Simultaneous onset of steroid resistant nephrotic syndrome and IDDM in two young children

Abstract

The cases are reported of two young children who developed insulin-dependent diabetes mellitus (IDDM) within 2 weeks of receiving a diagnosis of nephrotic syndrome. Neither patient responded to 8 weeks of daily prednisolone. The first patient presented at 2 years and 9 months of age. Her renal biopsy showed mesangial proliferation. The second child presented with steroid resistant nephrotic syndrome at 18 months of age and developed IDDM 2 weeks later. He achieved partial remission with cyclosporine therapy. His initial renal biopsy at 3 years of age showed minimal change disease and follow-up renal biopsy at 5 years of age showed early diabetic glomerulosclerosis. Tests for NPHS2 and WT1 genetic mutations were negative in both patients. To our knowledge this is the first report of steroid resistant nephrotic syndrome with almost simultaneous onset of IDDM in young children.

Figure 1

Micrographs of the renal biopsy findings in case 1. (A) Light micrograph of a glomerulus showing mainly mesangial matrix expansion and segmental mild increase in mesangial cells (thick arrow) (periodic acid-Schiff stain). (B) A glomerulus showing positive immunofluoresence staining for immunoglobulin M in the mesangial area. (C) Electron micrograph demonstrating expansion of mesangial matrix (arrows) and thickened glomerular basement membrane.

Figure 2

Micrographs of the renal biopsy findings in case 2. (A) Light micrograph of a glomerulus exhibiting mild mesangial matrix expansion (arrow) (periodic acid-Schiff stain). (B) A glomerulus showing negative immunofluoresence staining. (C) Electron micrograph showing increased mesangial matrix with nodule formation (arrows).