{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,5]],"date-time":"2026-06-05T05:56:23Z","timestamp":1780638983176,"version":"3.54.1"},"reference-count":30,"publisher":"FapUNIFESP (SciELO)","issue":"6","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. bras. pneumol."],"abstract":"<jats:p>ABSTRACT  Objective: To analyze HRCT findings in patients with Niemann-Pick disease (NPD) type B, in order to determine the frequency of HRCT patterns and their distribution in the lung parenchyma, as well as the most common clinical characteristics.  Methods: We studied 13 patients (3 males and 10 females) aged 5 to 56 years. HRCT images were independently evaluated by two observers, and disagreements were resolved by consensus. The inclusion criteria were presence of abnormal HRCT findings and diagnosis of NPD type B confirmed by histopathological examination of a bone marrow, lung, or liver biopsy specimen.  Results: The most common clinical findings were hepatosplenomegaly and mild to moderate dyspnea. The most common HRCT patterns were smooth interlobular septal thickening and ground-glass opacities, which were both present in all patients. Intralobular lines were present in 12 patients (92.3%). A crazy-paving pattern was observed in 5 patients (38.4%), and areas of air trapping were identified in only 1 case (7.6%). Pulmonary involvement was bilateral in all cases, with the most affected area being the lower lung zone.  Conclusions: Smooth interlobular septal thickening, with or without associated ground-glass opacities, in patients with hepatosplenomegaly is the most common finding in NPD type B.<\/jats:p>","DOI":"10.1590\/s1806-37562017000000062","type":"journal-article","created":{"date-parts":[[2018,1,10]],"date-time":"2018-01-10T23:32:40Z","timestamp":1515627160000},"page":"451-455","source":"Crossref","is-referenced-by-count":27,"title":["Niemann-Pick disease type B: HRCT assessment of pulmonary involvement"],"prefix":"10.1590","volume":"43","author":[{"given":"Heloisa Maria Pereira","family":"Freitas","sequence":"first","affiliation":[{"name":"Universidade Federal do Rio de Janeiro,  Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandre Dias","family":"Man\u00e7ano","sequence":"additional","affiliation":[{"name":"Hospital Anchieta,  Brasil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rosana Souza","family":"Rodrigues","sequence":"additional","affiliation":[{"name":"Universidade Federal do Rio de Janeiro,  Brazil; Instituto D\u2019Or de Pesquisa e Educa\u00e7\u00e3o,  Brasil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bruno","family":"Hochhegger","sequence":"additional","affiliation":[{"name":"Santa Casa de Porto Alegre,  Brasil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pedro Paulo Teixeira e Silva","family":"Torres","sequence":"additional","affiliation":[{"name":"Multimagem Diagn\u00f3sticos,  Brasil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dante","family":"Escuissato","sequence":"additional","affiliation":[{"name":"Universidade Federal do Paran\u00e1,  Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cesar Augusto","family":"Araujo Neto","sequence":"additional","affiliation":[{"name":"Universidade Federal da Bahia,  Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edson","family":"Marchiori","sequence":"additional","affiliation":[{"name":"Universidade Federal do Rio de Janeiro,  Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"530","published-online":{"date-parts":[[2017,12]]},"reference":[{"issue":"2","key":"ref1","doi-asserted-by":"crossref","first-page":"357","DOI":"10.1148\/rg.352140197","article-title":"Histiocytic disorders of the chest imaging findings","volume":"35","author":"Ahuja J","year":"2015","journal-title":"Radiographics"},{"issue":"1","key":"ref2","doi-asserted-by":"crossref","first-page":"134","DOI":"10.1111\/j.1440-1843.2008.01352.x","article-title":"Lung cyst an unusual manifestation of Niemann-Pick disease","volume":"14","author":"Baldi BG","year":"2009","journal-title":"Respirology"},{"issue":"1","key":"ref3","doi-asserted-by":"crossref","first-page":"339","DOI":"10.1148\/radiol.2381041696","article-title":"Type B Niemann-Pick 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