Kodama K, Hamada M, Shigematsu Y, Suzuki M, Hiwada K, Hayashi Y, Hashimoto H, Hashimoto H, Sugita A
Second Department of Internal Medicine, Ehime University School of Medicine, Japan.
Jpn J Med. 1991 May-Jun;30(3):273-7. doi: 10.2169/internalmedicine1962.30.273.
Familial primary pulmonary hypertension was found in two siblings (sister and brother). The woman noted exertional dyspnea at the age of 28 yr. The younger brother noticed exertional dyspnea, cough with bloody sputum and pretibial edema at the age of 38 yr. We diagnosed them as primary pulmonary hypertension as based on hemodynamic and histopathological findings. Regardless of the treatment, both patients died of right-sided heart failure with a short time course. We examined their family members, but there were no other members with primary pulmonary hypertension.
在一对姐弟中发现了家族性原发性肺动脉高压。该女性在28岁时出现劳力性呼吸困难。弟弟在38岁时出现劳力性呼吸困难、咯血痰咳嗽和胫前水肿。根据血流动力学和组织病理学检查结果，我们将他们诊断为原发性肺动脉高压。尽管进行了治疗，两名患者均在短时间内死于右心衰竭。我们对他们的家庭成员进行了检查，但没有其他原发性肺动脉高压患者。
