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我们报道的两例是亲兄弟,可能是家族性顽固性肺高压症的首次报道。例1:胎龄42周,经阴道产后表现中等度呼吸窘迫。肺部放射线摄片显示横膈高位及小量的纵隔积气,生后24小时经气管插管后发生两侧气胸,依据用大量的机械通气也难以纠正的持久性低氧血症以及导管前后的动脉血气分析值证实,有右向左的分流存在,而确诊为顽固性肺高压症。超声心动图表明,无结构上的心脏病。采用过度通气的方法,用箭毒和妥
We reported two cases of brothers, may be the first report of familial intractable pulmonary hypertension. Example 1: Gestational age 42 weeks, showing moderate respiratory distress after vaginal delivery. Pulmonary radiography showed high diaphragmatic and a small amount of mediastinal pneumoperitoneum, pneumothorax occurred on both sides of the trachea after intubation 24 hours after birth, on the basis of persistent hypoxemia that was difficult to correct with a large amount of mechanical ventilation, Arterial blood gas analysis confirmed that right-to-left shunt exists, and diagnosed as refractory pulmonary hypertension. Echocardiography showed no structural heart disease. Using hyperventilation method, with arrow poisoning and proper