To the Editor: Atypical hemolytic–uremic syndrome of infancyis a rare disorder that is associated with thrombotic microangiopathyand acute renal failure. It often involves complement dysregulation.1,2Plasma infusions have variable efficacy, and end-stage renaldisease often develops in children who are unresponsive to plasmatherapy.1,2 We report on a patient with congenital relapsingatypical hemolytic–uremic syndrome who was unresponsiveto plasma therapy but had a response to eculizumab, a humanizedmonoclonal antibody against terminal complement protein C5.3
An 18-month-old boy was admitted with a fourth relapse of congenitalatypical hemolytic–uremic syndrome. He was born at 34weeks' gestation, and . . . [Full Text of this Article]
Hirt-Minkowski, P., Schaub, S., Mayr, M., Schifferli, J. A., Dickenmann, M., Fremeaux-Bacchi, V., Steiger, J.
(2009). Haemolytic uraemic syndrome caused by factor H mutation: is single kidney transplantation under intensive plasmatherapy an option?. Nephrol Dial Transplant
24: 3548-3551
[Abstract][Full Text]
Noris, M., Remuzzi, G.
(2009). Atypical Hemolytic-Uremic Syndrome. NEJM
361: 1676-1687
[Full Text]
Mache, C. J., Acham-Roschitz, B., Fremeaux-Bacchi, V., Kirschfink, M., Zipfel, P. F., Roedl, S., Vester, U., Ring, E.
(2009). Complement Inhibitor Eculizumab in Atypical Hemolytic Uremic Syndrome. CJASN
4: 1312-1316
[Abstract][Full Text]
Lhotta, K., Janecke, A. R., Scheiring, J., Petzlberger, B., Giner, T., Fally, V., Wurzner, R., Zimmerhackl, L. B., Mayer, G., Fremeaux-Bacchi, V.
(2009). A Large Family with a Gain-of-Function Mutation of Complement C3 Predisposing to Atypical Hemolytic Uremic Syndrome, Microhematuria, Hypertension and Chronic Renal Failure. CJASN
4: 1356-1362
[Abstract][Full Text]
Shin, J. I., Lee, J. S., Gruppo, R. A.
(2009). More on Eculizumab for Congenital Atypical Hemolytic-Uremic Syndrome. NEJM
360: 2142-2143
[Full Text]
Grunfeld, J.-P.
(2009). Lessons from Rare Renal and Adrenal Diseases. CJASN
4: 870-872
[Full Text]