Comparison of radiographic and clinical characteristics of low-risk and high-risk cystic fibrosis genotypes
Background Patients with cystic fibrosis (CF) exhibit a wide range of disease severity, and can be broadly stratified into high‐risk and low‐risk groups based on cystic fibrosis transmembrane conductance regulator (CFTR) mutation class. Patients with a low‐risk genotype are often diagnosed as adults...
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Veröffentlicht in: | International forum of allergy & rhinology 2014-11, Vol.4 (11), p.915-920 |
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creator | Ferril, Geoffrey R. Nick, Jerry A. Getz, Anne E. Barham, Henry P. Saavedra, Milene T. Taylor-Cousar, Jennifer L. Nichols, David P. Curran-Everett, Douglas Kingdom, Todd T. Ramakrishnan, Vijay R. |
description | Background
Patients with cystic fibrosis (CF) exhibit a wide range of disease severity, and can be broadly stratified into high‐risk and low‐risk groups based on cystic fibrosis transmembrane conductance regulator (CFTR) mutation class. Patients with a low‐risk genotype are often diagnosed as adults, with milder disease and lower sweat chloride values. The aim of the current study was to better understand radiographic and clinical characteristics of sinus disease in adult CF patients within this risk category.
Methods
Adult CF patients were retrospectively compared to a control group of patients with chronic rhinosinusitis. CF diagnostic testing and pulmonary characteristics were compared between high‐risk and low‐risk CF groups, and sinus CT findings were compared among all 3 groups.
Results
When comparing CF cohorts (n = 25 and 30, respectively), earlier age at diagnosis (p < 0.001), higher sweat chloride values (p < 0.001), lower forced expiratory volume in 1 second (FEV1) values (p < 0.001), and a higher prevalence of pulmonary infection with Pseudomonas aeruginosa (p = 0.001) were found in the high‐risk genotype group. A significantly increased incidence of sinus hypoplasia/aplasia and bony sclerosis was seen when comparing both CF groups to the control cohort (n = 30), as well as when comparing the high‐risk and low‐risk CF genotype cohorts.
Conclusion
The current study describes clinicopathologic findings of sinus disease in adult CF patients in the context of genotype severity. Our data demonstrate that while patients within a low‐risk genotype cohort have generally milder lung disease, they retain classic radiographic findings of CF sinus disease that can help raise the index of suspicion for undiagnosed CF. |
doi_str_mv | 10.1002/alr.21412 |
format | Article |
fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_1618828595</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>1618828595</sourcerecordid><originalsourceid>FETCH-LOGICAL-c4612-bd18bfb38da642b252c2261bf1a0a6cb83bbbf930bdd8dcd8974fef97a973c1c3</originalsourceid><addsrcrecordid>eNp10U9P2zAUAHBrGhoIOOwLTJG4bIe0sR07zhGVDQYRk9Bg0i6W_7amaZzZrUq__RwCPSDhi_2k33t6fg-Az7CYwKJAU9GGCYIlRB_AESpKlNOalR_374oegtMYH4t0CCQEVp_AISIIlYTQI7Cc-VUvgou-y7zNgtDOz4PoF05lotOZal3nlGgztRBBqLVJdO1UHHDrt3kKl89w4eaLMVK7QWTWyeCji9ncdH696008AQdWtNGcvtzH4P7H99-zq7z5dflzdt7kqqQQ5VJDJq3ETAtaIpl6VQhRKC0UhaBKMiyltDUupNZMK83qqrTG1pWoK6ygwsfg61i3D_7fxsQ1X7moTNuKzvhN5JBCxhAjNUn07A199JvQpe4GVUFGGR7Ut1Gp9KMYjOV9cCsRdhwWfFgCT0vgz0tI9stLxY1cGb2XryNPYDqCrWvN7v1K_Ly5ey2Zjxlp9OZpnyHCktMKV4T_ub3kuLm4u35o_vIb_B_gUqFe</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>1617186835</pqid></control><display><type>article</type><title>Comparison of radiographic and clinical characteristics of low-risk and high-risk cystic fibrosis genotypes</title><source>MEDLINE</source><source>Wiley Online Library Journals Frontfile Complete</source><creator>Ferril, Geoffrey R. ; Nick, Jerry A. ; Getz, Anne E. ; Barham, Henry P. ; Saavedra, Milene T. ; Taylor-Cousar, Jennifer L. ; Nichols, David P. ; Curran-Everett, Douglas ; Kingdom, Todd T. ; Ramakrishnan, Vijay R.</creator><creatorcontrib>Ferril, Geoffrey R. ; Nick, Jerry A. ; Getz, Anne E. ; Barham, Henry P. ; Saavedra, Milene T. ; Taylor-Cousar, Jennifer L. ; Nichols, David P. ; Curran-Everett, Douglas ; Kingdom, Todd T. ; Ramakrishnan, Vijay R.</creatorcontrib><description>Background
