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特发性肺含铁血黄素沉着症是一组肺泡毛细血管出血性疾病,以铁的异常积累于肺脏为特征,主要为小儿期发病.1990～1994年我院收治5例长期误诊的特发性肺含铁血黄素沉着症,误诊为肺结核最长时间为2年、最短8个月,抗痨治疗无效.为提高本病早期诊断,报告如下.1 临床资料1.1 一般资料 本组5例,男2例,女3例;年龄3～9岁.1.2 症状及体征 重度贫血貌3例,中度贫血貌2例.5例均发热38～38.8℃,咳嗽、咳痰,痰中带血.呼吸急促2例,气喘2例,呼吸困难、口唇青紫1例.肝、脾肿大1例,肝肿大3例,脾肿1例.心尖部闻及SM Ⅲ/6杂音,局部不传导的1例.1.3 实验室检查Hb 30～90g/L,WBC总数4例在正常范围,1例12.2×10~9/L;RBC2例3.2×10~(12)/L,3例1.2～2.4×10~(12)/L;BPC均在正常值内.ESR1～15mm/h.肝功能TTT 12单位1例,谷一丙转氨酶65单
Idiopathic pulmonary hemosiderosis is a group of alveolar capillary hemorrhagic disease characterized by abnormal accumulation of iron in the lungs, mainly in infancy .1990 ~ 1994 years in our hospital admitted to 5 cases of long-term misdiagnosis of idiopathic Sexual pulmonary hemosiderosis, misdiagnosed as tuberculosis for a maximum period of 2 years, the shortest 8 months, anti-tuberculosis treatment is invalid.To improve the diagnosis of the disease early, the report is as follows.1 Clinical data 1.1 General Information The group of 5 patients, 2 males and 3 females, aged 3 to 9 years old.1.2 Symptoms and signs of severe anemia in 3 cases, moderate anemia in 2 cases .5 cases were fever 38 ~ 38.8 ℃, cough, sputum, bloody sputum. 2 cases of shortness of breath, 2 cases of asthma, dyspnea, bruise in 1 case, liver and splenomegaly in 1 case, hepatomegaly in 3 and splenomegaly in 1. Apical and SM Ⅲ / 6 murmur, local non-conduction 1 case .1.3 Laboratory examination Hb 30 ~ 90g / L, the total number of WBC 4 cases in the normal range, 1 case of 12.2 × 10 ~ 9 / L; RBC2 cases of 3.2 × 10-12 / L, 3 cases of 1.2 ~ 2.4 × 10 ~ (12) / L; BPC were within normal values .ESR1 ~ 15mm / h. 1 liver function TTT 12 units, 65 single alanine aminotransferase