Table 2.

Treatment and outcome of the last six Finnish CNS patients with recurrence of proteinuria and nephrotic syndrome after RTx

Patient	Mutation	dgn	RTx, age	Re-nephrosis (no)	Time after RTx (mo)	Therapy	Outcome (+ time since last remission in bold)
1 UTI, polyoma, rejections	Fin-major homozygote	at birth	1y 7mo	1)	51	MP, Cyclo, PE	Remission 7y 7mo
2	Fin-major homozygote	at birth	2y 4mo	1) 2) 3) 4) 5) 6)	4, 5 12 23 26 31 40	MP, Cyclo, PE MP, Cyclo, PE MP, Cyclo, PE MP, Cyclo, PE, ACE MP, Cyclo, PE, ACE MP, Rituxi x4, ACE	Remission “ “ “ “ Remission 6y
3	Fin-major homozygote	at birth	1y 1mo	1) 2)	40 42	MP, Cyclo, PE ACE MP, Rituxi x4 ACE	Remission after 1mo Remission 5y 6mo
4	Fin-major homozygote	at birth	2y 10mo	1) 2)	4 5 20	MP, PE, Rituxi x4 Cyclo MP, PE, Bortetsomibi x4 Rituxi x1	Remission after 12mo still γ-glob. infusions Remission 3y 7mo
5 H1N1	Fin-major homozygote	at birth	1y 4mo	1) 2)	32 60	MP, Cyclo, PE +Rituxi x2 +ACE +Bortetsomibi x4 +Rituxi x1 MP, Rituxi x4 ACE	U-prot: 1.5 g/l Transplant nephropathy, HD
6	Fin-major homozygote	at birth	1y 8mo	1)	13 14	MP, Cyclo, PE Rituxi x2	Remission after 11mo Remission 4y 6mo

RTx renal transplantation, y year, mo month, MP methylprednisolone, Cyclo cyclophosphamide, PE plasma exchange, ACE angiotensin-converting enzyme inhibition, Rituxi Rituximab, CNS congenital nephrotic syndrome, UTI urinary tract infection