Prognosis in Pediatric Idiopathic Pulmonary Hemosiderosis

Unexplained childhood anaemia: idiopathic pulmonary hemosiderosis

“…However, aggressive treatment with the use of corticosteroids and immunosuppressive agents are associated with a prolonged survival and improved prognosis. 12 Long-term low-dose corticosteroid therapy was also reported to result in a milder disease course and prevent bleeding crisis. 13 In conclusion, iron-deficiency anaemia results from poor dietary intake of iron in infants and toddlers.…”

◆ Idiopathic Pulmonary Hemosiderosis

“…Primary Pulmonary hemosiderosis is described as encompassing the diagnosis of IPH, Goodpaster syndrome (anti basement membrane antibody disease), and Heiners syndrome (Cow"s milk protein hyper reactivity). Among these, Goodpaster syndrome is the most common cause of pulmonary hemorrhage [5]. Secondary Pulmonary hemosiderosis includes diverse group of etiologies.…”

“…Other non specific pathological findings can include thickening of alveolar septae and hypertrophy of type II pneumocytes. Fibrosis may be seen with chronic disease [2,5,17] Pulmonary hemosiderosis is associated with decreased hemoglobin and hematocrit. The classical finding is a microcytic, hypochromic anemia.…”

“…The reticulocyte count is elevated. The anemia of IPH can mimic a hemolytic anemia [5]. Serum iron is reduced, iron binding capacity is generally elevated.…”

“…A subgroup of patients may not respond optimally to corticosteroid therapy alone. In these cases immunosuppressive agents such as methotrexate, cyclophosphamide, azathioprine and chloroquine have been used with variable results [1,5,18]. Kabra SK et al [1] have shown that inhaled corticosteroid used during the steroid tapering stage after the acute phase, had a positive effect on improvement in the outcome of IPH cases.…”