Severe form of hemolytic-uremic syndrome with multiple organ failure in a child: a case report

Dino Mijatovic,Ana Blagaic,Zeljko Zupan

doi:10.12688/f1000research.2762

Abstract

Introduction: Hemolytic-uremic syndrome (HUS) is a leading cause of acute renal failure in infants and young children. It is traditionally defined as a triad of acute renal failure, hemolytic anemia and thrombocytopenia that occur within a week after prodromal hemorrhagic enterocolitis. Severe cases can also be presented by acute respiratory distress syndrome (ARDS), toxic megacolon with ileus, pancreatitis, central nervous system (CNS) disorders and multiple organ failure (MOF). Case presentation: A previously healthy 4-year old Caucasian girl developed acute renal failure, thrombocytopenia and hemolytic anemia following a short episode of abdominal pain and bloody diarrhea. In the next week of, what initially appeared as typical HUS, she developed MOF, including ileus, pancreatitis, hepatitis, coma and ARDS, accompanied by hemodynamic instability and extreme leukocytosis. Nonetheless, the girl made a complete recovery after one month of the disease. She was successfully treated in the intensive care unit and significant improvement was noticed after plasmapheresis and continuous veno-venous hemodialysis. Conclusions: Early start of plasmapheresis and meticulous supportive treatment in the intensive care unit, including renal placement therapy, may be the therapy of choice in severe cases of HUS presented by MOF. Monitoring of prognostic factors is important for early performance of appropriate diagnostic and therapeutical interventions.

Highlights

Hemolytic-uremic syndrome (HUS) is a leading cause of acute renal failure in infants and young children
In typical HUS, those reactions are initiated by binding of STx to the endothelial membrane-bound Gb3 receptor and subsequent activation of platelets and leukocytes[3], while in atypical HUS, those reactions are mediated by excessive activation of complement
Here we presented the case of a 4-year old girl who developed a severe form of typical HUS which initially presented classically, but rapidly progressed into multiple organ failure (MOF) which severely affected the central nervous system, renal, gastrointestinal and cardiovascular function, and induced acute respiratory distress syndrome (ARDS) and hematological disorders

Summary

Introduction

Hemolytic-uremic syndrome (HUS) can be classified as typical, usually when provoked by Shiga-toxin (STx) produced by enterohemorrhagic Escherichia coli serotype H157:O7, or atypical, when triggered by other microbes’ antigens and toxins[1]. During the first week of disease she received multiple transfusions of packed red blood cells, fresh frozen plasma and platelets as continuous adrenergic and diuretic support was established with 5–8 mcg/kg/min of dopamine and 50 mg/24 h of furosemide, respectively. The renal replacement therapy was only stopped for 3 hours a day in order to carry out plasmapheresis which was initiated on the 8th day of disease This lasted for 10 days, for 2 hours each day and 40 ml/kg of fresh frozen plasma was used per day. We observed the gradual decrease in the WBC count after the initiation of plasmapheresis and its subsequent normalization by the end of the third week of disease. This trend was correlated with the values of LDH plasma levels (Figure 2). Respiratory function completely recovered by the end of the third week of disease without apparent

15 Course of disease 22

Findings

Discussion and conclusion