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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| description | 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 |
| doi_str_mv | 10.1136/thx.2010.150946.44 |
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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 old and those with affected blood relatives (FH) had significantly reduced levels compared with IPF onset >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 <100.Abstract S143 Table 1ParameterMean MBL±SEM pg/mlp Value v's HCp Value v's IPF>50 yearsp Value v's IPF <50 yearsp Value v's IPF & FH% >650 MBL% 100–600 MBL% <100 MBLHC n=1111315±136–0.480.0350.01533215COPD n=331492±2570.580.900.050.022552718TB n=471945±2680.980.240.0040.004721117Sarcoid n=382040±2750.020.120.0020.0012681616IPF >50 n=601475±2030.48–0.0120.007583210IPF <50 n=19632±2130.030.012–0.59264232IPF & FH n=18688±2790.010.0070.59–273340DiscussionSerum MBL forms part of the complement activation and innate immune system and protects the lung from infection by organisms that bind mannose sugar (eg, strep, staph, yeasts, P.Jiveci). MBL deficiency gives an opsonisation defect with reduced phagocytosis by alveolar macrophages. Blood levels are genetically determined, with UK population data showing:12% severe deficiency <100 pg/ml,34% moderate deficiency 100–600 pg/ml,54% normal < 650 pg/ml.Serum levels relate to polymorphisms of the MBL2 genes. χ2 analysis of frequency distribution showed no differences for HC, COPD & IPF>50 years. TB&Sarcoid had higher frequencies of normal MBL levels compared with HC (p=0.001 and 0.024 respectively). IPF <50 & IPF& FH showed higher frequencies of moderate and severe deficiency compared with HC and all other groups (p=0.001 and 0.001 respectively).The literature shows MBL to consistently have interesting and important central roles in lung defenses via effects on complement, apoptosis and cytokines. Its observed deficiency in young IPF and those with a FH could be a genetic risk factor of relevance, explaining its early occurrence in deficiency and later occurrence in ‘sufficiency’, where it gives protection from insult or injury to the lung.]]></description><identifier>ISSN: 0040-6376</identifier><identifier>EISSN: 1468-3296</identifier><identifier>DOI: 10.1136/thx.2010.150946.44</identifier><identifier>CODEN: THORA7</identifier><language>eng</language><publisher>London: BMJ Publishing Group Ltd and British Thoracic Society</publisher><ispartof>Thorax, 2010-12, Vol.65 (Suppl 4), p.A65-A65</ispartof><rights>2010, Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.</rights><rights>Copyright: 2010 (c) 2010, Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://thorax.bmj.com/content/65/Suppl_4/A65.1.full.pdf$$EPDF$$P50$$Gbmj$$H</linktopdf><linktohtml>$$Uhttps://thorax.bmj.com/content/65/Suppl_4/A65.1.full$$EHTML$$P50$$Gbmj$$H</linktohtml><link.rule.ids>114,115,314,780,784,3196,23571,27924,27925,77600,77631</link.rule.ids></links><search><creatorcontrib>Varney, V A</creatorcontrib><creatorcontrib>Evans, J</creatorcontrib><creatorcontrib>Parnell, H</creatorcontrib><creatorcontrib>Nicholas, A</creatorcontrib><creatorcontrib>Barjardeen, B</creatorcontrib><creatorcontrib>Sumar, N</creatorcontrib><title>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</title><title>Thorax</title><addtitle>Thorax</addtitle><description><![CDATA[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 old and those with affected blood relatives (FH) had significantly reduced levels compared with IPF onset >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 <100.Abstract S143 Table 1ParameterMean MBL±SEM pg/mlp Value v's HCp Value v's IPF>50 yearsp Value v's IPF <50 yearsp Value v's IPF & FH% >650 MBL% 100–600 MBL% <100 MBLHC n=1111315±136–0.480.0350.01533215COPD n=331492±2570.580.900.050.022552718TB n=471945±2680.980.240.0040.004721117Sarcoid n=382040±2750.020.120.0020.0012681616IPF >50 n=601475±2030.48–0.0120.007583210IPF <50 n=19632±2130.030.012–0.59264232IPF & FH n=18688±2790.010.0070.59–273340DiscussionSerum MBL forms part of the complement activation and innate immune system and protects the lung from infection by organisms that bind mannose sugar (eg, strep, staph, yeasts, P.Jiveci). MBL deficiency gives an opsonisation defect with reduced phagocytosis by alveolar macrophages. Blood levels are genetically determined, with UK population data showing:12% severe deficiency <100 pg/ml,34% moderate deficiency 100–600 pg/ml,54% normal < 650 pg/ml.Serum levels relate to polymorphisms of the MBL2 genes. χ2 analysis of frequency distribution showed no differences for HC, COPD & IPF>50 years. TB&Sarcoid had higher frequencies of normal MBL levels compared with HC (p=0.001 and 0.024 respectively). IPF <50 & IPF& FH showed higher frequencies of moderate and severe deficiency compared with HC and all other groups (p=0.001 and 0.001 respectively).The literature shows MBL to consistently have interesting and important central roles in lung defenses via effects on complement, apoptosis and cytokines. Its observed deficiency in young IPF and those with a FH could be a genetic risk factor of relevance, explaining its early occurrence in deficiency and later occurrence in ‘sufficiency’, where it gives protection from insult or injury to the lung.]]