Patients with cystic fibrosis (CF) exhibit a wide range of disease severity, and can be broadly stratified into high‐risk and low‐risk groups based on cystic fibrosis transmembrane conductance regulator (CFTR) mutation class. Patients with a low‐risk genotype are often diagnosed as adults, with milder disease and lower sweat chloride values. The aim of the current study was to better understand radiographic and clinical characteristics of sinus disease in adult CF patients within this risk category.
Methods
Adult CF patients were retrospectively compared to a control group of patients with chronic rhinosinusitis. CF diagnostic testing and pulmonary characteristics were compared between high‐risk and low‐risk CF groups, and sinus CT findings were compared among all 3 groups.
Results
When comparing CF cohorts (n = 25 and 30, respectively), earlier age at diagnosis (p < 0.001), higher sweat chloride values (p < 0.001), lower forced expiratory volume in 1 second (FEV1) values (p < 0.001), and a higher prevalence of pulmonary infection with Pseudomonas aeruginosa (p = 0.001) were found in the high‐risk genotype group. A significantly increased incidence of sinus hypoplasia/aplasia and bony sclerosis was seen when comparing both CF groups to the control cohort (n = 30), as well as when comparing the high‐risk and low‐risk CF genotype cohorts.
Conclusion
The current study describes clinicopathologic findings of sinus disease in adult CF patients in the context of genotype severity. Our data demonstrate that while patients within a low‐risk genotype cohort have generally milder lung disease, they retain classic radiographic findings of CF sinus disease that can help raise the index of suspicion for undiagnosed CF.</description><identifier>ISSN: 2042-6976</identifier><identifier>EISSN: 2042-6984</identifier><identifier>DOI: 10.1002/alr.21412</identifier><identifier>PMID: 25224556</identifier><language>eng</language><publisher>United States: Blackwell Publishing Ltd</publisher><subject>Adult ; Case-Control Studies ; CFTR ; Chlorides - metabolism ; Chronic Disease ; cystic fibrosis ; Cystic Fibrosis - diagnostic imaging ; Cystic Fibrosis - genetics ; Cystic Fibrosis Transmembrane Conductance Regulator - genetics ; Female ; Forced Expiratory Volume - physiology ; Genotype ; Humans ; Male ; Middle Aged ; Mutation - genetics ; Paranasal Sinuses - abnormalities ; Paranasal Sinuses - diagnostic imaging ; Pseudomonas aeruginosa ; Pseudomonas Infections - complications ; Retrospective Studies ; Rhinitis - diagnostic imaging ; Risk Factors ; sinus CT ; sinus radiography ; sinusitis ; Sinusitis - diagnostic imaging ; Sweat - chemistry ; Tomography, X-Ray Computed</subject><ispartof>International forum of allergy & rhinology, 2014-11, Vol.4 (11), p.915-920</ispartof><rights>2014 ARS‐AAOA, LLC</rights><rights>2014 ARS-AAOA, LLC.