Appelbaum L, Yigla M, Bendayan D, Reichart N, Fink G, Priel I, Schwartz Y, Richman P, Picard E, Goldman S, Kramer M R
Pulmonary Institute, Hadassah University Medical Center, Jerusalem, Israel.
Chest. 2001 Jun;119(6):1801-6. doi: 10.1378/chest.119.6.1801.
To characterize the incidence of patients with primary pulmonary hypertension (PPH) in Israel and their outcomes.
We have evaluated retrospectively all the patients in Israel in whom PPH was diagnosed between the years 1988 and 1997. We looked at medical history, hemodynamic data, pulmonary function and gas exchange, and demographic variables. Patients were followed up for survival until November 1997. Life table analysis and Kaplan-Meier statistics were used to estimate the overall survival distribution. Regression analysis was used to examine the relations between survival and selected variables.
Overall, we found 44 patients with PPH. The estimated incidence of PPH in Israel is 1.4 new cases per year per million population. The mean (+/- SD) age at diagnosis was 43 +/- 13 years. In the Jewish population, PPH was more frequent among immigrants from Europe and the United States. The mean interval from the onset of symptoms to diagnosis was 3 years (median, 2 years). The median survival time was 4 years. The 1-year, 3-year, and 5-year survival rates were 82%, 57%, and 43%, respectively. The major variables influencing the survival rate were the following: interval from symptom onset to diagnosis; and hemodynamic measurements (ie, mean pulmonary artery pressure, mean right atrial pressure, and cardiac index). In comparison to rates discerned from the National Institutes of Health registry data, the survival rate in Israel is somewhat better and prognosis is influenced by similar hemodynamic variables.
PPH is a rare and fatal disease in Israel. New therapeutic modalities such as prostacyclin therapy and lung transplantation may improve survival among patients with this malignant disease.
描述以色列原发性肺动脉高压（PPH）患者的发病率及其预后。
我们回顾性评估了1988年至1997年间在以色列被诊断为PPH的所有患者。我们研究了病史、血流动力学数据、肺功能和气体交换以及人口统计学变量。对患者进行随访直至1997年11月以了解生存情况。采用生命表分析和Kaplan-Meier统计方法来估计总体生存分布。使用回归分析来检验生存与选定变量之间的关系。
总体而言，我们发现了44例PPH患者。以色列PPH的估计发病率为每年每百万人口中有1.4例新发病例。诊断时的平均（±标准差）年龄为43±13岁。在犹太人群中，PPH在来自欧洲和美国的移民中更为常见。从症状出现到诊断的平均间隔时间为3年（中位数为2年）。中位生存时间为4年。1年、3年和5年生存率分别为82%、57%和43%。影响生存率的主要变量如下：从症状出现到诊断的间隔时间；以及血流动力学测量值（即平均肺动脉压、平均右心房压和心脏指数）。与美国国立卫生研究院登记数据得出的比率相比，以色列的生存率略高，且预后受类似的血流动力学变量影响。
PPH在以色列是一种罕见且致命的疾病。诸如前列环素治疗和肺移植等新的治疗方式可能会提高这种恶性疾病患者的生存率。
