Abstract
We encountered four cases of congenital nephrotic syndrome (CNS). The treatment schedule for CNS was planned as follows; (1) Albumin was administered by a central venous catheter (CVC). (2) Hemi-nephrectomy was performed at three to four months of age in each patient and peritoneal dialysis (PD) was started after the removal of the residual kidney around one year of age when weaning was completed. (3) The kidney transplantation was done around five years of age.
Although one patient died due to severe infection, the other three completed the kidney transplantation successfully as planned. Ten episodes of catheter infection occurred during the total 1,782 days of CVCs use. Four episodes of PD-associated peritonitis occurred during the total 4,927 days of PD treatment. There was no occlusion of mid or large sized veins due to complications of CVC placement. The surviving patients show normal height and mental development to date. We report the details of the successful treatments and the courses in patients with CNS, a disease that was previously described as having a poor prognosis.
