Idiopathic pulmonary haemosiderosis in Swedish children

Abstract

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.