Our experience with atypical hemolytic uremic syndrome
Case Report
A 14-year-old male, presented with complaints of sudden reddish discoloration of urine 5 days back followed by yellowing of eyes and skin, nausea, and vomiting associated with feeds. This was preceded by an episode of high-grade fever. At presentation, his vitals were normal. He was afebrile. Severe pallor, as well as icterus, was present. There was no palpable organomegaly. On routine investigation, he was anemic (hemoglobin 4.9 g%) with thrombocytopenia (platelets 97,000). Total leukocyte counts were normal. Renal functions were deranged with a serum creatinine of 4.27 and blood urea of 116.8. His total bilirubin was 5.3 with an indirect component of 4.1. Kidney sizes were normal with raised cortical echogenicity and a normal renal Doppler study. Serum lactate dehydrogenase levels were raised at 2944 IU/L. A peripheral blood smear revealed dimorphic anemia with thrombocytopenia with polymorphonuclear leukocytosis with abundant schistocytes and tear drop cells suggestive of hemolytic uremic syndrome with a reticulocyte count of 30%. Urine analysis showed granular and hyaline casts and strongly positive test for hemosiderin. Urine output was adequate. Anti-nuclear antibodies were absent with low complement 3 (C3) and normal C4 levels. Anti-complement factor H (CFH) antibodies were found to be significantly raised at 2043 AU/ml. A diagnosis of aHUS was made....
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