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原发性肺动脉高压症(Primary Pulmo-nary Hypertension)是一种罕见的原因未明的进行性疾病。系指原发于肺小动脉的增生性病变,伴有丛状病变形成,以致肺小动脉(管外径1mm 以下者)广泛阻塞,肺动脉压持续增高,继而右心肥大。其主要症状为咳嗽,咯血,运动后呼吸困难,乏力,晕厥发作及胸痛,晚期常致心衰死亡。本病常易误诊,预后不良。为进一步认识其病变特点,为临床研究提供可靠的病理形态学依据,现将本教研室1984年前收集的2例原发性肺动脉高压症的尸检材料报道如下。患者男、女各1例,发病年龄为3个月及12岁,病程(从出现症状至死亡)为半个月及3个月。组织切片按照常规方法制作,部分切片加染MCT,Weigert’s 弹力纤维染色,光
Primary Pulmo-nary Hypertension is a rare, progressive, unexplained disease. It refers to the primary proliferative lesions of the pulmonary arterioles, accompanied by the formation of plexiform lesions, resulting in extensive obstruction of the pulmonary arterioles (tube diameter below 1mm), pulmonary hypertension continued to increase, then right heart hypertrophy. The main symptoms of cough, hemoptysis, dyspnea after exercise, fatigue, fainting and chest pain, heart failure often caused by late death. The disease is often misdiagnosed, the prognosis is poor. In order to further understand the characteristics of its lesions, to provide a reliable basis for the clinical pathological morphology, now this department collected two cases of autopsy in 1984 before the autopsy materials are reported below. One patient was male and one female. The age of onset was 3 months and 12 years. The course of disease (from symptom to death) was half a month and 3 months. Tissue sections were prepared according to conventional methods, partially stained with MCT, Weigert’s spandex staining, light