Online Articles: Clinical and Laboratory ObservationsAn Atypical Case of Atypical Hemolytic Uremic SyndromeFrancois, Tine MD*; Vande Walle, Johan PhD†; Dhont, Evelyn MD*; Keenswijk, Werner MD†,‡ Author Information Departments of *Pediatric Intensive Care †Pediatrics, Pediatric Nephrology, Ghent University Hospital, Ghent, Belgium ‡Department of Pediatrics, Diakonessenhuis, Paramaribo, Suriname The authors declare no conflict of interest. Reprints: Werner Keenswijk, MD, Department of Pediatrics, Pediatric Nephrology, Ghent University Hospital, De Pintelaan 185, Ghent 9000, Belgium (e-mail: [email protected]). Journal of Pediatric Hematology/Oncology: March 2019 - Volume 41 - Issue 2 - p e111-e113 doi: 10.1097/MPH.0000000000001209 Buy Metrics Abstract We present the case of a 2-month-old infant presenting with pallor and laboratory results showing: hemoglobin 5.1 (10 to 1.5) g/dL, MCV 94.7 (75 to 105) fL, leukocytes 17.4 (7 to 15) ×103/μL, platelets 259 (150 to 450) ×103/μL, hyperbilirubinemia and renal dysfunction. A hemolytic anemia with tubular injury secondary to hemoglobinuria was suspected. Hyperhydration and packed cells were given but she deteriorated. Fluid overload with anuria further complicated the course necessating hemodialysis. Atypical hemolytic uremic syndrome was suspected and eculizumab was administered resulting in rapid improvement. Genetic analysis revealed a mutation in the gene encoding complement factor H and atypical hemolytic uremic syndrome was confirmed. Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.