Alpha-1-Antitrypsin Deficiency Associated with Hepatic Cirrhosis and IgA Nephritis

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Abstract

The association between α<sub>1</sub>-antitrypsin (A1AT) deficiency and glomerulonephritis has only sporadically been reported, and mostly based upon autopsy findings, as opposed to the more frequent linkage between A1AT deficiency and lung emphysema with or without hepatic cirrhosis. The present case report describes a 30-year-old man with A1AT deficiency, without evidence of lung disease, who developed hepatic cirrhosis in early childhood and IgA glomerulonephritis and hypertension in adult life. The IgA nephritis followed an unusual course, with a sudden deterioration of the renal function, possibly induced by uncontrolled hypertension or the possible occurrence of vasculitis. After 6 months of hemodialysis, the patient successfully underwent living-related-donor kidney transplantation.

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Author and article information

Journal

Journal ID (publisher-id): NEF

Journal ID (nlm-ta): Nephron

Journal ID (doi): 10.1159/issn.1660-8151

Title: Nephron

Publisher: S. Karger AG

ISSN (Print): 1660-8151

ISSN (Electronic): 2235-3186

Publication date Subscription-year: 1997

Publication date (Print): 1997

Publication date (Electronic): 23 December 2008

Volume: 77

Issue: 2

Pages: 235-237

Affiliations

Departments of ^aInternal Medicine and ^bPathology, Ullevål Hospital, Oslo, Norway; ^cDepartment of Pathology, Sahlgrenska Hospital, Gothenburg, Sweden

Article

Publisher ID: 190278 Other ID: Nephron 1997;77:235–237

DOI: 10.1159/000190278

PubMed ID: 9346392

SO-VID: 4ba2229b-0ad3-4f5b-b20a-867541360b4c

License:

Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

History

Date accepted : 10 January 1997

Page count

Pages: 3

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