Pulmonary Manifestations in Cerebrotendinous Xanthomatosis.
-
- 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
書誌事項
- タイトル別名
-
- Pulmonary Manifestations in Cerebrotend
この論文をさがす
説明
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)
収録刊行物
-
- Internal Medicine
-
Internal Medicine 37 (11), 922-926, 1998
一般社団法人 日本内科学会
- Tweet
キーワード
詳細情報 詳細情報について
-
- CRID
- 1390282679845825408
-
- NII論文ID
- 10007605716
-
- NII書誌ID
- AA10827774
-
- COI
- 1:STN:280:DyaK1M%2FosVaqtQ%3D%3D
-
- ISSN
- 13497235
- 09182918
-
- NDL書誌ID
- 4614188
-
- PubMed
- 9868952
-
- 本文言語コード
- en
-
- データソース種別
-
- JaLC
- NDL
- Crossref
- PubMed
- CiNii Articles
-
- 抄録ライセンスフラグ
- 使用不可