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目的:总结对特发性肺含铁血黄素沉着症(IPH)的诊断及治疗体会。方法:回顾性分析38例IPH患者的临床资料。结果:38例中,中位年龄4.5岁,病程2个月～4.5年。入院时确诊IPH10例,其余分别误诊为:肺炎18例,支气管扩张、肺结核、缺铁性贫血各3例,肾炎-肺出血(Goodpasture)综合征1例,误诊率为74%。所有病例痰液或胃液均找到含铁血黄素细胞。经治疗后完全不发作9例,偶尔发作11例,经常发作12例,死亡4例。结论:特发性肺含铁血黄素沉着症临床表现多样,容易误诊,怀疑本病时应仔细询问有否有机磷农药接触史、在痰液或胃液找含铁黄素细胞,以尽快确诊。治疗以肾上腺皮质激素为主,辅以其它对症治疗。
Objective: To summarize the diagnosis and treatment of idiopathic pulmonary hemosiderosis (IPH). Methods: The clinical data of 38 patients with IPH were retrospectively analyzed. Results: In 38 cases, the median age was 4.5 years and the course of disease was 2 months to 4.5 years. IPH was diagnosed on admission 10 cases, the rest were misdiagnosed as: pneumonia in 18 cases, bronchiectasis, tuberculosis, iron deficiency anemia in 3 cases, nephritis - pulmonary hemorrhage (Goodpasture) syndrome in 1 case, the misdiagnosis rate was 74%. All cases sputum or gastric juice to find hemosiderin cells. After treatment, 9 cases did not attack at all, occasionally attack in 11 cases, 12 cases were recurrent and 4 died. Conclusions: Idiopathic pulmonary hemosiderosis clinical manifestations of diverse and easily misdiagnosed, suspected disease should be carefully asked whether there is history of exposure to organophosphorus pesticides, in the sputum or gastric fluid to find containing flavin cells, to confirm as soon as possible. Treatment of adrenal cortex hormones, supplemented by other symptomatic treatment.