Nonenteropathic Hemolytic Uremic Syndrome: The Experience of a Medical Center

BackgroundHemolytic uremic syndrome (HUS) is an uncommon cause of acute renal failure in children. In contrast to Western countries, most HUSs in Taiwan are caused by Streptococcus pneumoniae. In this article, we demonstrate the clinical courses of children with HUS in Taiwan and try to explain the pathophysiology of complications.MethodsWe retrospectively reviewed the medical records of children with HUS who were admitted to the Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan, between January 1997 and February 2008. The clinical courses, laboratory data, complications, and prognosis were recorded. We divided the patients into dialysis group and non-dialysis group. The laboratory data for the two groups were expressed as mean, standard deviation, and range.ResultsNine patients were enrolled in the study, and all were diagnosed with S pneumoniae–associated HUS. One patient had meningitis, and the others had pneumonia. The mean durations of anemia and thrombocytopenia were 19.2 days and 10.2 days, respectively. Five patients received renal replacement therapy because of oligouria. The average of the peak total bilirubin levels of patients in the dialysis and non-dialysis groups were 24.6±20.7mg/dL and 3.8±1.9mg/dL, respectively. In addition to one patient who had meningitis, four other patients experienced central nervous system (CNS) complications. The mean durations of hypertension in five patients with CNS manifestations and four patients without CNS manifestations were 16.8±7.8 days and 4.8±6.6 days, respectively. Two patients died in acute stage, and most of the others had regained normal renal function at discharge or during follow-up.Conclusion(1) Streptococcus pneumoniae is the most common causative pathogen of HUS in Taiwan; (2) the mean duration of hypertension in patients with CNS manifestations was much longer than that in patients without CNS manifestations; and (3) the average of the peak total bilirubin levels of patients in the dialysis group was much higher than that in the patients of the non-dialysis group