Clinical characteristics and long-term outcome of diarrhea-associated hemolytic uremic syndrome: a single center experience

Objectives To clarify the clinical characteristics and long-term outcomes of patients with diarrhea-associated hemolytic uremic syndrome (D + HUS) with a particular focus on time course. Methods We retrospectively analyzed the medical records of 61 patients with D + HUS who were admitted to Kobe University Hospital between 1995 and 2015. The onset of D + HUS was defined as day 1 of diarrhea. Results The age of onset was 4.1 (1.5–13.4) years, and the period between onset and diagnosis of D + HUS was 5 (3–18) days. The platelet count was lowest on day 7 (4–24), and the lactase dehydrogenase level was maximal on day 8 (4–25). Twenty-three patients required dialysis for 13 (2–37) days, starting at day 5–9. Seventeen patients showed central nervous system (CNS) symptoms at day 4–18. They were followed up for 3.7 (0–18.4) years. At the final follow-up, estimated glomerular filtration rate was 113.7 (57.9–159.9) ml/min/1.73 m 2 with five patients having chronic kidney disease. Three patients developed CNS sequelae. The time to diagnosis was significantly shorter in the group of patients receiving dialysis than without dialysis ( p = 0.018) and in the group with CNS complications than without ( p = 0.013). Conclusion CNS complications were often apparent after blood examination results improved. Moreover, a shorter period between the onset of diarrhea and a diagnosis of D + HUS indicated a more severe clinical course or long-term sequelae, and it should be considered as a risk factor for poor prognosis.