PEDIATRICS Vol. 66 No. 3 September 1980, pp. 391-398
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Nephropathy in the Wiskott-Aldrich Syndrome

Lynn E. Spitler MD1, Betty B. Wray M.D.1, Stanley Mogerman MD1, John J. Miller III MD, PhD1, Richard J. O'Reilly MD1, and Michael Lagios MD1

1 Paul M. Aggeler Memorial Laboratory and the Department of Pathology, Children's Hospital of San Francisco; the Department of Medicine, University of California Medical Center, the Kaiser Permanente Hospital of San Francisco; the Department of Pediatrics, the Medical College of Georgia, Augusta, Georgia; Children's Hospital at Stanford, Department of Pediatrics, Stanford University School of Medicine; and Memorial Sloan-Kettering Cancer Center, New York

Nephropathy was detected in five of 32 patients with the Wiskott-Aldrich syndrome who were participating in a study of transfer factor (TF) therapy. In two patients, nephropathy was present before TF and did not appear changed by TF therapy. One of these patients subsequently developed progressive renal failure requiring dialysis beginning 5frac12 years after TF therapy. In two patients, decreased renal function appeared very soon after the administration of TF. One patient showed gradually decreasing renal function beginning after two years of TF therapy. An additional patient was identified who died with renal failure without having received TF. The results suggest that renal failure occurs in the Wiskott-Aldrich syndrome more frequently than generally recognized and that administration of TF may precipitate or accelerate the renal disease in patients with this syndrome.

Submitted on July 6, 1979
Accepted on December 13, 1979




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