{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,8]],"date-time":"2026-07-08T15:51:44Z","timestamp":1783525904561,"version":"3.55.0"},"reference-count":18,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2006,2,7]],"date-time":"2006-02-07T00:00:00Z","timestamp":1139270400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Histopathology"],"published-print":{"date-parts":[[2006,4]]},"abstract":"<jats:p><jats:bold>Aims<\/jats:bold> :\u2002Although pulmonary involvement is a known cause of morbidity in Niemann\u2013Pick disease, histological features in the lung are not well characterized. The purpose of this study is to document the histological features seen in pulmonary involvement by types B and C Niemann\u2013Pick disease and to correlate them with clinical and imaging data.<\/jats:p><jats:p><jats:bold>Methods and results<\/jats:bold> :\u2002Surgical lung biopsies from six patients (four with type B and two with type C disease) were reviewed and all showed diffuse endogenous lipid pneumonia, with lesser involvement of the interstitium by fibrosis and foamy macrophage accumulation. In type B disease only, there was also fine cytoplasmic vacuolation within the cytoplasm of ciliated epithelial cells. Neither disease showed foamy changes within pneumocytes. One patient had a bronchial cast removed on whole lung lavage. Electron microscopy showed abnormal lamellar inclusions within lysosomes of affected cells in type B disease. In patients with type C disease, biopsies were undertaken as part of investigations into acute respiratory failure in the context of multiorgan systemic presentation. Three patients with type B disease had clinical disease limited to the lung, all adults (mean age of 40\u2003years) with unexplained diffuse parenchymal lung disease and mainly ground\u2010glass shadowing on high\u2010resolution computed tomography.<\/jats:p><jats:p><jats:bold>Conclusions<\/jats:bold> :\u2002Niemann\u2013Pick disease should be considered for any patient with unexplained diffuse endogenous lipid pneumonia, even when disease is limited to the lungs and presentation is during adulthood.<\/jats:p>","DOI":"10.1111\/j.1365-2559.2006.02355.x","type":"journal-article","created":{"date-parts":[[2006,2,7]],"date-time":"2006-02-07T15:49:14Z","timestamp":1139327354000},"page":"596-603","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":78,"title":["Pulmonary involvement by Niemann\u2013Pick disease. A report of six cases"],"prefix":"10.1111","volume":"48","author":[{"given":"A G","family":"Nicholson","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R","family":"Florio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D M","family":"Hansell","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R M","family":"du Bois","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A U","family":"Wells","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P","family":"Hughes","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H K","family":"Ramadan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C I","family":"Mackinlay","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E","family":"Brambilla","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G R","family":"Ferretti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A","family":"Erichsen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M","family":"Malone","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S","family":"Lantuejoul","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2006,2,7]]},"reference":[{"key":"e_1_2_6_2_2","first-page":"2601","volume-title":"The metabolic and molecular bases of inherited disease.","author":"Schuchman EH","year":"1995"},{"key":"e_1_2_6_3_2","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.165.1.2103113"},{"key":"e_1_2_6_4_2","doi-asserted-by":"publisher","DOI":"10.1007\/s004390100531"},{"key":"e_1_2_6_5_2","doi-asserted-by":"publisher","DOI":"10.1086\/324068"},{"key":"e_1_2_6_6_2","first-page":"373","article-title":"A fatal respiratory form of type C Niemann\u2013Pick disease","volume":"47","author":"Pin I","year":"1990","journal-title":"Arch. 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