S143 Serum mannose binding lectin deficiency is present in patients with early onset interstitial pulmonary fibrosis and those with affected relatives suggesting a genetic risk factor for defects in the innate immune system

BackgroundIdiopathic pulmonary fibrosis (IPF) is a serious progressive lung disease with likely environmental and genetic risk factors that are thought to contribute to the disease even though their exact nature is unknown. It is increasingly recognised that siblings and close blood relatives can de...

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Veröffentlicht in:Thorax 2010-12, Vol.65 (Suppl 4), p.A65-A65
Hauptverfasser: Varney, V A, Evans, J, Parnell, H, Nicholas, A, Barjardeen, B, Sumar, N
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Sprache:eng
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Zusammenfassung:BackgroundIdiopathic pulmonary fibrosis (IPF) is a serious progressive lung disease with likely environmental and genetic risk factors that are thought to contribute to the disease even though their exact nature is unknown. It is increasingly recognised that siblings and close blood relatives can develop the same condition suggesting an unknown genetic predisposition.MethodWe have examined the serum mannose binding lectin levels (MBL) in healthy controls (HC), frequently exacerbating COPD, pulmonary TB & Sarcoidosis along with IPF patients.ResultsMean serum MBL levels were not statistically different in HC, COPD or TB using an unpaired t test. Cases with sarcoid had higher levels. Those with IPF onset at 50 years without a family history. Abstract S143 Table 1 shows means, SEM and p values, and the per cent of each patient group with normal >650, moderate 100–600 or severe deficiency levels 50 yearsp Value v's IPF
ISSN:0040-6376
1468-3296
DOI:10.1136/thx.2010.150946.44