A RARE FORM HISTIOCYTIC DISORDERS OF THE LUNG: ADULT NIEMANN PICK DISEASE TYPE B

descriptionPublicationkeyboard_double_arrow_right Article , Other literature type 01 Jan 2019Publisher:ScopeMedJournal:International Journal of Medical Reviews and Case Reports (eissn: 2534-9821,

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Authors: Demirag, Funda; Gungor Dulkar; Erkmen Gulhan; Saka, Fatma Ikbal;

doi: 10.5455/ijmrcr.adult-niemann-pick-disease , 10.5281/zenodo.7545650 , 10.5281/zenodo.7545649

A RARE FORM HISTIOCYTIC DISORDERS OF THE LUNG: ADULT NIEMANN PICK DISEASE TYPE B

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Abstract

Although pulmonary involvement in Niemann-Pick disease is common, the appearance of interstitial lung disease in adulthood is rare. The differential diagnosis of adult-type Niemann-Pick disease includes pulmonary histiocytic disorders. We reported a 21-year-old man with a family history of Niemann-Pick disease type B and hepatosplenomegaly and a miliary appearance on chest X-ray suspicious of tuberculosis. The clinical, laboratory and radiologic data of our case were discussed.

Keywords

Niemann-Pick, histiocytic disorders, lung, lipoid pneumonia

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