| Autor: |
VA Varney, Amolak S Bansal, Jane Evans, A Nicholas, G Quirke, Nazira Sumar, H Parnell |
| Rok vydání: |
2015 |
| Předmět: |
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| Zdroj: |
Journal of Clinical & Cellular Immunology. |
| ISSN: |
2155-9899 |
| Popis: |
Background: Idiopathic pulmonary fibrosis (UIP/IPF) is increasing. The condition occurs both sporadically and in close blood relatives suggesting a genetic predisposition. Histology suggests that the fibrosis is linked to disordered apoptosis, but the causal agent is so far unrecognised. We previously published evidence of mannose binding lectin deficiency (MBL) in early onset disease and in those with a family history of UIP/IPF. MBL is part of the innate immune system with levels genetically determined. MBL activation is impaired if the mannose associated serine protease2 (MASP2) is severely deficient in the serum (levels 100 ng/ml) showed no differences between Healthy Controls, COPD, TB and sarcoid. UIP/IPF showed a significant increase in the frequency of severe deficiency. This was seen for early onset disease 55yrs (20% p=0.0001) and those with a family history (19% p=0.0143). The expected frequency for the Caucasian population is |
| Databáze: |
OpenAIRE |
| Externí odkaz: |
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