Nephrotic syndrome, congenital

Nephrotic syndrome that presents during the first 3 months of life: protein leakage, hypoalbuminemia, edemas.

Treatment: albumin IV, treatment with conversion enzyme inhibitor to decrease proteinuria, uni- or bilateral nephrectomy, peritoneal dialysis in case of renal failure, early kidney transplantation. Significant morbidity and mortality.


Variable genetic origin:



Anesthetic implications:

check blood electrolytes, creatininemia, total blood counts; difficult venous access; risk of thrombosis (central venous access); antibioprophylaxis.

Treatment with conversion enzyme inhibitor. Management of the volemia. Peritoneal dialysis or hemodialysis. Risk of persistent arterial hypotension and neurological complications after bilateral nephrectomy.


References :

-            van Lieburg AF, Monnens LAH.
Persistent arterial hypotension after bilateral nephrectomy in a 4-month-old infant.
Pediatr Nephrol 2001 ; 16 :604-5

-        Dufek S, Hollta T, Trautmann A, Ylinen E, Alpay H et al.
Management of children with congenital nephrotic syndrome : challenging treatment paradigms.
Nephrol Dial Transplant 2019 ; 34 :1369-77


Updated: May 2021