LAUSR

A 6-year-old girl with E beta thalassemia on regular blood transfusion and iron chelation therapy was brought to the emergency room with vomiting for the previous 2 days, low grade temperature, and decreasing consciousness. On examination, her distal extremities were cool; she was pale, tachycardic, tachypneic, and had a Glasgow Coma Scale (GCS) of 9/15 without any meningeal signs. Initial investigations including arterial blood gas (ABG) are shown in Table 1. Subsequently, the child was stabilized with intravenous f luids and broad-spectrum antibiotics as well as acyclovir. Despite hemodynamic stabilization, her encephalopathy persisted and her repeat blood investigation at 8 h revealed worsening liver function tests and elevated ammonia (Table 1). Repeat ABG showed persisting metabolic acidosis despite improving serum lactate. Magnetic resonance imaging (MRI) of the brain was reported normal. Child responded well with ammonia-scavenging therapy with sodium benzoate (500 mg/kg/day) and other liver protective measures. Her GCS normalized by 48 h postadmission but metabolic acidosis, hypokalemia, and hypophosphatemia persisted. In addition, her urine dipstick was noted to be 2 + for proteinuria although blood sugar was absent. Her spot urinary protein creatinine ratio (UPCR) was elevated. In summary, we have a 6-year-old girl with thalassemia on regular iron chelation therapy and blood transfusion presenting with acute kidney injury, acute liver failure, persisting metabolic acidosis along with hypokalemia, hypophosphatemia, and proteinuria.