A case of allogenic renal transplantation in patient with Denys-Drash syndrome

Abstract

  We reported a boy with Denys-Drash syndrome who received allogenic renal transplantation at the age of 31 months. He had hypospadias and bilateral cryptorchidism at birth, and presented with nephrotic syndrome at the age of 18 months. 2 months later, he developed renal failure with severe hypertension and started on continuous ambulatory peritoneal dialysis (CAPD).
  The histological findings of a renal biopsy showed diffuse glomerulosclerosis and tubullar atrophy.
  Chromosomal analysis showed a normal make karyotype, but molecular genetic analysis revealed C to T transition missense mutation at amino acid 394 in exon 9 at one allele of WT 1 gene, and the diagosis was made.
  At the age of 2 year 7 months, he received allogenic renal transplantation with bilateral nephrectomy and the post-operative course was uneventful with good renal function on immunosuppresion consisting of methylpredonisolone, mizoribine and tacrolimus.