Abstract

Congenital nephrotic syndrome of Finnish type is a rare disease in Taiwan characterized by intrauterine onset of massive urinary loss of protein. We describe a typical baby of congenital nephrotic syndrome with generalized edema occurring at 3 months of age. Renal biopsy at 4 months of age showed a tubular microcystic change in histology. He had a partial response to corticosteroid. We tried persantin, indomethacin, and captopril since 10 months of age without significant improvement. The baby suffered from recurrent infections and respiratory difficulties due to having upper airway edema since 3 months of age. The baby passed away at 1 year and 2 months of age with severe psychomotor retardation. The first try of the combination of persantin, indomethacin, and captopril for congenital nephrotic syndrome is described.

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