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      Renal Failure in Familial Lecithin-Cholesterol Acyltransferase Deficiency

      , , ,

      Nephron

      S. Karger AG

      Renal failure, Inborn error, Lipid metabolism, Ultrastructure

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          Abstract

          Familial lecithin-cholesterol acyltransferase deficiency is a hereditary disorder of lipid metabolism. Lipid material is deposited in the kidneys, the glomerular capillary basement membrane is irregularly thickened, detachment and even loss of endothelial cells are seen in the glomeruli. Proteinuria was present in 8 out of 9 cases studied, usually it has not been detected before the age of 15–20. After 15–30 years with symptomless proteinuria, terminal renal failure has developed in 6 of the patients. Possible pathogenetic mechanisms of the renal damage is discussed; a large-molecular-weight low-density lipoprotein is suggested to be an important factor.

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          Author and article information

          Journal
          NEF
          Nephron
          10.1159/issn.1660-8151
          Nephron
          S. Karger AG
          1660-8151
          2235-3186
          1977
          1977
          28 November 2008
          : 18
          : 4
          : 239-248
          Affiliations
          Medical Department A, Surgical Department B and Institute of Pathology, University Hospital, Rikshospitalet, Oslo
          Article
          180835 Nephron 1977;18:239–248
          10.1159/000180835
          404580
          © 1977 S. Karger AG, Basel

          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.

          Page count
          Pages: 10
          Categories
          Original Paper

          Cardiovascular Medicine, Nephrology

          Lipid metabolism, Renal failure, Inborn error, Ultrastructure

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