SUCCESSFUL RENAL TRANSPLANTATION IN 3 CHILDREN WITH CONGENITAL NEPHROTIC SYNDROME

J. R. Hoyer; C. M. Kjellstrand; R. L. Simmons; J. S. Najarian; S. M. Mauer; T. J. Buselmeier; A. F. Michael; R. L. Vernier

doi:10.1016/S0140-6736(73)91738-8

SUCCESSFUL RENAL TRANSPLANTATION IN 3 CHILDREN WITH CONGENITAL NEPHROTIC SYNDROME

J. R. Hoyer, C. M. Kjellstrand, R. L. Simmons, J. S. Najarian, S. M. Mauer, T. J. Buselmeier, A. F. Michael, R. L. Vernier

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6 Scopus citations

Abstract

Massive proteinuria and pronounced œdema in the congenital nephrotic syndrome are always resistant to corticosteroid and immunosuppressive therapy. Children with this disease invariably die in early childhood. Kidneys were transplanted to three children with congenital nephotic syndrome (including one of Finnish ancestry) and mild to severe uræmia at 21/2, 21/2, and 41/2 years of age. These three children are now well with normal serum-creatinine levels at 4, 7, and 14 months after renal transplantation and have not had recurrence of nephrosis or significant proteinuria. In contrast, recurrence of the steroid-resistant idiopathic nephrotic syndrome is often seen shortly after transplantation. These observations suggest that the basic defect leading to proteinuria in the congenital nephrotic syndrome resides in the kidney and that renal transplantation may successfully cure this disease.

Original language	English (US)
Pages (from-to)	1410-1412
Number of pages	3
Journal	The Lancet
Volume	301
Issue number	7817
DOIs	https://doi.org/10.1016/S0140-6736(73)91738-8
State	Published - Jun 23 1973

Bibliographical note

Funding Information:
J. R. H. is a special postdoctoral fellow of the National In- stitutes of Health. This work was supported by grants from the National Institutes of Health.

Copyright:
Copyright 2014 Elsevier B.V., All rights reserved.

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@article{941d834bf1e24039bb29ef555bd30592,

title = "SUCCESSFUL RENAL TRANSPLANTATION IN 3 CHILDREN WITH CONGENITAL NEPHROTIC SYNDROME",

abstract = "Massive proteinuria and pronounced {\oe}dema in the congenital nephrotic syndrome are always resistant to corticosteroid and immunosuppressive therapy. Children with this disease invariably die in early childhood. Kidneys were transplanted to three children with congenital nephotic syndrome (including one of Finnish ancestry) and mild to severe ur{\ae}mia at 21/2, 21/2, and 41/2 years of age. These three children are now well with normal serum-creatinine levels at 4, 7, and 14 months after renal transplantation and have not had recurrence of nephrosis or significant proteinuria. In contrast, recurrence of the steroid-resistant idiopathic nephrotic syndrome is often seen shortly after transplantation. These observations suggest that the basic defect leading to proteinuria in the congenital nephrotic syndrome resides in the kidney and that renal transplantation may successfully cure this disease.",

author = "Hoyer, {J. R.} and Kjellstrand, {C. M.} and Simmons, {R. L.} and Najarian, {J. S.} and Mauer, {S. M.} and Buselmeier, {T. J.} and Michael, {A. F.} and Vernier, {R. L.}",

note = "Funding Information: J. R. H. is a special postdoctoral fellow of the National In- stitutes of Health. This work was supported by grants from the National Institutes of Health. Copyright: Copyright 2014 Elsevier B.V., All rights reserved.",

year = "1973",

month = jun,

day = "23",

doi = "10.1016/S0140-6736(73)91738-8",

language = "English (US)",

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pages = "1410--1412",

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T1 - SUCCESSFUL RENAL TRANSPLANTATION IN 3 CHILDREN WITH CONGENITAL NEPHROTIC SYNDROME

AU - Hoyer, J. R.

AU - Kjellstrand, C. M.

AU - Simmons, R. L.

AU - Najarian, J. S.

AU - Mauer, S. M.

AU - Buselmeier, T. J.

AU - Michael, A. F.

AU - Vernier, R. L.

N1 - Funding Information: J. R. H. is a special postdoctoral fellow of the National In- stitutes of Health. This work was supported by grants from the National Institutes of Health. Copyright: Copyright 2014 Elsevier B.V., All rights reserved.

PY - 1973/6/23

Y1 - 1973/6/23

N2 - Massive proteinuria and pronounced œdema in the congenital nephrotic syndrome are always resistant to corticosteroid and immunosuppressive therapy. Children with this disease invariably die in early childhood. Kidneys were transplanted to three children with congenital nephotic syndrome (including one of Finnish ancestry) and mild to severe uræmia at 21/2, 21/2, and 41/2 years of age. These three children are now well with normal serum-creatinine levels at 4, 7, and 14 months after renal transplantation and have not had recurrence of nephrosis or significant proteinuria. In contrast, recurrence of the steroid-resistant idiopathic nephrotic syndrome is often seen shortly after transplantation. These observations suggest that the basic defect leading to proteinuria in the congenital nephrotic syndrome resides in the kidney and that renal transplantation may successfully cure this disease.

AB - Massive proteinuria and pronounced œdema in the congenital nephrotic syndrome are always resistant to corticosteroid and immunosuppressive therapy. Children with this disease invariably die in early childhood. Kidneys were transplanted to three children with congenital nephotic syndrome (including one of Finnish ancestry) and mild to severe uræmia at 21/2, 21/2, and 41/2 years of age. These three children are now well with normal serum-creatinine levels at 4, 7, and 14 months after renal transplantation and have not had recurrence of nephrosis or significant proteinuria. In contrast, recurrence of the steroid-resistant idiopathic nephrotic syndrome is often seen shortly after transplantation. These observations suggest that the basic defect leading to proteinuria in the congenital nephrotic syndrome resides in the kidney and that renal transplantation may successfully cure this disease.

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SUCCESSFUL RENAL TRANSPLANTATION IN 3 CHILDREN WITH CONGENITAL NEPHROTIC SYNDROME

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