Pulmonary Manifestations in Cerebrotendinous Xanthomatosis.

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  • KAWABATA Masaharu
    The Third Department of Internal Medicine, Kagoshima University School of Medicine
  • KURIYAMA Masaru
    The Third Department of Internal Medicine, Kagoshima University School of Medicine
  • MORI Shinitirou
    The Third Department of Internal Medicine, Kagoshima University School of Medicine
  • SAKASHITA Izumi
    The Third Department of Internal Medicine, Kagoshima University School of Medicine
  • OSAME Mitsuhiro
    The Third Department of Internal Medicine, Kagoshima University School of Medicine

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  • Pulmonary Manifestations in Cerebrotend

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Abstract

We investigated five cases with cerebrotendinous xanthomatosis (CTX) with particular reference to biochemical and pathological pulmonary disorders. To date, few reports discuss the pathophysiology of pulmonary disorders of CTX patients. This study is the first investigation of such pulmonary disorders. All 5 patients had no pulmonary symptoms and no disturbances on radiological studies and pulmonary function tests. However, in bronchoalveolar lavage (BAL) fluids, many cells with cruciform reflexes, which is characteristic of intracellular sterol accumulation, were found under phase contrast microscopy. Biochemically, cholestanol was found to be increased in the BAL fluid as well as in serum. Pathological findings of transbronchial lung biopsy (TBLB) samples disclosed foamy macrophages and small granulomas in alveolar septa. In conclusion, the lung was apparently involved in CTX, and the lesions were characterized with the accumulation of foamy and giant cells with a high concentration of cholestanol, which likely results in the formation of foreign body granulomas.<br>(Internal Medicine 37: 922-926, 1998)

Journal

  • Internal Medicine

    Internal Medicine 37 (11), 922-926, 1998

    The Japanese Society of Internal Medicine

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