</rights><rights>2014 ARS-AAOA, LLC</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c4612-bd18bfb38da642b252c2261bf1a0a6cb83bbbf930bdd8dcd8974fef97a973c1c3</citedby><cites>FETCH-LOGICAL-c4612-bd18bfb38da642b252c2261bf1a0a6cb83bbbf930bdd8dcd8974fef97a973c1c3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1002%2Falr.21412$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1002%2Falr.21412$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,780,784,1416,27915,27916,45565,45566</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/25224556$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Ferril, Geoffrey R.</creatorcontrib><creatorcontrib>Nick, Jerry A.</creatorcontrib><creatorcontrib>Getz, Anne E.</creatorcontrib><creatorcontrib>Barham, Henry P.</creatorcontrib><creatorcontrib>Saavedra, Milene T.</creatorcontrib><creatorcontrib>Taylor-Cousar, Jennifer L.</creatorcontrib><creatorcontrib>Nichols, David P.</creatorcontrib><creatorcontrib>Curran-Everett, Douglas</creatorcontrib><creatorcontrib>Kingdom, Todd T.</creatorcontrib><creatorcontrib>Ramakrishnan, Vijay R.</creatorcontrib><title>Comparison of radiographic and clinical characteristics of low-risk and high-risk cystic fibrosis genotypes</title><title>International forum of allergy & rhinology</title><addtitle>Int Forum Allergy Rhinol</addtitle><description>Background
Patients with cystic fibrosis (CF) exhibit a wide range of disease severity, and can be broadly stratified into high‐risk and low‐risk groups based on cystic fibrosis transmembrane conductance regulator (CFTR) mutation class. Patients with a low‐risk genotype are often diagnosed as adults, with milder disease and lower sweat chloride values. The aim of the current study was to better understand radiographic and clinical characteristics of sinus disease in adult CF patients within this risk category.
Methods
Adult CF patients were retrospectively compared to a control group of patients with chronic rhinosinusitis. CF diagnostic testing and pulmonary characteristics were compared between high‐risk and low‐risk CF groups, and sinus CT findings were compared among all 3 groups.
Results
When comparing CF cohorts (n = 25 and 30, respectively), earlier age at diagnosis (p < 0.001), higher sweat chloride values (p < 0.001), lower forced expiratory volume in 1 second (FEV1) values (p < 0.001), and a higher prevalence of pulmonary infection with Pseudomonas aeruginosa (p = 0.001) were found in the high‐risk genotype group. A significantly increased incidence of sinus hypoplasia/aplasia and bony sclerosis was seen when comparing both CF groups to the control cohort (n = 30), as well as when comparing the high‐risk and low‐risk CF genotype cohorts.
Conclusion
The current study describes clinicopathologic findings of sinus disease in adult CF patients in the context of genotype severity. Our data demonstrate that while patients within a low‐risk genotype cohort have generally milder lung disease, they retain classic radiographic findings of CF sinus disease that can help raise the index of suspicion for undiagnosed CF.</description><subject>Adult</subject><subject>Case-Control Studies</subject><subject>CFTR</subject><subject>Chlorides - metabolism</subject><subject>Chronic Disease</subject><subject>cystic fibrosis</subject><subject>Cystic Fibrosis - diagnostic imaging</subject><subject>Cystic Fibrosis - genetics</subject><subject>Cystic Fibrosis Transmembrane Conductance Regulator - genetics</subject><subject>Female</subject><subject>Forced Expiratory Volume - physiology</subject><subject>Genotype</subject><subject>Humans</subject><subject>Male</subject><subject>Middle Aged</subject><subject>Mutation - genetics</subject><subject>Paranasal Sinuses - abnormalities</subject><subject>Paranasal Sinuses - diagnostic imaging</subject><subject>Pseudomonas aeruginosa</subject><subject>Pseudomonas Infections - complications</subject><subject>Retrospective Studies</subject><subject>Rhinitis - diagnostic