open access

Vol 78, No 3 (2010)
REVIEWS
Published online: 2010-05-11
Submitted: 2013-02-22
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Interstitial lung disease associated with surfactant protein B and C deficiencies

Aleksandra Szczawińska-Popłonyk, Anna Bręborowicz, Renata Langfort
Pneumonol Alergol Pol 2010;78(3):224-228.

open access

Vol 78, No 3 (2010)
REVIEWS
Published online: 2010-05-11
Submitted: 2013-02-22

Abstract

Etiology and pathogenesis of the interstitial lung disease in children result from a heterogeneous group of infectious, immunological and metabolic factors. In children an important role plays a surfactant protein B and C deficiency. SP-C deficiency is determined by it’s defective synthesis or impaired production of ABCA3 transporter, as well as with abnormalities within different metabolic pathways. In the paper clinical manifestation, radiological findings, molecular background and prognosis in interstitial lung diseases associated with SP-B and SP-C defects have been discussed.

Abstract

Etiology and pathogenesis of the interstitial lung disease in children result from a heterogeneous group of infectious, immunological and metabolic factors. In children an important role plays a surfactant protein B and C deficiency. SP-C deficiency is determined by it’s defective synthesis or impaired production of ABCA3 transporter, as well as with abnormalities within different metabolic pathways. In the paper clinical manifestation, radiological findings, molecular background and prognosis in interstitial lung diseases associated with SP-B and SP-C defects have been discussed.
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Keywords

interstitial lung disease; surfactant; children

About this article
Title

Interstitial lung disease associated with surfactant protein B and C deficiencies

Journal

Advances in Respiratory Medicine

Issue

Vol 78, No 3 (2010)

Pages

224-228

Published online

2010-05-11

Bibliographic record

Pneumonol Alergol Pol 2010;78(3):224-228.

Keywords

interstitial lung disease
surfactant
children

Authors

Aleksandra Szczawińska-Popłonyk
Anna Bręborowicz
Renata Langfort

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