Prognostic criteria in idiopathic pulmonary hemosiderosis in children.

An epidemiologic survey was undertaken of 30 children in whom idiopathic pulmonary hemosiderosis (IPH) had been identified. In determining the prognostic significance of various parameters, a clinical severity score was used. This included the year of onset of IPH, fever, difficulty in breathing, the severity of the anemia, the roentgenographic findings and therapeutic modalities such as transfusions, administration of steroids, antibiotic treatment and splenectomy. Eighteen patients had died. The results of this investigation suggest that: (1) The severity of the disease at its onset does not determine the survival; (2) Females survived longer; (3) Young age of the patients at the onset of IPH seems to carry a less favorable prognosis; (4) The common therapeutic modalities in use have not proved to be beneficial. We believe that some environmental insult to the developing lung in genetically predisposed persons may contribute to a higher morbidity in the younger patients.