Beaumont F, Jansen H M, Elema J D, ten Kate L P, Sluiter H J
Thorax. 1981 Apr;36(4):252-8. doi: 10.1136/thx.36.4.252.
The coexistence of interstitial pulmonary fibrosis and alveolar cell carcinoma is well known. The familial occurrence of a combination of these two entities, however, is very rare. We present a family of which five members had diffuse interstitial pulmonary fibrosis. Three of them had in addition alveolar cell carcinoma. In a sixth family member, evidence of alveolar cell carcinoma was present without proven interstitial fibrosis. An autosomal dominant trait is suggested as the mode of inheritance of both interstitial fibrosis and alveolar cell carcinoma in this family.
间质性肺纤维化与肺泡细胞癌并存是众所周知的。然而,这两种病症同时在家族中出现的情况非常罕见。我们报告一个家族,其中五名成员患有弥漫性间质性肺纤维化。他们中的三人还患有肺泡细胞癌。在第六名家族成员中,存在肺泡细胞癌的证据,但未证实有间质性纤维化。在这个家族中,间质性纤维化和肺泡细胞癌的遗传模式提示为常染色体显性性状。