@article{29100107d81d4aa994d2f82a8066cc3c,
title = "RECURRENCE OF IDIOPATHIC NEPHROTIC SYNDROME AFTER RENAL TRANSPLANTATION",
abstract = "Three patients with steroid-resistant idiopathic nephrotic syndrome were studied at onset and during recurrent nephrotic syndrome after renal transplantation. Renal biopsies at the onset of the nephrotic syndrome showed typical features of the idiopathic nephrotic syndrome; no or minimal focal glomerular abnormalities were present by light microscopy and glomerular-basement-oriented deposits were not demonstrated by immunofluorescent or electron microscopy. Progression to renal failure occurred in 2, 2, and 6 years. Recurrence of nephrotic syndrome was noted 1, 11/2, and 5 months after transplantation. Renal biopsies done 11/2, 5, and 7 months after transplantation when proteinuria was 12·8, 7·6, and 8·5 g. per 24 hours, respectively, showed minimal or no glomerular abnormalities by light microscopy. Immunofluorescent and electron microscopic studies revealed no evidence suggesting immunological injury. Subsequent kidney specimens after transplantation obtained from two of these patients with recurrent nephrotic syndrome showed focal segmental glomerulosclerosis limited primarily to the juxtamedullary glomeruli-a feature further suggesting recurrence of the original disease in the transplanted kidney. These observations suggest that the pathogenesis of the steroid-resistant idiopathic nephrotic syndrome may involve systemic circulating factors; this hypothesis would account for development of a recurrent nephrotic syndrome in the transplanted kidney.",
author = "Hoyer, {John R.} and Leopoldo Raij and Vernier, {Robert L.} and Simmons, {Richard L.} and Najarian, {John S.} and Michael, {Alfred F.}",
note = "Funding Information: Partial renal venous thrombosis was demonstrated radiographically 6 weeks after transplantation of case 2. Renal venograms of cases 1 and 3 showed no evidence of thrombosis. Although the coexistence of renal- vein thrombosis and the nephrotic syndrome is a well-recognised clinical association, the precise patho- genetic relationship has not been established. Con- siderable clinical and experimental evidence, however, indicates that renal-vein thrombosis may be a conse- quence of the nephrotic syndrome. This evidence includes the occurrence of renal-vein thrombosis in the nephrotic syndrome secondary to such diverse diseases as diabetes mellitus,23 amyloidosis,24 systemic lupus erythematosus, 23, 25 and most frequently in membranous glomerulopathy.23,26 Renal venous thrombosis has also been described in minimal-lesion idiopathic nephrotic syndrome. 2 8 The abnormalities of coagulation 25 and fibrinolysis 27 demonstrated in patients with the nephrotic syndrome may predispose to development of thrombotic complications. Renal- vein thrombosis has also been observed as a frequent complication of an experimental model of glomerulo- nephritis in rats (Heymann nephritis), having morphological features similar to those observed in human membranous glomerulopathy.29 However, morpho- logical features of membranous glomerulopathy were not observed in the transplanted kidney of case 2 and have not been produced in the laboratory by partial renal venous occlusion. 26 6 Furthermore, massive proteinuria after transplantation preceded the period of dehydration that presumably led to renal venous thrombosis. Frequency of recurrence of nephrotic syndrome after renal transplantation is also of interest. One additional patient with biopsy-documented idiopathic nephrotic syndrome received a renal transplant at the University of Minnesota during the period 1963-71. Onset of the nephrotic syndrome was at 3 years of age, with progression to renal failure in 11 years. Proteinuria was not observed during the first 2 years after transplantation. Thus, in three out of four patients with the nephrotic syndrome the syndrome recurred after renal transplantation. In the absence of evidence that the recurrent nephrotic syndrome of these patients resulted from recurrent glomerulonephritis or homograft rejection, an alternate aetiology must be considered. An appealing hypothesis is that a circulating humoral substance in these patients injures glomerular membranes, causing increased permeability to protein. The nature of such a factor(s) is not known. Careful clinical studies of patients with the idiopathic nephrotic syndrome after renal transplantation may throw light on the patho- genesis of this disorder. J. R. H. is a National Institutes of Health special postdoctoral fellow AM 50447. This work is supported by grants from the Kational Institutes of Health (AM 12375, AM 05671, AM 13756, HE 06314) and the American Heart Association. Requests for reprints should be addressed to J. R. H., Depart-ment of Pediatrics, University of Minnesota Hospitals, Min- neapolis, Minnesota 55455, U.S.A. Copyright: Copyright 2014 Elsevier B.V., All rights reserved.",
year = "1972",
month = aug,
day = "19",
doi = "10.1016/S0140-6736(72)91734-5",
language = "English (US)",
volume = "300",
pages = "343--348",
journal = "The Lancet",
issn = "0140-6736",
publisher = "Elsevier Limited",
number = "7773",
}