Respiratory Syndrome at the Onset of Idiopathic Pulmonary Hemosiderosis in Children

Abstract

Background: Idiopathic pulmonary hemosiderosis (IPH) is a rare, chronic pulmonary disease with etiology still unknown and a dual symptomatology, sanguine and respiratory, that is evolving especially alternative and as a result many error of interpretation can appear.Material and Method: We conducted a retrospective and prospective study, collecting cases of IPH from Romanian Pediatric Clinics, diagnosed between 1958 and 2010.Results: We found 42 cases diagnosed with IPH, although only 4 cases presented predominantly respiratory syndrome at the onset (9,5%), 3 of those being first interpreted as primary pulmonary tuberculosis and treated as such. 17 cases (40,4%) presented both syndromes at the beginning and 21 cases (50%) presented predominantly anemic symptomatology. Dry cough and dyspnea were the principal symptoms of the functional respiratory syndrome in our cases (13 cases, respectively, 12 cases). Dyspnea was associated, more frequently, with severe anemia due to hemosiderin deposition from pulmonary hemorrhages. Haemoptysis was the principal physical respiratory sign and it was present in only 11 cases at the beginning of the disease. Its intensity varied from haemoptoic sputum until normal macroscopic sputum, with positive hemosiderin-laden macrophages at the histological exam. We also had two cases of false hematemesis/occult gastrointestinal hemorrhages which has increased the incidence of haemoptysis in our study at 30,95%.Conclusions: Although IPH is a pulmonary disease, the respiratory syndrome is present on a second level, anemia being the primary symptom in our cases.