2021 Volume 34 Issue 2 Pages 145-151
Atypical hemolytic uremic syndrome (aHUS) is one of the thrombotic microangiopathies caused by uncontrolled complement activation. In recent years, eculizumab therapy has significantly improved its prognosis, and early administration is recommended after diagnosis. A 4-month-old girl whose sister had already been diagnosed with aHUS presented with poor feeding 9 days after her vaccination. On the next day, she became pale and had hematuria detected in a home dipstick urine test; thus, she was brought to her physician. She presented with hemolytic anemia and acute kidney injury and was suspected to have aHUS based on her family history and clinical course. She also had thrombocytopenia when she was referred to our hospital on the following day and was diagnosed with aHUS. She was immediately treated with eculizumab, and her symptoms resolved immediately. Thereafter, she was found to have the same complement factor H (CFH) mutation as her sister. Due to her family history of aHUS, home screening for aHUS was performed on her using a dipstick urinalysis, which allowed early detection and treatment. We believe that home urinalysis is useful in detecting the onset of aHUS, especially after events that can trigger the disease such as vaccination.
