Posted November 15, 200915 yr 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?
December 8, 200915 yr comment_2683 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
December 14, 200915 yr comment_2712 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.
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