Abstract
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). 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. By the end of the first week the diagnosis of the typical HUS was established. During the second week the disease progressed into MOF that included 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. 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
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]. On the first postoperative day she appeared confused, dyspnoic, hypotensive, pale, oliguric and her laboratory findings were: hemoglobin (Hb) 60 g/L, hematocrit (Hct) 19%, platelet count 43×109/L, white blood cell (WBC) count 17×109/L, urea 16.6 mmol/L, creatinine 308 μmol/L, creatine kinase 971 U/L, aspartate aminotransferase (AST) 317 U/L, alanine transaminase (ALT) 276 U/L, C-reactive protein (CRP) 175.2 mg/L, lactate dehydrogenase (LDH) 3856 U/L, albumins 19 g/L, complement component 3 (C3) 0.5 g/L, complement component 4 (C4) 0.08 g/L, degree of in vivo hemolysis 50 mg/L, pH 7.265, pCO2 3.64 kPa, pO2 9.2 kPa, HCO3 12 mmol/l, SBE -13.1 mmol/L She was immediately admitted to intensive care where she was analgosedated with 6 mg of midazolam and 200 mcg of fentanyl, intubated with an uncuffed orotracheal tubus and invasive mechanical ventilation was started. We observed the gradual decrease in the WBC count after the initiation of plasmapheresis
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