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  • Title: Lipidosis with sea-blue histiocytes. Report of two siblings with lung involvement.
    Author: Göğüş S, Göçmen A, Koçak N, Kiper N, Küçükali T, Yüce A, Büyükpamukçu N.
    Journal: Turk J Pediatr; 1994; 36(2):139-44. PubMed ID: 8016915.
    Abstract:
    Two siblings, an eight-year-old girl and a three-year-old boy with lipid storage disease, most likely non-neuropathic Niemann-Pick disease (NPD) with sea-blue histiocytes, are presented. Both of them had foamy and sea-blue histiocytes in their bone marrow smears and reticulo-nodular appearance of both lungs on their chest X-rays. Case 1 had diffuse, biopsy-proven, pulmonary involvement associated with sea-blue histiocytes. Although diffuse reticulo-nodular pulmonary infiltration of non-neuropathic NPD (type B) is frequently detected on chest X-rays, to our knowledge there is only one reported adult case of lipidosis resembling NPD in which severe pulmonary involvement associated with pigmented histiocytes and Niemann-Pick cells were demonstrated at autopsy.
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