open access
Vol 78, No 3 (2010)
Review paper
Submitted: 2013-02-22
Published online: 2010-05-11
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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
Submitted: 2013-02-22
Published online: 2010-05-11
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.
Get Citation
Keywords
interstitial lung disease; surfactant; children
About this articleTitle
Interstitial lung disease associated with surfactant protein B and C deficiencies
Journal
Advances in Respiratory Medicine
Issue
Article type
Review paper
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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