Congenital Nephrotic Syndrome

[JACK]

We had two cases of congenital nephrotic syndrome with poor outcome.

Just wanted to know how has your experience been with this disease? How aggressive are you in the management?

[Guest Dukstar]

Hello Jack,

We recently had 2 CNS (One finnnish type & 1 undetermined type as now) who are 1 year old now. One of the cases was prenatally diagnosed.

At 1 year of age, both are still alive with quite an agresssive managament :

- CVC (Broviak catheter) with high doses of albumin supplementation daily

- Gastrostomy at 3 months of age for continuous night enteral feeding to avoid denutrition

- supplementation in usual deficiencies : phosphocalcic metabolism, hypothyrodoism, etc...

- intravenous Ig if sepsis

- Both were hospitalized in ICU (NICU then PICU) for a while and both developed 1 or more septic shock during the first year

Binephrectomy is programmed, followed by dialysis and hopefully renal transplantation

[JACK]

Am happy to read about your cases. When is the transplantation planned?

[Guest Dukstar]

Renal transplantation is usually planned at about 10 kgs after binephrectomy due to massive protein loss. As weight gain is poor, our patients are still at about 7-8 kgs despite agressive enteral feeding (gastrostomy at 3 months of age). Perhaps, by the end of their second year of life, they will be transplanted after binephrectomy performed by debut 2010.