imaging</subject><subject>Risk Factors</subject><subject>sinus CT</subject><subject>sinus radiography</subject><subject>sinusitis</subject><subject>Sinusitis - diagnostic imaging</subject><subject>Sweat - chemistry</subject><subject>Tomography, X-Ray Computed</subject><issn>2042-6976</issn><issn>2042-6984</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2014</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp10U9P2zAUAHBrGhoIOOwLTJG4bIe0sR07zhGVDQYRk9Bg0i6W_7amaZzZrUq__RwCPSDhi_2k33t6fg-Az7CYwKJAU9GGCYIlRB_AESpKlNOalR_374oegtMYH4t0CCQEVp_AISIIlYTQI7Cc-VUvgou-y7zNgtDOz4PoF05lotOZal3nlGgztRBBqLVJdO1UHHDrt3kKl89w4eaLMVK7QWTWyeCji9ncdH696008AQdWtNGcvtzH4P7H99-zq7z5dflzdt7kqqQQ5VJDJq3ETAtaIpl6VQhRKC0UhaBKMiyltDUupNZMK83qqrTG1pWoK6ygwsfg61i3D_7fxsQ1X7moTNuKzvhN5JBCxhAjNUn07A199JvQpe4GVUFGGR7Ut1Gp9KMYjOV9cCsRdhwWfFgCT0vgz0tI9stLxY1cGb2XryNPYDqCrWvN7v1K_Ly5ey2Zjxlp9OZpnyHCktMKV4T_ub3kuLm4u35o_vIb_B_gUqFe</recordid><startdate>201411</startdate><enddate>201411</enddate><creator>Ferril, Geoffrey R.</creator><creator>Nick, Jerry A.</creator><creator>Getz, Anne E.</creator><creator>Barham, Henry P.</creator><creator>Saavedra, Milene T.</creator><creator>Taylor-Cousar, Jennifer L.</creator><creator>Nichols, David P.</creator><creator>Curran-Everett, Douglas</creator><creator>Kingdom, Todd T.</creator><creator>Ramakrishnan, Vijay R.</creator><general>Blackwell Publishing Ltd</general><general>Wiley Subscription Services, Inc</general><scope>BSCLL</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>K9.</scope><scope>7X8</scope></search><sort><creationdate>201411</creationdate><title>Comparison of radiographic and clinical characteristics of low-risk and high-risk cystic fibrosis genotypes</title><author>Ferril, Geoffrey R. ; Nick, Jerry A. ; Getz, Anne E. ; Barham, Henry P. ; Saavedra, Milene T. ; Taylor-Cousar, Jennifer L. ; Nichols, David P. ; Curran-Everett, Douglas ; Kingdom, Todd T. ; Ramakrishnan, Vijay R.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c4612-bd18bfb38da642b252c2261bf1a0a6cb83bbbf930bdd8dcd8974fef97a973c1c3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2014</creationdate><topic>Adult</topic><topic>Case-Control Studies</topic><topic>CFTR</topic><topic>Chlorides - metabolism</topic><topic>Chronic Disease</topic><topic>cystic fibrosis</topic><topic>Cystic Fibrosis - diagnostic imaging</topic><topic>Cystic Fibrosis - genetics</topic><topic>Cystic Fibrosis Transmembrane Conductance Regulator - genetics</topic><topic>Female</topic><topic>Forced Expiratory Volume - physiology</topic><topic>Genotype</topic><topic>Humans</topic><topic>Male</topic><topic>Middle Aged</topic><topic>Mutation - genetics</topic><topic>Paranasal Sinuses - abnormalities</topic><topic>Paranasal Sinuses - diagnostic imaging</topic><topic>Pseudomonas aeruginosa</topic><topic>Pseudomonas Infections - complications</topic><topic>Retrospective Studies</topic><topic>Rhinitis - diagnostic imaging</topic><topic>Risk Factors</topic><topic>sinus CT</topic><topic>sinus radiography</topic><topic>sinusitis</topic><topic>Sinusitis - diagnostic imaging</topic><topic>Sweat - chemistry</topic><topic>Tomography, X-Ray Computed</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Ferril, Geoffrey R.</creatorcontrib><creatorcontrib>Nick, Jerry A.</creatorcontrib><creatorcontrib>Getz, Anne E.</creatorcontrib><creatorcontrib>Barham, Henry P.</creatorcontrib><creatorcontrib>Saavedra, Milene T.</creatorcontrib><creatorcontrib>Taylor-Cousar, Jennifer L.</creatorcontrib><creatorcontrib>Nichols, David P.</creatorcontrib><creatorcontrib>Curran-Everett, Douglas</creatorcontrib><creatorcontrib>Kingdom, Todd T.</creatorcontrib><creatorcontrib>Ramakrishnan, Vijay R.