[Interstitial lung disease associated with surfactant protein B and C deficiencies].
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.
Szczawińska-Popłonyk
Aleksandra
A
Klinika Pneumonologii, Alergologii Dzieciecej i Immunologii Klinicznej III Katedry Pediatrii Uniwersytetu Medycznego im. Karola Marcinkowskiego w Poznaniu. ola@malwa.com.pl
Breborowicz
Anna
A
Langfort
Renata
R
D018528:ATP-Binding Cassette Transporters / Q000235:genetics / Q000378:metabolism*; D002648:Child; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000209:etiology* / Q000378:metabolism*; D011379:Prognosis; D037701:Pulmonary Surfactant-Associated Protein B / Q000172:deficiency* / Q000235:genetics / Q000378:metabolism; D037721:Pulmonary Surfactant-Associated Protein C / Q000172:deficiency* / Q000235:genetics / Q000378:metabolism; D012307:Risk Factors
C441524:ABCA3 protein, human; D018528:ATP-Binding Cassette Transporters; D037701:Pulmonary Surfactant-Associated Protein B; D037721:Pulmonary Surfactant-Associated Protein C