Introduction: Non-ketotic hyperglycaemic hyperosmolar (HHNK) coma is rare in children, is associated with high mortality rate. The incidence of this condition is reported to be increasing and considered to be related to increased prevalence in obesity and type 2 diabetes in children. Hyperglycaemic hyperosmolar syndrome (HHS) is rare in children. The pathophysiology and management of HHS is a distinct entity from diabetic ketoacidosis (DKA).
Case report: We report a case of HHNK coma in a 4-year-old African boy who presented with severe dehydration (pH-6.8, base deficit-27.1, lactate 13.7), non-ketotic hyperglycaemia (blood glucose 49.7 mmol/l), hyperosmolality (400 mOsm/l) and serum sodium of 163 mmol/l. On admission he presented in coma (GCS 3/15) and shock, requiring intensive resuscitation with airway support, fluids (40 ml/kg of fluid boluses) and inotropic support. He was noted to have abdominal distension with a serum amylase of 188 IU/l (normal 30110). With meticulous attention to vigorous initial fluid replacement (10% deficit+maintenance 0.9% saline followed by 0.45% saline) as opposed to over 48 h in DKA and low dose insulin infusion (0.025 IU/kg per h), there was a sustained correction of the hyperglycaemia over 4 days.
His BMI was 16.2-kg/m2 -nonobese, with no acanthosis nigricans, no insulin antibodies but with an elevated insulin level (>100 mIU/l). No family history was available as he was adopted from Uganda. Currently he is in good health with no insulin requirement.
Conclusion: This is a rare case of HHS in childhood, which demonstrates that the use of low dose insulin infusion and strict attention to fluid replacement mitigates against co-morbidity. The learning point: one should always suspect HHS in a child presenting with hyperosmolar hyperglycaemia without ketosis and that pursuing standard management for diabetic ketoacidosis is not indicated.
09 - 11 Nov 2011
British Society for Paediatric Endocrinology and Diabetes