</creatorcontrib><collection>Istex</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>MEDLINE - Academic</collection><jtitle>International forum of allergy & rhinology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Ferril, Geoffrey R.</au><au>Nick, Jerry A.</au><au>Getz, Anne E.</au><au>Barham, Henry P.</au><au>Saavedra, Milene T.</au><au>Taylor-Cousar, Jennifer L.</au><au>Nichols, David P.</au><au>Curran-Everett, Douglas</au><au>Kingdom, Todd T.</au><au>Ramakrishnan, Vijay R.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Comparison of radiographic and clinical characteristics of low-risk and high-risk cystic fibrosis genotypes</atitle><jtitle>International forum of allergy & rhinology</jtitle><addtitle>Int Forum Allergy Rhinol</addtitle><date>2014-11</date><risdate>2014</risdate><volume>4</volume><issue>11</issue><spage>915</spage><epage>920</epage><pages>915-920</pages><issn>2042-6976</issn><eissn>2042-6984</eissn><abstract>Background
Patients with cystic fibrosis (CF) exhibit a wide range of disease severity, and can be broadly stratified into high‐risk and low‐risk groups based on cystic fibrosis transmembrane conductance regulator (CFTR) mutation class. Patients with a low‐risk genotype are often diagnosed as adults, with milder disease and lower sweat chloride values. The aim of the current study was to better understand radiographic and clinical characteristics of sinus disease in adult CF patients within this risk category.
Methods
Adult CF patients were retrospectively compared to a control group of patients with chronic rhinosinusitis. CF diagnostic testing and pulmonary characteristics were compared between high‐risk and low‐risk CF groups, and sinus CT findings were compared among all 3 groups.
Results
When comparing CF cohorts (n = 25 and 30, respectively), earlier age at diagnosis (p < 0.001), higher sweat chloride values (p < 0.001), lower forced expiratory volume in 1 second (FEV1) values (p < 0.001), and a higher prevalence of pulmonary infection with Pseudomonas aeruginosa (p = 0.001) were found in the high‐risk genotype group. A significantly increased incidence of sinus hypoplasia/aplasia and bony sclerosis was seen when comparing both CF groups to the control cohort (n = 30), as well as when comparing the high‐risk and low‐risk CF genotype cohorts.
Conclusion
The current study describes clinicopathologic findings of sinus disease in adult CF patients in the context of genotype severity. Our data demonstrate that while patients within a low‐risk genotype cohort have generally milder lung disease, they retain classic radiographic findings of CF sinus disease that can help raise the index of suspicion for undiagnosed CF.</abstract><cop>United States</cop><pub>Blackwell Publishing Ltd</pub><pmid>25224556</pmid><doi>10.1002/alr.21412</doi><tpages>6</tpages></addata></record> |
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subjects | Adult Case-Control Studies CFTR Chlorides - metabolism Chronic Disease cystic fibrosis Cystic Fibrosis - diagnostic imaging Cystic Fibrosis - genetics Cystic Fibrosis Transmembrane Conductance Regulator - genetics Female Forced Expiratory Volume - physiology Genotype Humans Male Middle Aged Mutation - genetics Paranasal Sinuses - abnormalities Paranasal Sinuses - diagnostic imaging Pseudomonas aeruginosa Pseudomonas Infections - complications Retrospective Studies Rhinitis - diagnostic imaging Risk Factors sinus CT sinus radiography sinusitis Sinusitis - diagnostic imaging Sweat - chemistry Tomography, X-Ray Computed |
title | Comparison of radiographic and clinical characteristics of low-risk and high-risk cystic fibrosis genotypes |
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