Kjellman B, Elinder G, Garwicz S, Svan H
Acta Paediatr Scand. 1984 Sep;73(5):584-8. doi: 10.1111/j.1651-2227.1984.tb09978.x.
During the 3 decades 1950-1979 onset of idiopathic pulmonary haemosiderosis occurred in 10 Swedish children. Complete records were available from the eight children with onset during 1960-1979, which indicates that the yearly risk of onset is 0.24 case per million children. The first symptoms started at the mean age of 5.8 years (range: 10 months-11 years). From the beginning all children had a severe microcytic, hypochromic, sideropenic anaemia. Pulmonary symptoms were present from the beginning in four children (but haemoptysis in only one) and developed in the remaining children after 1 1/2-2 1/2 years. Various therapeutical regimens were tried. Iron therapy seemed of temporary beneficial effect and most children seemed to benefit from prednisone therapy during disease bouts, although the effect of the therapy in the long run could not be determined. The four children with onsets during the 1960s died of their disease after 2-13 years. The four children with onset during the 1970s are still alive. One of them--a 20-year-old female, has for two years complete clinical remission and has normalized haematological, X-ray and pulmonary function data.
在1950年至1979年的30年间，10名瑞典儿童患上了特发性肺含铁血黄素沉着症。有8名儿童在1960年至1979年发病，其完整记录表明，每年每百万儿童的发病风险为0.24例。首发症状开始出现的平均年龄为5.8岁（范围：10个月至11岁）。从一开始，所有儿童都患有严重的小细胞低色素性缺铁性贫血。4名儿童从一开始就有肺部症状（但只有1名咯血），其余儿童在1年半至2年半后出现肺部症状。尝试了各种治疗方案。铁剂治疗似乎有暂时的有益效果，大多数儿童在疾病发作期间似乎从泼尼松治疗中获益，尽管从长远来看治疗效果无法确定。20世纪60年代发病的4名儿童在发病2至13年后死于该病。20世纪70年代发病的4名儿童仍然存活。其中一名20岁女性已经临床完全缓解两年，血液学、X线和肺功能数据均已正常。
