycaemia, acidosis with moderate or large ketonemia. The relative rarity of HHS in the paediatric age group leads to common misdiagnosis and sub optimal care which ultimately affects treatment outcome.

Objective: It is crucial to differentiate HHS as a distinct entity from DKA because of distinctly different treatment approaches. There is still paucity of data on the management of HHS and severe hypernatraemic dehydration. This report describes an adolescent with complex needs presenting with typical features of HHS with emphasis on potential pitfalls in management.

Method: A female adolescent with background complex medical needs presented with marked hyperglycaemia (56mmol/l) without significant ketonaemia (2.6mmol/L) as a first presentation of type 1 diabetes. Her clinical course was complicated with profound hypernatreamic (>180mm/L) dehydration and acute kidney injury (AKI) which rendered management challenging. She was initially managed on the DKA protocol for the first few hours before HHS diagnosis with consequent significant fluid therapy modification. Her perceived recalcitrant hypernatreamia resulted in overzealous fluid administration although, without negative outcome. Her case buttresses the role of calculating corrected sodium in the management of hypernatremia in hyperglycaemic states. Normalisation of serum sodium level was achieved in one week.

Conclusion: Given that delayed diagnosis of HHS can lead to grave consequences, it is pertinent to consider HHS diagnosis in the face of severe hyperglycaemia with discordant ketosis.