Wagener O E, Roncoroni A J, Barcat J A
Instituto de Investigaciones Médicas Alfredo Lanari, Facultad de Medicina, Universidad de Buenos Aires, Argentina.
Chest. 1989 Jan;95(1):234-7. doi: 10.1378/chest.95.1.234.
A 49-year-old man with normal intelligence and no cutaneous lesions had symptoms and signs of cor pulmonale. The chest roentgenogram showed an interstitial pattern. Hemodynamic studies revealed increased pulmonary arterial and wedge pressures. The patient died in shock, and the postmortem findings were a diffuse smooth muscle proliferation in the lungs as the cause of the pulmonary hypertension. We presume that this is a form of pulmonary tuberous sclerosis of which few cases are reported with such severe pulmonary hypertension as shown by our measurements.
一名49岁男性，智力正常，无皮肤病变，有肺心病的症状和体征。胸部X线片显示间质纹理。血流动力学研究显示肺动脉压和楔压升高。患者死于休克，尸检结果显示肺部弥漫性平滑肌增生是肺动脉高压的病因。我们推测这是一种肺结节性硬化症，据我们测量，很少有病例报道有如此严重的肺动脉高压。
