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Table of Contents
Vol. 21, No. 1, 2001
Issue release date: January – February
Section title: Case Report
Am J Nephrol 2001;21:58–62
(DOI:10.1159/000046221)

IgA Nephropathy, Antineutrophil Cytoplasmic Antibodies and Crescentic Glomerulonephritis in a Patient with the Hermansky-Pudlak Syndrome

Tagboto S. · Carr S. · Varghese A. · Allen A. · Feehally J. · Furness P.
Leicester General Hospital, Leicester, UK

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Article / Publication Details

First-Page Preview
Abstract of Case Report

Published online: March 12, 2001
Issue release date: January – February

Number of Print Pages: 5
Number of Figures: 2
Number of Tables: 0

ISSN: 0250-8095 (Print)
eISSN: 1421-9670 (Online)

For additional information: http://www.karger.com/AJN

Abstract

Hermansky-Pudlak syndrome is an uncommon cause of renal dysfunction. Because of the risk of bleeding in this condition, few patients have undergone a renal biopsy. Renal dysfunction has been attributed to the deposition of ceroid pigment in the tubules and interstitial fibrosis. We report a case with renal biopsy findings of ceroid deposition and interstitial fibrosis, but also of mesangial IgA deposition, crescentic glomerulonephritis, and an interstitial lymphocytic infiltrate. Furthermore, perinuclear antineutrophil cytoplasmic antibodies of the IgG subclass were detected in a blood sample. It is well known that ceroid pigment in this syndrome accumulates in monocytes, macrophages and T lymphocytes and it has been suggested that this may affect their function. We suggest that this novel combination of renal changes might be explained on the basis of alterations in immune mechanisms in the Hermansky-Pudlak syndrome.

© 2001 S. Karger AG, Basel


Article / Publication Details

First-Page Preview
Abstract of Case Report

Published online: March 12, 2001
Issue release date: January – February

Number of Print Pages: 5
Number of Figures: 2
Number of Tables: 0

ISSN: 0250-8095 (Print)
eISSN: 1421-9670 (Online)

For additional information: http://www.karger.com/AJN


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