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PATHOGENETIC VARIABILITY OF NEPHROPATHY (N) IN JUVENILE DIABETES MELLITUS (JDM)

Clinicopathologic studies of 4 patients with JDM and N demonstrated the pathogenetic variability of the nephropathy. Only 1 patient had the typical diabetic N in form of proteinuria (P), hypertension (H), decreased glomerular filtration rate (GFR) and diffuse and nodular diabetic glomerulosclerosis (DG). In another patient the N had the features of steroid responsive nephrotic syndrome (SRNS) of childhood superimposed on a very mild DG. This patient had HLA-B12 antigen associated with SRNS. The third patient also had a nephrotic syndrome (NS) which, unlike the other, responded only partially to treatment. In addition, she had Grave's disease (GD). Kidney biopsy 9 years after appearance of the NS showed only mild DG. The presence of HLA-B8 antigen, shown to be associated with SRNS, JDM and GD could be suggestive of a common pathogenesis of all 3 disorders in this patient. In the fourth patient, the N in form of H, P and rapidly declining GFR could have been related more to the severe tubulointerstitial disease of unknown etiology than to the moderate DO. The presence of renal tubular epithelial antigen in the mesangium in this case, may have had some pathogenetic significance. The poor prognosis of diabetic N warrants a careful search for other potentially treatable, causes of N in patients with JDM.