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Niemann-Pick Disease Type B Diagnosed in the Adulthood [Eastern J Med]
Eastern J Med. 2018; 23(4): 322-324 | DOI: 10.5505/ejm.2018.19484  

Niemann-Pick Disease Type B Diagnosed in the Adulthood

Sinan Demircioğlu1, Celalettin Korkmaz2, Hilal Akay Çizmecioğlu3, Necdet Poyraz4
1Van Yüzüncü Yıl University, Faculty of Medicine, Department of Hematology, Van
2Necmettin Erbakan University, Meram Medical Faculty, Department of Chest Diseases, Konya
3Academy Hospital, Internal Medicine Clinic, Konya
4Necmettin Erbakan University, Meram Medical Faculty, Department of Radiology, Konya

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 are presented NPD type B patient who were diagnosed at 41 years old while being followed for years with the diagnosis of interstitial lung disease.

Keywords: Niemann-Pick Disease, Type B, Lung, Splenomegaly


Sinan Demircioğlu, Celalettin Korkmaz, Hilal Akay Çizmecioğlu, Necdet Poyraz. Niemann-Pick Disease Type B Diagnosed in the Adulthood. Eastern J Med. 2018; 23(4): 322-324

Corresponding Author: Sinan Demircioğlu, Türkiye


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