ESPN 51th Annual Meeting

ESPN 2018


 
Limited Dose Eculizumab Treatment in Hemolytic Uremic Syndrome Following Bloody Diarrhea
CANER ALPARSLAN 1 BELDE KASAP DEMIR 2 FATMA MUTLUBAŞ 1 EREN SOYALTIN 1 DEMET ALAYGUT 1 SEÇIL ARSLANSOYU ÇAMLAR 1 ÖNDER YAVAŞCAN 1

1- İZMIR TEPECIK TRAINING AND RESEARCH HOSPITAL DEPARTMENT OF PEDIATRICS DIVISION OF NEPHROLOGY, İZMIR, TURKEY
2- İZMIR KATIP ÇELEBI UNIVERSITY DEPARTMENT OF PEDIATRICS DIVISION OF NEPHROLOGY, İZMIR, TURKEY
 
Introduction:

 Typical hemolytic uremic syndrome is a frequent cause of acute kidney injury in childhood. Herein, we presented the follow up of 4 patients diagnosed with typical HUS treated with Eculizumab, which is usually used for atypical HUS.

Material and methods:

Results:

 4 cases were diagnosed with HUS after bloody diarrhea in our clinic between 2014 and 2107. The cases were between 1 and 2 years old; 2 cases were female and 2 cases were male. Acute kidney injury was present in all cases and 3 cases required renal replacement therapy. ADAMTS 13 levels were normal in all cases. Neurological involvement was present in 2 cases. In 1 case severe gastrointestinal system involvement and invagination developed. Three patients received plasmapheresis. Conventional hemodialysis treatment was applied in 1 patient and 2 patients received continuous hemodiafiltration. In one patient, clinical manifestations and renal functional tests recovered and hemolysis parameters regressed after the first day of eculizumab treatment. 2 patients were treated with eculizumab on the 2nd day of admission. In 1 case, the first dose of eculizumab treatment was applied on the 10th day of admission. Two patients received a second dose of eculizumab because of plasmapheresis application. In all cases, improvement in clinical conditions and renal function tests and regression in hemolysis findings were observed immediately after eculizumab treatment. Eculizumab was discontinued after discharge and recurrent HUS clinic was not observed in any of the patients during the follow-up period. In 2 cases, no mutation was detected in the genetic screening tests for atypical HUS, while results of the other two cases are still expected

Conclusions:

Treatment with limited, mostly one single dose of eculizumab in the early period of typical HUS has a positive effect on survival by regressing hemolysis findings, improving kidney function and reducing the need for renal replacement therapy.