RESUMEN
Idiopathic pulmonary hemosiderosis (IPH), a rare disease of unknown etiology, is characterized by iron deficiency anemia and chronic recurrent pulmonary symptoms such as cough, hemoptysis, and dyspnea. Recurrent intra-alveolar hemorrhage resulting in hemosiderin accumulation and progressive fibrosis often leads to death. We experienced a case of IPH in a two-year-old male who was presented with cough, dyspnea, hemoptysis and anemia. The diagnosis was confirmed by hemosiderin-laden macrophages in a gastric aspirate. He was initially treated with oral iron and prednisolone for one year. But pulmonary symptoms such as cough and hemoptysis recurred five times while he was on oral prednisolone and the side effects of moon face and truncal obesity developed. So inhaled steroid (budesonide) was administered for 18 months, with improvement of pulmonary symptoms. This suggests that early treatment with inhaled steroids may work in IPH without the serious side effects of systemic steroid. The effect of inhaled steroid should be evaluated as the initial treatment of IPH.
Asunto(s)
Niño , Humanos , Masculino , Anemia , Anemia Ferropénica , Tos , Diagnóstico , Disnea , Fibrosis , Hemoptisis , Hemorragia , Hemosiderina , Hemosiderosis , Inhalación , Hierro , Macrófagos , Obesidad , Prednisolona , Enfermedades Raras , EsteroidesRESUMEN
Idiopathic pulmonary hemosiderosis (IPH), a rare disease of unknown etiology, is characterized by iron deficiency anemia and chronic recurrent pulmonary symptoms such as cough, hemoptysis, and dyspnea. Recurrent intra-alveolar hemorrhage resulting in hemosiderin accumulation and progressive fibrosis often leads to death. We experienced a case of IPH in a two-year-old male who was presented with cough, dyspnea, hemoptysis and anemia. The diagnosis was confirmed by hemosiderin-laden macrophages in a gastric aspirate. He was initially treated with oral iron and prednisolone for one year. But pulmonary symptoms such as cough and hemoptysis recurred five times while he was on oral prednisolone and the side effects of moon face and truncal obesity developed. So inhaled steroid (budesonide) was administered for 18 months, with improvement of pulmonary symptoms. This suggests that early treatment with inhaled steroids may work in IPH without the serious side effects of systemic steroid. The effect of inhaled steroid should be evaluated as the initial treatment of IPH.