Endocrine Abstracts

Background: Bloom’s syndrome is a rare autosomal recessive disorder due to chromosomal instability. It is associated with endocrinopathies such as growth deficiency, insulin resistance, type 2 diabetes, dyslipidemia and hypothyroidism. We present a case to highlight the challenges in management of diabetic emergencies in patients with complex syndromes.

Case report: A 38-year-old male of Asian descent known to have Bloom’s syndrome presented to A&E with severe diarrhoea and poor oral intake. He had ongoing chemotherapy for mixed phenotype acute leukaemia and Type 2 diabetes of nine years duration treated with oral hypoglycemic agents. During his previous hospital admission, he was discharged on Metformin, Alogliptin and Dapagliflozin. On examination he was short statured, emaciated with BMI 13.4kg/m^2 and signs of severe dehydration. At presentation, Glucose 13 mmol/l, Ketones 6 mmol/l, pH 7.32 and HCO3 14.7 mmol/l, indicative of diabetic ketoacidosis (DKA) likely precipitated by neutropenic sepsis, SGLT-2 use and preceding starvation. A paediatric scale was initially contemplated but was then commenced on the adult fixed rate insulin infusion (FRII), intravenous antibiotics and product transfusion. After 24 hours he had persistent hyperglycaemia with no ketoacidosis. FRII was changed to variable rate insulin infusion (VRII) and he was commenced on Lantus. Blood cultures revealed multiple bacteremias and echocardiography, Chest Xray and Urine microscopy revealed no abnormality. He was on empirical treatment for sepsis. Over the next 24hours glycaemia improved, VRII was stopped, and Lantus continued in glycemic control.

Conclusions: Fluid resuscitation in DKA is guided by severity of dehydration and body weight in pediatric patients; however, in adults it is standardized. We highlight the lack of evidence-based recommendations we for DKA management in adults with requirements that differ from the normal population.