Extracorporeal Blood Purification Therapy to Deal a Deferasirox Induced Life-Threatening Hepatic Encephalopathy in a Septic Child With Sickle-Cell Disease: A Case Report

Abstract

This report details a rare pediatric case of hyperammonemic encephalopathy caused by the oral iron chelating drug deferasirox (DFR) in a septic patient. It is our contention that this study lends support to the existing literature, as it describes the case of a 15-year-old female patient with a history of sickle-cell disease who presented with a fever and vomiting, rapid development of sleepiness, consciousness disturbances, medium mydriasis, neck stiffness, and trismus with seizure. Her Glasgow Coma Scale (GCS) score was 5. Laboratory tests revealed an increase in creatinine, metabolic acidosis, hyperammonemia, high cerebrospinal fluid (CSF) glutamine levels, alterations in coagulation and in liver function, rising inflammatory markers, cerebral oedema on brain Computerized Tomography (CT) scan, 10^6 copies/ml of Methicillin-Resistant Staphylococcus Aureus (MRSA) in pulmonary swab film array, and elevated DFR blood level. The treatment plan involved the early cessation of DFR, the correction of acidosis, mechanical ventilation, mannitol and bioarginine, vasoactive drug, antibiotics, and supportive care with continuous veno-venous hemodiafiltration (CVVHDF) for hyperammonemia and therapeutic plasma exchange (TPE) for a high CSF glutamine level resulting from cytotoxic encephalopathy. The patient successfully overcame the multiorgan failure, with no permanent neurologic complications. It is our opinion that healthcare providers and family caregivers of patients with chronic disease may be particularly attuned to the emergence of any sign or symptom, and thus well positioned to take prompt action to avert life-threatening clinical deterioration due to rising DFR levels. It is recommended that critical care providers commence extracorporeal blood purification therapies (EBPT) at the earliest opportunity, taking care to adapt the technique to the specific needs of the patient and to avoid the potential for fatal neurological complications.

Keywords: continuous renal replacement therapy; deferasirox; encephalopathy; hyperammonemia; liver failure; plasma exchange; sepsis.

FIGURE 1

DFR and NH4 trend.