{"id":1851,"date":"2023-05-18T01:48:10","date_gmt":"2023-05-18T01:48:10","guid":{"rendered":"https:\/\/blogs.bmj.com\/jmg\/?p=1851"},"modified":"2026-02-23T00:13:04","modified_gmt":"2026-02-23T00:13:04","slug":"pulmonary-function-and-structure-abnormalities-in-children-and-young-adults-with-osteogenesis-imperfecta-point-to-intrinsic-and-extrinsic-lung-abnormalities","status":"publish","type":"post","link":"https:\/\/blogs.bmj.com\/jmg\/2023\/05\/18\/pulmonary-function-and-structure-abnormalities-in-children-and-young-adults-with-osteogenesis-imperfecta-point-to-intrinsic-and-extrinsic-lung-abnormalities\/","title":{"rendered":"Pulmonary function and structure abnormalities in children and young adults with osteogenesis imperfecta point to intrinsic and extrinsic lung abnormalities (Contributed by Dr. Joan Marini)"},"content":{"rendered":"<p>Lung disease is the major cause of illness and death in individuals with osteogenesis imperfecta (OI), a collagen-related bone disorder. Previously, scoliosis and chest wall deformities were considered responsible for abnormal lung function in OI. We studied children and adults with several types of OI using lung function tests, X-Rays and lung CTs. Most patients had restrictive lung disease and decreased gas exchange, independent of scoliosis. Analysis of chest scans and breathing tests showed thickening of the walls of small airways and lung tissue abnormalities.\u00a0 We conclude that OI also causes intrinsic lung disease that negatively impacts Quality of Life (QOL) in OI. Further study of intrinsic lung abnormalities may lead to improved treatment options and improve QOL. (<a href=\"https:\/\/jmg.bmj.com\/content\/early\/2023\/05\/16\/jmg-2022-109009\">https:\/\/jmg.bmj.com\/content\/early\/2023\/05\/16\/jmg-2022-109009<\/a> )<!--TrendMD v2.4.8--><\/p>\n","protected":false},"excerpt":{"rendered":"<p>Lung disease is the major cause of illness and death in individuals with osteogenesis imperfecta (OI), a collagen-related bone disorder. Previously, scoliosis and chest wall deformities were considered responsible for abnormal lung function in OI. We studied children and adults with several types of OI using lung function tests, X-Rays and lung CTs. Most patients [&#8230;]<\/p>\n<p><a class=\"btn btn-secondary understrap-read-more-link\" href=\"https:\/\/blogs.bmj.com\/jmg\/2023\/05\/18\/pulmonary-function-and-structure-abnormalities-in-children-and-young-adults-with-osteogenesis-imperfecta-point-to-intrinsic-and-extrinsic-lung-abnormalities\/\">Read More&#8230;<\/a><\/p>\n","protected":false},"author":123,"featured_media":0,"comment_status":"open","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[1],"tags":[],"class_list":["post-1851","post","type-post","status-publish","format-standard","hentry","category-uncategorized"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.6 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Pulmonary function and structure abnormalities in children and young adults with osteogenesis imperfecta point to intrinsic and extrinsic lung abnormalities (Contributed by Dr. Joan Marini) - JMG Contact blog<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/blogs.bmj.com\/jmg\/2023\/05\/18\/pulmonary-function-and-structure-abnormalities-in-children-and-young-adults-with-osteogenesis-imperfecta-point-to-intrinsic-and-extrinsic-lung-abnormalities\/\" \/>\n<meta property=\"og:locale\" content=\"en_US\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Pulmonary function and structure abnormalities in children and young adults with osteogenesis imperfecta point to intrinsic and extrinsic lung abnormalities (Contributed by Dr. Joan Marini) - JMG Contact blog\" \/>\n<meta property=\"og:description\" content=\"Lung disease is the major cause of illness and death in individuals with osteogenesis imperfecta (OI), a collagen-related bone disorder. Previously, scoliosis and chest wall deformities were considered responsible for abnormal lung function in OI. We studied children and adults with several types of OI using lung function tests, X-Rays and lung CTs. Most patients [...]Read More...