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

Sorumlu Yazar: Sinan Demircioğlu, Türkiye

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