Japanese Journal of Clinical Immunology
Online ISSN : 1349-7413
Print ISSN : 0911-4300
ISSN-L : 0911-4300
Membranoproliferative glomerulonephritis in Wiskott-Aldrich syndrome: improvement by alternative day methylprednisolone therapy
Kimiyuki SaitoYouichi KohnoKiri HonmaMasahiko AoyagiAkira HoshiokaSadao MakinoHideaki KurayamaNaoki ShimojoToshiyuki KatukiMinako TomiitaHiroo NiimiBunshiro Akikusa
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1993 Volume 16 Issue 4 Pages 305-311

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Abstract
This report presents a 2-year-old boy with Wiskott-Aldrich syndrome, who developed membranoproliferative glomerulonephritis. The patient presented microhematuria, prote-inuria and hypertension at one year old when the patient developed recurrent episodes of respiratory infection. A histological study on the renal biopsy specimen revealed a diffuse and global double contour lesion in the capillary wall by light microscopy, but C 3 deposits were not recognized by immunofluorescent study.
There have been reports of nephropathy development in some patients with Wiskott-Aldrich syndrome after treatment with a transfer factor; however, our patient developed renal disease without the use of a transfer factor. The patient improved with methylpred-nisolone, at an alternate day regimen. This case provides valuable information of the clinical features of renal involvement in the course of Wiskott-Aldrich syndrome especially in relation to recurrent respiratory infections.
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© The Japan Society for Clinical Immunology
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