Endocrine Abstracts

Introduction: A 7 year old boy with history of Joubert’s syndrome presented with first presentation of type 1 diabetes mellitus in Diabetic ketoacidosis (DKA) and a hyperglycaemic hyperosmolar state (HHS). His underlying neurological condition made clinical assessment difficult and he had reduced GCS with intermittent apnoeas but no respiratory compromise. Using the BSPED guideline (2015) for DKA he was managed with cautious fluid replacement despite his hyperosmolar state as he did not demonstrate clinical evidence of shock.

Methods: IV fluids were started at maintenance rates with 5% rehydration as 0.9% saline and replacement of urinary losses ml for ml. First laboratory blood glucose was 95.7 mmol/l, pH 7.21, blood ketones 5.9, serum osmolality 422 osmol/l. Blood pH and ketones first normalised at 18 hours from starting insulin therapy at 0.05 units/kg per hour. Intravenous heparin infusion was used to reduce risk of thrombosis.

Results: Serum sodium (corrected for glucose level) was 148 and rose to 200 mmol/l (estimated as laboratory assay range usual cut off 183 mmo/l) on day 3. iv Fluid rates were unchanged but content changed to 0.45% sodium chloride. Rhabdomyolysis ensued from day 3 (peak CK 40,000 day 5). Serum sodium normalised by day 10 with no further intervention. Renal function (peak creatinine 290 mmo/l) normalised by day 8 of admission. Serum antibodies were high for GAD, IA-2 and Zn transporter antibodies confirming likely type 1 diabetes mellitus. The child made a full recovery and is now managed on MDI insulin regimen of 0.5 units/kg per day.

Discussion: HHS is more typically seen in adult patients or adolescents usually with type 2 diabetes mellitus. It has high associated mortality rate (10–20%) and high volume iv fluid resuscitation and haemofiltration are recommended due to paucity of paediatric data in the literature. However, this case illustrates that DKA and HHS can co-exist in a young patient with type 1 diabetes. There is limited literature to suggest it more commonly occurs in children with type 1 DM and learning difficulties. Despite very high osmolality and severe hypernatraemia slow fluid replacement was associated with a successful outcome in this case.