Yamamoto S, Hishinuma S, Tachibana K, Hamada H, Hara H, Sakatani M, Ueda E, Akira M, Yamamoto S
Department of Internal Medicine, National Kinki-Chuo Hospital for chest diseases, Japan.
Nihon Kyobu Shikkan Gakkai Zasshi. 1996 Feb;34(2):226-30.
Two patients with von Recklinghausen's disease had diffuse pulmonary diseases. The pulmonary diseases were interstitial fibrosis and emphysematous bullae. In case 2, examination of a specimen obtained by transbronchial lung biopsy revealed fibrotic interstitial thickening and adenomatous hyperplasia. Diffuse pulmonary abnormalities in von Recklinghausen's disease have been said to be fibrotic changes and emphysematous changes, as shown in roentgenograms in these two cases. The biopsy specimen obtained in case 2, however, revealed fibrotic changes only, which could not be distinguished from findings in cases of idiopathic interstitial pneumonia. In case 2, bronchoalveolar lavage fluid was also examined but it did not reveal any unique features of von Recklinghausen's disease. Since 1978, von Recklinghausen's disease has been diagnosed 10 times in our hospital, but diffuse pulmonary diseases was seen in only two of those 10 cases.
两名患有冯雷克林霍增氏病(神经纤维瘤病)的患者出现了弥漫性肺部疾病。肺部疾病为间质性纤维化和肺气肿大疱。在病例2中,经支气管肺活检获取的标本检查显示纤维化间质增厚和腺瘤样增生。如这两个病例的X线片所示,冯雷克林霍增氏病中的弥漫性肺部异常据说是纤维化改变和肺气肿改变。然而,病例2中获取的活检标本仅显示纤维化改变,这与特发性间质性肺炎病例的表现无法区分。在病例2中,还对支气管肺泡灌洗 fluid进行了检查,但未发现冯雷克林霍增氏病的任何独特特征。自1978年以来,我院已诊断出10例冯雷克林霍增氏病,但在这10例中仅有2例出现了弥漫性肺部疾病。 (注:原文中“bronchoalveolar lavage fluid”表述有误,可能是“bronchoalveolar lavage fluid”,意为支气管肺泡灌洗)