An Atypical Case of Atypical Hemolytic Uremic Syndrome.

Journal of Pediatric Hematology/oncology
Tine FrancoisWerner Keenswijk

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) ×10/μL, platelets 259 (150 to 450) ×10/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.

References

Jun 30, 2007·Journal of the American Society of Nephrology : JASN·Anne-Laure Sellier-LeclercUNKNOWN French Society of Pediatric Nephrology
May 18, 2011·BMC Health Services Research·Jeffrey L WintersChester Andrzejewski
Mar 14, 2012·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Lianne M GeerdinkNicole C A J van de Kar
Jul 24, 2014·Jornal brasileiro de nefrologia : ʹorgão oficial de Sociedades Brasileira e Latino-Americana de Nefrologia·Maria Helena Vaisbich
Oct 24, 2017·Therapeutic Apheresis and Dialysis : Official Peer-reviewed Journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy·Werner Keenswijk, Johan Vande Walle

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