CAJ | 학술논문

目的：探讨成人特发性肺含铁血黄素沉着症（ idiopathic pulmonary hemosiderosis ，IPH）的病因、临床症状、影像学特点及治疗方法。方法本例患者采用支气管镜肺活检确诊，病例资料完整，同时结合国内外文献进行综合性回顾分析。结果本例患者为45岁成年女性，经支气管镜肺活检确诊为弥漫性肺泡出血（ diffuse alveolar hemorrhage ，DPH），同时实验室检查排除其他DPH，确诊为IPH，予糖皮质激素治疗效果好，经随访观察未复发。结论特发性肺含铁血黄素沉着症是一种少见、病因未明的弥漫性肺泡出血综合征，多见于儿童，急性期激素治疗效果好，成人预后较儿童好。
목적：탐토성인특발성폐함철혈황소침착증（ idiopathic pulmonary hemosiderosis ，IPH）적병인、림상증상、영상학특점급치료방법。방법본례환자채용지기관경폐활검학진，병례자료완정，동시결합국내외문헌진행종합성회고분석。결과본례환자위45세성년녀성，경지기관경폐활검학진위미만성폐포출혈（ diffuse alveolar hemorrhage ，DPH），동시실험실검사배제기타DPH，학진위IPH，여당피질격소치료효과호，경수방관찰미복발。결론특발성폐함철혈황소침착증시일충소견、병인미명적미만성폐포출혈종합정，다견우인동，급성기격소치료효과호，성인예후교인동호。
Objective Discuss the pathogenesis , clinical symptoms , imaging and therapy of idiopathic pulmonary hemosiderosis ( IPH) .Methods The case of this report was diagnosed by bronchoscope ,the data was complete,and review the domestic and overseas literature .Results The case was a 45-year-old woman, was confirmed diffuse alveolar hemorrhage ( DPH ) by bronchoscope , other DPH was excluded by laboratory examination,the glucocorticoid therapeutic effect was satisfactory , and there was no relapse during the follow-up observation.Conclusions IPH is a disease of DPH which is rare and agnogenic , it occurs in child more often, the hormonotherapy is satisfactory ,the prognosis in adult is better than in child .