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Case Report

A rare form histiocytic disorders of the lung: adult niemann pick disease type b

Funda Demirag, Gungor Dulkar, Erkmen Gulhan, Fatma Ikbal Saka.

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.

Key words: Niemann-Pick, histiocytic disorders, lung, lipoid pneumonia

Article Language: Turkish English

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Archives of Clinical and Experimental Surgery (ACES)


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