{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,26]],"date-time":"2026-02-26T18:32:14Z","timestamp":1772130734155,"version":"3.50.1"},"reference-count":29,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2016,10,21]],"date-time":"2016-10-21T00:00:00Z","timestamp":1477008000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Progetto MIUR-PRIN2012","award":["20128PNX83_002"],"award-info":[{"award-number":["20128PNX83_002"]}]},{"name":"RC 2016 Fondazione IRCCS Ca\u2019 Granda - Ospedale Maggiore Policlinico, Milano"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2017,1]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Although pulmonary function abnormalities in thalassaemia major (<jats:styled-content style=\"fixed-case\">TM<\/jats:styled-content>) were described in 1980, the pathogenetic mechanism is not clear and data are contradictory, probably because of study heterogeneity and the multifactorial nature of the pathogenesis. We retrospectively analysed 73 adult <jats:styled-content style=\"fixed-case\">TM<\/jats:styled-content> patients to evaluate the prevalence of pulmonary dysfunction in adult <jats:styled-content style=\"fixed-case\">TM<\/jats:styled-content> and investigate relationships with iron load. All patients underwent body plethysmography and carbon monoxide diffusion (<jats:styled-content style=\"fixed-case\">DLCO<\/jats:styled-content>) was assessed in 63, in addition to blood tests, echocardiogram and T2* myocardial and liver magnetic resonance imaging. Restrictive lung disease was present in 26 (35\u00b76%) patients. Serum ferritin levels were higher in patients with restrictive pattern (1526\u00a0\u03bcg\/l vs. 975\u00a0\u03bcg\/l, <jats:italic>P<\/jats:italic>\u00a0=\u00a00\u00b705). Restrictive lung disease did not correlate with cardiac or liver iron overload. However, considering only patients with serum ferritin &gt;2500\u00a0\u03bcg\/l, those with restrictive pattern also had heart (T2* 14\u00b728\u00a0\u00b1\u00a09\u00b799\u00a0ms vs. 31\u00b759\u00a0\u00b1\u00a07\u00b743\u00a0ms) and liver iron overload (<jats:styled-content style=\"fixed-case\">LIC<\/jats:styled-content> 16\u00b702\u00a0\u00b1\u00a08\u00b744\u00a0mg vs. 5\u00b702\u00a0\u00b1\u00a02\u00b769\u00a0mg Fe\/g dry weight) compared to those without restrictive pattern. Twenty\u2010five patients (39\u00b77%) had decreased <jats:styled-content style=\"fixed-case\">DLCO<\/jats:styled-content>. No correlation was observed with iron parameters. In our data restrictive pattern was predominant; we observed a relationship with serum ferritin levels suggesting that iron, particularly its chronic effect, could play a role in the pathogenesis of pulmonary disease.<\/jats:p>","DOI":"10.1111\/bjh.14396","type":"journal-article","created":{"date-parts":[[2016,10,21]],"date-time":"2016-10-21T07:06:39Z","timestamp":1477033599000},"page":"309-314","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Pulmonary dysfunction in thalassaemia major: is there any relationship with body iron stores?"],"prefix":"10.1111","volume":"176","author":[{"given":"Francesca","family":"Guidotti","sequence":"first","affiliation":[{"name":"Rare Diseases Centre Department of Medicine and Medical Specialities Fondazione IRCCS Ca\u2019 Granda \u2013 Ospedale Maggiore Policlinico Milan 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