Fatal Hyperosmolar Hyperglycaemic Syndrome complicated by severe Rhabdomyolysis

Abstract

A 49-year-old African man, with no known past medical history, presented to the emergency department with three day history of vomiting, lethargy, confusion and drowsiness on a background of two week history of thirst, polyuria with drinking up to 10 pints of water a day. More recently he had been drinking litres of non-diet lemonade in an effort to sustain energy levels. On examination, Glasgow Coma Score was 6 (eyes 4, verbal 1, motor 1). He was severely dehydrated with dry mucous membranes, blood pressure 75/40 mmHg, pulse rate 120 bpm and cool peripheries. He was pyrexial 38.5°C, and hypoxic (oxygen saturations 84% on room air). Cardiovascular and respiratory examination was otherwise unremarkable. On arrival to the emergency department, he was intubated and ventilated due to hypoxaemia and poor conscious level. Baseline investigations are shown in Box 1. Chest radiograph showed patchy consolidation in the right base, suggestive of an aspiration pneumonia. A diagnosis of HHS, acute renal failure and severe mixed metabolic and respiratory acidosis was made. The patient was managed on the intensive care unit (ICU) with careful fluid resuscitation with 0.9% saline, according to central venous pressure. Slow reduction of plasma glucose was an explicit aim, using a maximum of 1 unit per hour of intravenous insulin, aiming to reduce plasma glucose by no faster than 1 mmol/L every 4 hours.1 He was fully anticoagulated in view of his high risk of stroke, and was given broad spectrum antibiotics. On ICU, he required inotropic support to maintain blood pressure, and haemofiltration in view of his acute renal failure and severe acidosis. Box 1 Investigations on admission Sodium 157 mmol/L (136–146 mmol/L) Corrected Sodium 181 mmol/L (136–146 mmol/L) Potassium 3.9 mmol/L (3.5–5.1 mmol/L) Urea 23.7 mmol/L (2.5–6.4 mmol/L) Creatinine 253 µmol/L (62–106 µmol/L) Creatine Kinase 1097 mmol/L (9–168 IU/L) Calcium 2.45 mmol/L (2.15–2.58 mmol/L) Phosphate 1.52 mmol/L (0.74–1.52 mmol/L) Serum osmolality 480 mOsm/kg (280–295 mOsm/kg) Haemoglobin 14.8 g/dl (11.5–16.6) White cell count 17.6 × 109/l (4–11) Platelets 211 × 109/l (150–400) Plasma Glucose 90.3 mmol/L (4–7 mmol/L) pH 6.998 (7.35–7.45) pCO2 5.54 kPa (4.67–6.40 kPa) pO2 (on 100% oxygen) 12.9 kPa (11.1–14.4 kPa) Bicarbonate 14.3 mmol/L (22–26 mmol/L) Lactate 5.6 mmol/L (0.5–1.6 mmol/L) Base Excess −12.3 mmol/L (−3–+3 mmol/L) Chest radiograph Right sided volume loss and patchy consolidation ECG Sinus tachycardia Urinalysis Protein + Ketones trace Blood + + + Leucocytes negative Nitrites negative View it in a separate window His condition did not improve despite full intensive care. Serum creatine kinase continued to increase to a peak of 417,907 IU/L, and a presumptive diagnosis of compartment syndrome leading to muscle infarction and acute rhabdomyolysis was made, which was supported by raised compartment pressures in the thigh and calf muscles. He underwent multiple fasciotomies, which confirmed necrotic muscle. His acidosis did not resolve despite haemofiltration, and oxygenation deteriorated, probably as a result of acute lung injury. He developed uncontrollable bleeding from disseminated intravascular coagulopathy, and after careful discussion with his family, further support was withdrawn, and he died 72 hours after arrival in hospital. Post mortem examination showed a fatty liver, evidence of muscle infarction in the legs, but no evidence of cardiovascular or cerebrovascular events.