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Vol. 74, No. 3, 2007
Issue release date: May 2007
Section title: Thematic Review Series 2007
Respiration 2007;74:264–275
(DOI:10.1159/000101784)

Genetic Causes of Bronchiectasis: Primary Immune Deficiencies and the Lung

Notarangelo L.D. · Plebani A. · Mazzolari E. · Soresina A. · Bondioni M.P.
aDivision of Immunology, Children’s Hospital, Harvard Medical School, Boston, Mass., USA; bDepartment of Pediatrics and cDivision of Pediatric Radiology, University of Brescia, Ospedale dei Bambini, Brescia, Italy

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Article / Publication Details

First-Page Preview
Abstract of Thematic Review Series 2007

Received: 2/8/2007
Accepted: 2/28/2007
Published online: 3/9/2007

Number of Print Pages: 12
Number of Figures: 3
Number of Tables: 2

ISSN: 0025-7931 (Print)
eISSN: 1423-0356 (Online)

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

Abstract

Primary immune deficiencies (PID) comprise a heterogeneous group of genetically determined disorders that affect development and/or function of innate or adaptive immunity. Consequently, patients with PID suffer from recurrent and/or severe infections that frequently involve the lung. While the nature of the immune defect often dictates the type of pathogens that may cause lung infection, there is substantial overlap of radiological findings, so that appropriate laboratory tests are mandatory to define the nature of the immune defect and to prompt appropriate treatment. At the same time, the recent identification of a large number of PID-causing genes now allows early, even presymptomatic diagnosis, thus representing an essential tool for prevention of lung damage. This review article describes the most common forms of PID, their cellular and molecular bases, and the associated lung abnormalities, and reports on available treatment.


  

Author Contacts

Prof. Luigi D. Notarangelo, MD, Division of Immunology
Children’s Hospital, Harvard Medical School, Karp Building, 9th floor
Room 09210, 1 Blackfan Circle, Boston, MA 02115 (USA)
Tel. +1 617 919 2276, Fax +1 617 730 0709
E-Mail luigi.notarangelo@childrens.harvard.edu

  

Article Information

Previous articles in this series: 1. Contopoulos-Ioannidis DG, Kouri IN, Ioannidis JPA: Genetic predisposition to asthma and atopy. Respiration 2007;74:8–12. 2. Sztrymf B, Yaïci A, Girerd B, Humbert M: Genes and pulmonary arterial hypertension. Respiration 2007;74:123–132.

Number of Print Pages : 12
Number of Figures : 3, Number of Tables : 2, Number of References : 104

  

Publication Details

Respiration (International Journal of Thoracic Medicine)

Vol. 74, No. 3, Year 2007 (Cover Date: May 2007)

Journal Editor: Bolliger, C.T. (Cape Town)
ISSN: 0025–7931 (print), 1423–0356 (Online)

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


Article / Publication Details

First-Page Preview
Abstract of Thematic Review Series 2007

Received: 2/8/2007
Accepted: 2/28/2007
Published online: 3/9/2007

Number of Print Pages: 12
Number of Figures: 3
Number of Tables: 2

ISSN: 0025-7931 (Print)
eISSN: 1423-0356 (Online)

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


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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 goverment 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.
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