The association between abnormal chloride transport, resulting from mutations in the cystic fibrosis transmembrane regulator (CFTR) gene, and the immunologic processes involved in the development of CF lung disease is poorly understood. However, neutrophil-dominated inflammation on the respiratory epithelial surface is a common finding in CF patients and suggests a mechanism for the immunologic abnormalities described in CF. Of particular importance for the pathophysiology of CF are proteases such as neutrophil elastase (NE) which are released from neutrophils in CF airways and cause direct structural damage to respiratory tissue. In healthy individuals, the deleterious effects of excess protease activity in the respiratory system are inhibited by antiproteases such as α1-antitrypsin (α1AT) and secretory leukoprotease inhibitor (SLPI). However, in CF, antiproteases are outnumbered by proteases and this protective mechanism is rendered ineffective. Restoration of this protease/antiprotease balance through antiprotease replacement therapy is currently under clinical investigation and preliminary results are promising.

This content is only available via PDF.
Copyright / Drug Dosage / Disclaimer
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.
You do not currently have access to this content.