{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,12]],"date-time":"2026-02-12T08:20:39Z","timestamp":1770884439237,"version":"3.50.1"},"reference-count":36,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2024,2,5]],"date-time":"2024-02-05T00:00:00Z","timestamp":1707091200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Pediatr."],"abstract":"<jats:sec><jats:title>Introduction<\/jats:title><jats:p>Transforming growth factor \u03b2 (TGF\u03b2) metabolism plays an important role in the pathogenesis of Marfan syndrome (MFS). Accordingly, drug therapy uses TGF\u03b2 receptor blockade to slow down the cardiovascular manifestations, above all aortic root dilatation. Angiotensin II type 1 receptor blockers (ARBs) have been shown to reduce TGF\u03b2 levels in adults. Data on childhood are lacking and are now being investigated in the TiGer For Kids study presented here.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We examined 125 children without chronic disease and 31 pediatric Marfan patients with a proven <jats:italic>FBN1<\/jats:italic> variant with regard to TGF\u03b2 levels. In addition, we measured TGF\u03b2 levels during the initiation of ARB therapy in pediatric Marfan patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In children without chronic disease, TGF\u03b2 levels were found to decrease from childhood to adolescence (<jats:italic>p<\/jats:italic>\u2009&amp;lt;\u20090.0125). We could not measure a relevantly increased TGF\u03b2 level in pediatric Marfan patients. However, we showed a significant suppression of the TGF\u03b2 level after treatment with ARBs (<jats:italic>p<\/jats:italic>\u2009&amp;lt;\u20090.0125) and a renewed increase shortly before the next dose.<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>The TGF\u03b2 level in childhood changes in an age-dependent manner and decreases with age. The TGF\u03b2 level drops significantly after taking ARBs. Based on our experience and data, a TGF\u03b2 receptor blockade in childhood seems reasonable. So far, TGF\u03b2 level cannot be used as an MFS screening biomarker.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fped.2024.1276215","type":"journal-article","created":{"date-parts":[[2024,2,5]],"date-time":"2024-02-05T04:58:40Z","timestamp":1707109120000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":3,"title":["TGF\u03b2 level in healthy and children with Marfan syndrome\u2014effective reduction under sartan therapy"],"prefix":"10.3389","volume":"12","author":[{"given":"Veronika C.","family":"Stark","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jakob","family":"Olfe","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daniel","family":"Diaz-Gil","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yskert","family":"von 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