Congenital nephrotic syndrome: Evolution of medical management and results of renal transplantation

John D. Mahan, S. Michael Mauer, Richard K. Sibley, Robert L. Vernier

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105 Scopus citations

Abstract

We analyzed the clinical course, pathologic findings, and results of aggressive medical management and renal transplantation in 41 infants with onset of nephrotic syndrome in the first 3 months of life. All but one infant with congenital onset failed to thrive and had progressive renal insufficiency; 17 were given steroids or cytotoxic drugs or both, without benefit. Severe bacterial infections occurred in 85% of the infants, pyloric stenosis in 12%, gastroesophageal reflux in 8%, and thrombotic events in 10%. All children prior to the era of renal transplantation died before 4 years of age. The last 24 infants received aggressive medical management, which allowed renal transplantation in 17. Two-year patient and graft survival rates were 82% and 71%, respectively. There was no recurrence of the nephrotic syndrome in the children who underwent transplantation. All but one surviving infants has had normal or accelerated growth, although mean height for the group is 3.1 SD below the mean. School and social performance has been normal in 80%. Thus intensive medical therapy combined with renal transplantation offers a very good opportunity for survival with an acceptable quality of life for infants with congenital nephrotic syndrome.

Original languageEnglish (US)
Pages (from-to)549-557
Number of pages9
JournalThe Journal of pediatrics
Volume105
Issue number4
DOIs
StatePublished - Oct 1984

Bibliographical note

Funding Information:
From the Departments of Pediatrics, Laboratory Medicine, and Pathology University of Minnesota. Supported in part by training grant AM 07087from the National Institutes of Health and by a National Kidney Foundation General Fellowship for 1981-1982. Submitted for publication Nov. 14, 1983; accepted March 30, 1984. Reprint requests: John D. Mahan, M.D., Department of Pediatrics, Box 491 Mayo, University of Minnesota, Minneapolis, MN 55455.

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