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特发性肺含铁血黄素沉着症(IPH)系铁在肺内异常蓄积,临床特点为反复顽固的肺泡内出血,多发生于10岁以下小儿,发生于成人者罕见。由于本病比较少见,绝大部分病例在发病早斯常被误珍。现将笔者自1988年以来收治的8例IPH分析如下。 1 临床资料 1.1 一般资料:男4例。女4例:4岁4例,5岁2例,7岁1例,12岁1例。出生史:足月顺产7例,35周早产1例。病程:3—8个月3例,—3年4例,4年1例。 1.2 症状和体征:咳嗽6例,发热3例,气促3例,咳血4例,呼吸困难1例.面色苍白6例,杵状指1例,肝肿大3例。
Idiopathic pulmonary hemosiderosis (IPH) is an abnormal accumulation of iron in the lungs, the clinical features of recurrent stubborn alveolar hemorrhage, occurred in children under 10 years of age, occurred in adults rare. As the disease is relatively rare, the vast majority of cases in the early onset of illness often Jane Jane. Now the author admitted since 1988, eight cases of IPH analysis is as follows. 1 clinical data 1.1 General Information: 4 males. 4 females: 4 years old in 4 cases, 2 cases in 5 years old, 1 case in 7 years old and 1 case in 12 years old. Birth history: Full-term follow-up of 7 cases, 35 weeks of premature birth in 1 case. Course of disease: 3-8 months in 3 cases, - 3 years in 4 cases, 4 years in 1 case. 1.2 Symptoms and signs: 6 cases of cough, fever in 3 cases, 3 cases of shortness of breath, hemoptysis in 4 cases, dyspnea in 1. Pale pale in 6 cases, clubbing in 1 case, hepatomegaly in 3 cases.