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Niemann-Pick Type B Disease Diagnosed in the Adulthood

dc.authorscopusid 49861369200
dc.authorscopusid 47961186600
dc.authorscopusid 57203790605
dc.authorscopusid 56481519800
dc.contributor.author Demircioğlu, S.
dc.contributor.author Korkmaz, C.
dc.contributor.author Çizmecioğlu, H.A.
dc.contributor.author Poyraz, N.
dc.date.accessioned 2025-05-10T17:01:32Z
dc.date.available 2025-05-10T17:01:32Z
dc.date.issued 2018
dc.department T.C. Van Yüzüncü Yıl Üniversitesi en_US
dc.department-temp Demircioğlu S., Van Yüzüncü Yıl University, Faculty of Medicine, Department of Hematology, Van, Turkey; Korkmaz C., Necmettin Erbakan University, Meram Medical Faculty, Department of Chest Diseases, Konya, Turkey; Çizmecioğlu H.A., Academy Hospital, Internal Medicine Clinic, Konya, Turkey; Poyraz N., Necmettin Erbakan University, Meram Medical Faculty, Department of Radiology, Konya, Turkey en_US
dc.description.abstract Niemann-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. en_US
dc.identifier.doi 10.5505/ejm.2018.19484
dc.identifier.endpage 324 en_US
dc.identifier.issn 1301-0883
dc.identifier.issue 4 en_US
dc.identifier.scopus 2-s2.0-85055128286
dc.identifier.scopusquality Q4
dc.identifier.startpage 322 en_US
dc.identifier.trdizinid 325928
dc.identifier.uri https://doi.org/10.5505/ejm.2018.19484
dc.identifier.uri https://hdl.handle.net/20.500.14720/5203
dc.identifier.volume 23 en_US
dc.identifier.wosquality N/A
dc.language.iso en en_US
dc.publisher Yuzuncu Yil Universitesi Tip Fakultesi en_US
dc.relation.ispartof Eastern Journal of Medicine en_US
dc.relation.publicationcategory Makale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanı en_US
dc.rights info:eu-repo/semantics/openAccess en_US
dc.subject Lung en_US
dc.subject Niemann-Pick Disease en_US
dc.subject Splenomegaly en_US
dc.subject Type B en_US
dc.title Niemann-Pick Type B Disease Diagnosed in the Adulthood en_US
dc.type Article en_US

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