A Case Report of Atypical Hemolytic Uremic Syndrome Presenting with Massive Proteinuria

摘要

Atypical hemolytic uremic syndrome (HUS) is most commonly due to dysregulation of the alternative complement pathway. It is characterized by the classic triad of hemolytic anemia, thrombocytopenia, and acute kidney injury. Treatment includes supportive care including hemodialysis along with steroids, plasmapheresis, and eculizumab. We report a case of a 22 year old female who was diagnosed with atypical HUS and was also noted to have a severe degree of proteinuria, nearly 24 grams in the 24 hour urine collection. Proteinuria is observed in cases of atypical HUS because of the glomerular leakage of protein due to severe endothelial and epithelial injury. However, there is little data directly addressing the degree of proteinuria observed in these patients. This leads us to ask whether the degree of proteinuria has any prognostic significance or impact on disease progression and recovery.