idiopathic pulmonary hemosiderosis


id·i·o·path·ic pul·mo·nar·y he·mo·sid·er·o·sis

a rare, generally fatal disease of unknown cause, typically affecting small children; characterized by infiltration of pulmonary alveoli with hemosiderin-containing macrophages progressing to diffuse pulmonary fibrosis. Clinical features include recurrent pulmonary hemorrhages, anemia, dyspnea, and cardiorespiratory failure. Synonym(s): Ceelen-Gellerstedt syndrome

idiopathic pulmonary hemosiderosis

Clinical immunology A rare possibly autoimmune condition affecting children < age 10 characterized by hemosiderin deposition in lungs Clinical Recurrent hemoptysis, anemia, weakness, clubbing, hepatosplenomegaly Prognosis Poor, recurrent episodes; death often in 5 yrs due to cor pulmonale; it may be associated with celiac disease and improve with a gluten-free diet. See Hemosiderosis.

Ceelen,

Wilhelm, 1884-1964. Ceelen-Gellerstedt syndrome - repeated sudden attacks of dyspnea and hemoptysis leading to diffuse pulmonary hemosiderosis. Synonym(s): idiopathic pulmonary hemosiderosis