\" \/>\n<meta property=\"og:url\" content=\"https:\/\/blogs.bmj.com\/jmg\/2023\/05\/18\/pulmonary-function-and-structure-abnormalities-in-children-and-young-adults-with-osteogenesis-imperfecta-point-to-intrinsic-and-extrinsic-lung-abnormalities\/\" \/>\n<meta property=\"og:site_name\" content=\"JMG Contact blog\" \/>\n<meta property=\"article:published_time\" content=\"2023-05-18T01:48:10+00:00\" \/>\n<meta property=\"article:modified_time\" content=\"2026-02-23T00:13:04+00:00\" \/>\n<meta name=\"author\" content=\"hqqu\" \/>\n<meta name=\"twitter:card\" content=\"summary_large_image\" \/>\n<meta name=\"twitter:creator\" content=\"@HuiQiQu\" \/>\n<meta name=\"twitter:label1\" content=\"Written by\" \/>\n\t<meta name=\"twitter:data1\" content=\"hqqu\" \/>\n\t<meta name=\"twitter:label2\" content=\"Est. reading time\" \/>\n\t<meta name=\"twitter:data2\" content=\"1 minute\" \/>\n<script type=\"application\/ld+json\" class=\"yoast-schema-graph\">{\"@context\":\"https:\\\/\\\/schema.org\",\"@graph\":[{\"@type\":\"Article\",\"@id\":\"https:\\\/\\\/blogs.bmj.com\\\/jmg\\\/2023\\\/05\\\/18\\\/pulmonary-function-and-structure-abnormalities-in-children-and-young-adults-with-osteogenesis-imperfecta-point-to-intrinsic-and-extrinsic-lung-abnormalities\\\/#article\",\"isPartOf\":{\"@id\":\"https:\\\/\\\/blogs.bmj.com\\\/jmg\\\/2023\\\/05\\\/18\\\/pulmonary-function-and-structure-abnormalities-in-children-and-young-adults-with-osteogenesis-imperfecta-point-to-intrinsic-and-extrinsic-lung-abnormalities\\\/\"},\"author\":{\"name\":\"hqqu\",\"@id\":\"https:\\\/\\\/blogs.bmj.com\\\/jmg\\\/#\\\/schema\\\/person\\\/be0250f8d5b52412c3e7c222dabd591b\"},\"headline\":\"Pulmonary function and structure abnormalities in children and young adults with osteogenesis imperfecta point to intrinsic and extrinsic lung abnormalities (Contributed by Dr. Joan Marini)\",\"datePublished\":\"2023-05-18T01:48:10+00:00\",\"dateModified\":\"2026-02-23T00:13:04+00:00\",\"mainEntityOfPage\":{\"@id\":\"https:\\\/\\\/blogs.bmj.com\\\/jmg\\\/2023\\\/05\\\/18\\\/pulmonary-function-and-structure-abnormalities-in-children-and-young-adults-with-osteogenesis-imperfecta-point-to-intrinsic-and-extrinsic-lung-abnormalities\\\/\"},\"wordCount\":151,\"commentCount\":0,\"publisher\":{\"@id\":\"https:\\\/\\\/blogs.bmj.com\\\/jmg\\\/#organization\"},\"inLanguage\":\"en-US\",\"potentialAction\":[{\"@type\":\"CommentAction\",\"name\":\"Comment\",\"target\":[\"https:\\\/\\\/blogs.bmj.com\\\/jmg\\\/2023\\\/05\\\/18\\\/pulmonary-function-and-structure-abnormalities-in-children-and-young-adults-with-osteogenesis-imperfecta-point-to-intrinsic-and-extrinsic-lung-abnormalities\\\/#respond\"]}]},{\"@type\":\"WebPage\",\"@id\":\"https:\\\/\\\/blogs.bmj.com\\\/jmg\\\/2023\\\/05\\\/18\\\/pulmonary-function-and-structure-abnormalities-in-children-and-young-adults-with-osteogenesis-imperfecta-point-to-intrinsic-and-extrinsic-lung-abnormalities\\\/\",\"url\":\"https:\\\/\\\/blogs.bmj.com\\\/jmg\\\/2023\\\/05\\\/18\\\/pulmonary-function-and-structure-abnormalities-in-children-and-young-adults-with-osteogenesis-imperfecta-point-to-intrinsic-and-extrinsic-lung-abnormalities\\\/\",\"name\":\"Pulmonary function and structure abnormalities in children and young adults with osteogenesis imperfecta point to intrinsic and extrinsic lung abnormalities (Contributed by Dr. Joan Marini) - 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