中国临床实用医学
中國臨床實用醫學
중국림상실용의학
CHINA CLINICAL PRACTICAL MEDICINE
2010年
12期
56-58
,共3页
特发性肺含铁血黄素沉着症%儿童%文献复习
特髮性肺含鐵血黃素沉著癥%兒童%文獻複習
특발성폐함철혈황소침착증%인동%문헌복습
Idiopathic pulmonary hemosiderosis%Children%Literature review
目的 总结儿童特发性肺含铁血黄素沉着症(idiopathic pulmonary bemosiderosis,IPH)的临床表现、实验室检查、影像学、病理学特点,并探讨诊断、治疗方法和预后.方法 本例患者进行实验室、影像学、深部吸痰并行组织病理学检查确诊,糖皮质激素治疗并随访,探讨本例患者并结合国内外相关报道文献进行复习.结果 临床表现主要是反复发作咯血、咳嗽、贫血,部分心慌、胸闷、乏力,肺部体征不明显,影像学改变可见肺部弥漫性毛玻璃样改变,相关免疫学检查(如抗核抗体、抗中性粒细胞包浆抗体)均阴性,血分析呈小细胞低色素性贫血,血清铁降低,胆红素升高,反复深部痰液可找到含铁血黄素巨噬细胞.结论 儿童特发性肺含铁血黄素沉着症临床表现多样,需与缺铁性贫血、支气管肺炎、肺结核相鉴别,容易误诊、漏诊,痰、胃液及支气管肺泡灌洗液活检可提高阳性率,应加强对本病的认知,及早诊断和干预;长时间糖皮质激素治疗后可使病情处于较稳定状态;预后不良.
目的 總結兒童特髮性肺含鐵血黃素沉著癥(idiopathic pulmonary bemosiderosis,IPH)的臨床錶現、實驗室檢查、影像學、病理學特點,併探討診斷、治療方法和預後.方法 本例患者進行實驗室、影像學、深部吸痰併行組織病理學檢查確診,糖皮質激素治療併隨訪,探討本例患者併結閤國內外相關報道文獻進行複習.結果 臨床錶現主要是反複髮作咯血、咳嗽、貧血,部分心慌、胸悶、乏力,肺部體徵不明顯,影像學改變可見肺部瀰漫性毛玻璃樣改變,相關免疫學檢查(如抗覈抗體、抗中性粒細胞包漿抗體)均陰性,血分析呈小細胞低色素性貧血,血清鐵降低,膽紅素升高,反複深部痰液可找到含鐵血黃素巨噬細胞.結論 兒童特髮性肺含鐵血黃素沉著癥臨床錶現多樣,需與缺鐵性貧血、支氣管肺炎、肺結覈相鑒彆,容易誤診、漏診,痰、胃液及支氣管肺泡灌洗液活檢可提高暘性率,應加彊對本病的認知,及早診斷和榦預;長時間糖皮質激素治療後可使病情處于較穩定狀態;預後不良.
목적 총결인동특발성폐함철혈황소침착증(idiopathic pulmonary bemosiderosis,IPH)적림상표현、실험실검사、영상학、병이학특점,병탐토진단、치료방법화예후.방법 본례환자진행실험실、영상학、심부흡담병행조직병이학검사학진,당피질격소치료병수방,탐토본례환자병결합국내외상관보도문헌진행복습.결과 림상표현주요시반복발작각혈、해수、빈혈,부분심황、흉민、핍력,폐부체정불명현,영상학개변가견폐부미만성모파리양개변,상관면역학검사(여항핵항체、항중성립세포포장항체)균음성,혈분석정소세포저색소성빈혈,혈청철강저,담홍소승고,반복심부담액가조도함철혈황소거서세포.결론 인동특발성폐함철혈황소침착증림상표현다양,수여결철성빈혈、지기관폐염、폐결핵상감별,용역오진、루진,담、위액급지기관폐포관세액활검가제고양성솔,응가강대본병적인지,급조진단화간예;장시간당피질격소치료후가사병정처우교은정상태;예후불량.
Objective To summarize the clinical manifestation, laboratory examination, radiological and pathological characteristics of idiopathic pulmonary hemosiderosis in children and discusses the diagnosis,treatment and prognosis. Methods The patient for laboratory, imaging, deep sucking phlegm parallel histopathological examination confirmed, corticosteroid and followed-up, discusse the patient and combined domestic and foreign literature review reports. Results Clinical manifestations of main were recurrent haemoptysis,cough, anemia, partial bosom frowsty, flustered, feeble, no obvious signs in lungs. Radiculopathy lung diffuse ground-glass appearance was changed, relevant immunological tests (e. g, antinuclear antibodies, neutrophils patina antibodies) were negative, blood analysis was a hypochromic microcytic anemia, elevated bilirubin, serum iron reduces, deep sputum containing blood can be found macrophages. Conclusion Children idiopathic pulmonary hemosiderin clinical manifestations of diversity, with iron deficiency anemia, bronchopneumonia, tuberculosis, it is easy to be misdiagnosed, distinguish repeatedly, the diagnosis of gastric juice and bronchoaleolar laage biopsy can improve the rate of disease, should strengthen the cognition, early diagnosis and intervention. Long-range cocorticoid therapy can make an illness in a stable state. The poor prognosis.