></description><issn>0040-6376</issn><issn>1468-3296</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2010</creationdate><recordtype>article</recordtype><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><recordid>eNqNUU1v1DAQjRCVWAp_gNNInLPYiddJjmjFl1SBRGmRuFh2Mt71NnGC7UD3xoUfSn8JExbBtQfryZ55b57nZdkzztacl_JF2t-uC7bcNqwRci3Eg2zFhazzsmjkw2zFmGC5LCv5KHsc44ExVnNerbJfl1yUdz9-XmKYBxi092NEMM53zu-gxzY5Dx1a1zr07RFchClgRJ-ACpNO9JwifHdpD6hDf4TRR1yKCUNMLjndwzT3w-h1OIJ1JoyRRLTvIO2XWX-o2loahR0E7EnzG0aI826HpEA2NOzQY3ItBBdvwOo2jQEsHXJGvLh4SXsk8DoRDMPsEeIxJhyeZGdW9xGf_sXz7Or1q0_bt_nFhzfvti8vcsMbLvKmYdiVLRcarZUFiqIVWMuiM3yDVtelNRJbQ1DXUlad5bqoGoNoNqZAWuJ59vykO4Xx60zO1WGcg6eRilc1rzkTUlJXcepqaQ8xoFVTcAOtRnGmligVRamWKNUpSiUW6fxEcvSh238MHW6UrMpqo95fbxUvvnwUjfisrv_3m-FwH_3fFTC3ug</recordid><startdate>201012</startdate><enddate>201012</enddate><creator>Varney, V A</creator><creator>Evans, J</creator><creator>Parnell, H</creator><creator>Nicholas, A</creator><creator>Barjardeen, B</creator><creator>Sumar, N</creator><general>BMJ Publishing Group Ltd and British Thoracic Society</general><general>BMJ Publishing Group LTD</general><scope>BSCLL</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>BENPR</scope><scope>BTHHO</scope><scope>CCPQU</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>K9.</scope><scope>M0S</scope><scope>M1P</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope></search><sort><creationdate>201012</creationdate><title>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</title><author>Varney, V A ; Evans, J ; Parnell, H ; Nicholas, A ; Barjardeen, B ; Sumar, N</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-b1914-990ed3c14aeff62e42c4e862db15efa83fb6ecb3fb88667df1a279beeb5b2e143</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2010</creationdate><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Varney, V A</creatorcontrib><creatorcontrib>Evans, J</creatorcontrib><creatorcontrib>Parnell, H</creatorcontrib><creatorcontrib>Nicholas, A</creatorcontrib><creatorcontrib>Barjardeen, B</creatorcontrib><creatorcontrib>Sumar, N</creatorcontrib><collection>Istex</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>ProQuest Central</collection><collection>BMJ Journals</collection><collection>ProQuest One Community College</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><jtitle>Thorax</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Varney, V A</au><au>Evans, J</au><au>Parnell, H</au><au>Nicholas, A</au><au>Barjardeen, B</au><au>Sumar, N</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>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</atitle><jtitle>Thorax</jtitle><addtitle>Thorax</addtitle><date>2010-12</date><risdate>2010</risdate><volume>65</volume><issue>Suppl 4</issue><spage>A65</spage><epage>A65</epage><pages>A65-A65</pages><issn>0040-6376</issn><eissn>1468-3296</eissn><coden>THORA7</coden><abstract><![CDATA[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 old and those with affected blood relatives (FH) had significantly reduced levels compared with IPF onset >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 <100.Abstract S143 Table 1ParameterMean MBL±SEM pg/mlp Value v's HCp Value v's IPF>50 yearsp Value v's IPF <50 yearsp Value v's IPF & FH% >650 MBL% 100–600 MBL% <100 MBLHC n=1111315±136–0.480.0350.01533215COPD n=331492±2570.580.900.050.022552718TB n=471945±2680.980.240.0040.004721117Sarcoid n=382040±2750.020.120.0020.0012681616IPF >50 n=601475±2030.48–0.0120.007583210IPF <50 n=19632±2130.030.012–0.59264232IPF & FH n=18688±2790.010.0070.59–273340DiscussionSerum MBL forms part of the complement activation and innate immune system and protects the lung from infection by organisms that bind mannose sugar (eg, strep, staph, yeasts, P.Jiveci). MBL deficiency gives an opsonisation defect with reduced phagocytosis by alveolar macrophages. Blood levels are genetically determined, with UK population data showing:12% severe deficiency <100 pg/ml,34% moderate deficiency 100–600 pg/ml,54% normal < 650 pg/ml.Serum levels relate to polymorphisms of the MBL2 genes. χ2 analysis of frequency distribution showed no differences for HC, COPD & IPF>50 years. TB&Sarcoid had higher frequencies of normal MBL levels compared with HC (p=0.001 and 0.024 respectively). IPF <50 & IPF& FH showed higher frequencies of moderate and severe deficiency compared with HC and all other groups (p=0.001 and 0.001 respectively).The literature shows MBL to consistently have interesting and important central roles in lung defenses via effects on complement, apoptosis and cytokines. Its observed deficiency in young IPF and those with a FH could be a genetic risk factor of relevance, explaining its early occurrence in deficiency and later occurrence in ‘sufficiency’, where it gives protection from insult or injury to the lung.]]></abstract><cop>London</cop><pub>BMJ Publishing Group Ltd and British Thoracic Society</pub><doi>10.1136/thx.2010.150946.44</doi><oa>free_for_read</oa></addata></record> |
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| title | 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 |
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