Intravenous Methylprednisolone Pulse Therapy In Paediatric Patients With Typical Haemolytic Uremic Syndrome And Neurological Complications

Typical haemolytic uremic syndrome (HUS), caused by the enterohaemorrhagic Escherichia coli (EHEC), is thrombotic microangiopathy in which neurological complications often lead to serious long-term sequelae, being also responsible for the majority of deaths in paediatric population. We performed a retrospective study of 89 patients with HUS, at the Nephrology Department of "Maria Sklodowska Curie" Children's Emergency Hospital, Bucharest, Romania. The aim of this study was to determine whether methylprednisolone (mPSL) intravenous pulse therapy could be of clinical benefits in patients diagnosed with HUS and neurological complications. Based on the severity of the cases, the patients were grouped into two categories: patients with mild-HUS (serum creatinine < 1.5mg/dL), were compared to patients with severe-HUS (creatinine serum > 1.5 and neurological complications). Administration of mPSL pulse therapy resulted in significant reductions of neurological complications, and a positive outcome was noted in 15 of 16 patients with severe-HUS. The mPSL therapy did not cause significant adverse effects. Receiver operating characteristic (ROC) curve was constructed to assess the predictive performance on laboratory parameters, in group with severe-HUS, after mPSL administration. The product of haemoglobin showed an AUC (area under the curve) of 0.8105, and serum creatinine was a predictor of severe vs. mild HUS. Serum creatinine showed a good prediction for mPSL administration in severe-HUS group (AUC 0.9453, 95% CI, 0.8577 - 1.00). Our data suggest that mPSL pulse therapy in children with neurological complications during the acute phase of HUS was associated with significant improvement of neurological, haematological, and renal function.