Demircioğlu, S.Korkmaz, C.Çizmecioğlu, H.A.Poyraz, N.2025-05-102025-05-1020181301-088310.5505/ejm.2018.194842-s2.0-85055128286https://doi.org/10.5505/ejm.2018.19484https://hdl.handle.net/20.500.14720/5203Niemann-Pick disease (NPD) type B is an autosomal recessive liposomal storage disorder. NPD type B can present later in life with predominantly visceral symptoms, and has a more favorable prognosis. The natural history of NPD-B is one of progressive hypersplenism and gradual deterioration of pulmonary function. In this paper, we presented NPD type B patient who were diagnosed at 41 years old while being followed for years with the diagnosis of interstitial lung disease. © 2018, Yuzuncu Yil Universitesi Tip Fakultesi. All rights reserved.eninfo:eu-repo/semantics/openAccessLungNiemann-Pick DiseaseSplenomegalyType BArticle234N/AQ4322324325928