Background: The hyperosmolar hyperglycaemic state (HHS) is rare, but recognised, life-threatening clinical entity in children with type 2 diabetes (T2DM). It is also reported as presenting feature in other types of diabetes and metabolic disorders. The estimated mortality in HHS is 1020%, ten times higher than Diabetic Ketoacidosis (DKA).The mainstay of management involves aggressive fluid therapy with insulin and managing complications namely; rhabdomyolysis, multi-organ failure, cerebral oedema and malignant hyperthermia.
Objective: We report three cases, where HHS was the presenting feature, only one of which has confirmed T2DM, highlighting the challenges for early diagnosis and management.
Case-series report: i) 16-year-old-Asian female, with neurodegenerative disease, immobility, pubertal delay and obesity (BMI 42) presented acutely unwell. Examination revealed Acanthosis Nigricans. She had a complicated course with acute kidney injury, vascular thrombosis and cerebral oedema. ii) 4.5-year-old-Black African male, BMI 16.5, previously well, presented with abdominal distention and collapse (GCS 3/15). Aetiology of presentation was unclear; fluids and insulin were stopped within 4 days. Recovery was complete. iii) 8-year-old Caucasian girl with quadriplegic cerebral palsy and Haemophagocytic Lymphohistiocytosis, receiving pulsed steroid therapy, presented with stupor and severe dehydration. Recovery was to premorbid state. Management in all cases involved aggressive isotonic fluid resuscitation and insulin therapy. Investigations consistent with HHS are shown below.
|Investigations||Case 1||Case 2||Case 3|
|Blood glucose (mmol/l)||33||47||38|
|Blood Ketones (mmol/l)||1.8||Negative||2.4|
Conclusion: This heterogeneous case series includes new-onset T2DM, steroid induced and transient HHS with complete recovery. The latter two have not been previously described in the literature. Significant neurodevelopmental delay in two of our patients delayed diagnosis similar to other case reports. There is imperative need for increased awareness and evidence-based paediatric guidelines for management of HHS to avert adverse outcomes.
01 - 03 Oct 2015
European Society for Paediatric Endocrinology