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S 25 Lunch Session

S 25 Complex Case Management
Omega 1, 12:30 – 14:00

Atypical hemolityc uremic syndrome (HUS): from diagnosis to new therapies till end-stage renal failure
B. Gianoglio1
1Nephrology, Dialysis and Kidney Transplant Dept, Azienda Ospedaliera Citta' della Salute e della Scienza, Torino, Italy

Family History
Both parents and 2 older twin brothers in good health.
The child’s aunt (mother’s sister) at age 26, developed increase in creatinine, hypertension, severe anaemia. A Hemolityc Uremic Syndrome was diagnosed. She was treated by several plasma exchanges but she developed end-stage renal failure and started chronic emodialysis. At that time (about 15 years ago) no genetic mutations were detected and she wasn't included in kidney transplant list
Results: Case History AD, male, born in 2006.
At the age of 6 months he presented gross haematuria and paleness. The clinical and laboratory investigation showed a of atypical Hemolityc Uremic Syndrome. We started therapy with plasma exchanges.
Genetic analysis showed a complement factor H mutation was found in the child, his mother, his aunt and his grand-mother
From the age of 2 years chronic peritoneal dialysis with 2 more HUS relapses treated by plasma exchanges. For repeated peritoneal catheter infections at age 3 he was switched to chronic haemodialysis (no more relapses of HUS, no signs of haemolisis but repeated CVC infection).
At age 5, before kidney transplant he was treated with Eculizumab (humanized monoclonal antibody to inhibit terminal complement activity in children and adults) repeated on post-transplant day 1 and 7 and every other week thereafter.
His renal function promptly recovered to normal range and in the following 2 years the child was treated by Eculizumab every other week


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