Objective Idiopathic pulmonary hemosiderosis IPH is a rare disease, with unknown etiology and variable outcome, characterized by recurrent episodes of hypochromic anemia, alveolar bleeding and pulmonary infiltrates. Because IPH has a severe outcome, we established a severity score with the purpose of analyzing some of the most important parameters in the progression of an IPH pediatric patient.
Methods We carried out a multiple center retrospective study in five Romanian Pediatric Departments, using cases diagnosed with IPH between 1957 and 2007. We analyzed 8 parameters which were able to influence the prognosis of our patients. The parameters used were age at onset, average delay until diagnosis, hemoglobin level at onset, frequency and severity of crises, presence of both syndromes anemic and respiratory at onset, crises therapy, longterm therapy and survival. For each parameter we assigned a score from 0 to 2.
Results We selected 25 cases diagnosed with IPH. The final score had two stages of severity high, 07 points and moderate, 815 points. Most of our cases received a 7 or less score, hence they were severe. The highest score was 12 points, from a patient who survived 17 years after the onset of the disease. The smallest score was 3, from a patient with severe episodes of anemia.
Conclusion The analysis of these parameters is useful for the assessment of the IPH prognosis.