A stepwise approach to the treatment of early onset nephrotic syndrome

We have retrospectively reviewed our single-center experience of the treatm ent of early onset nephrotic syndrome (NS), From 1991 to 1998, ten children with NS were treated. Kidney biopsy showed focal sclerosis (n=1), diffuse mesangial sclerosis (n=7), and congenital NS of the Finnish type (n=2), Ass ociated conditions included incomplete Drash syndrome (n=1), Galloway-Mowat syndrome (n=1), and severe mental and motor retardation of unknown origin (n=3). From 1991 to 1997, five children with NS were treated. Bilateral nep hrectomy (NX) was performed in three, one patient with severe retardation d ied at 4 years and NX was not performed in one patient who showed satisfact ory growth and development. Three of these children were dialyzed and two w ere successfully transplanted. One patient was transplanted without previou s dialysis, From 1997 to 1998, five children were treated with a regimen th at included captopril and indomethacin (CAPTO/INDO). CAPTO/INDO was success ful in increasing serum protein in all patients and producing growth and de velopment in four patients. In two patients CAPTO/INDO was successful only after unilateral NX, Our experience indicates that CAPTO/INDO may be a valu able treatment in patients with early onset NS. An individualized stepwise approach including unilateral NX should be considered to achieve optimal re sults.