Bohadana A B, Maaledj M, Uffholtz H, Peslin R, Sadoul P
Nouv Presse Med. 1978 Jun 24;7(25):2227-30.
We report two cases of patients with Von Recklinghausen disease. They exhibited typical cutaneous and diffuse intrapulmonary lesions accompanied by effort dyspnea. Multiple radiological abnormalities were noticed and the pictures showed nodular and interstitial lesions as well as emphysematous areas. Pulmonary function tests revealed more or less marked hypoxemia without hypercapnia, and decreased maximal expiratory flows due, at least in part, to a loss of elastic recoil.
我们报告了两例冯·雷克林霍增氏病患者。他们表现出典型的皮肤和弥漫性肺内病变，并伴有劳力性呼吸困难。发现了多种放射学异常，影像学显示有结节状和间质性病变以及肺气肿区域。肺功能测试显示或多或少有明显的低氧血症，无高碳酸血症，最大呼气流量降低，至少部分原因是弹性回缩丧失。
