Background.  Unexplained pulmonary hemorrhage and hemosiderosis are rarely seen in infancy. A geographic cluster of 10 infants with this illness was identified in a large pediatric referral hospital in Cleveland, Ohio, during the period of January 1993 through December 1994. One infant died of severe respiratory failure. Methods.  A case-control study was conducted. Three control infants were matched by age with each case infant. All study infants’ guardians were interviewed. Questions were asked about child care practices and home conditions for the period before case infants’ illnesses. All infants’ records were reviewed, their homes were visited, and a structural and environmental survey was conducted. Results.  All 10 case infants were black, and 9 were male, whereas 50.0% of control infants were male, and 83.3% were black. The case infants’ mean age was 10.2 weeks (range, 6 weeks to 6 months). Matched analysis demonstrated that case infants’ homes were more likely to have had water damage preceding the pulmonary hemorrhage event (odds ratio, 16.25; 95% confidence interval, 2.55 to ∞). Case infants were also more likely to have had close relatives with pulmonary hemorrhage (odds ratio, 33.14; 95% confidence interval, 5.10 to ∞). In addition, 50.0% of case infants experienced recurrent pulmonary hemorrhaging after returning to their homes. Conclusion.  The results of this investigation of a cluster of infants with massive, acute pulmonary hemorrhage and hemosiderosis suggest that the affected infants may have been exposed to contaminants in their homes. Epidemiologic clues, such as water damage in the case infants’ homes, suggest that environmental risk factors may contribute to pulmonary hemorrhage. pulmonary hemorrhage, hemosiderosis, infancy, indoor air pollution, pesticides, volatile organic compounds, cholinesterase.

https://pediatrics.aappublications.org/content/pediatrics/99/1/e5.full.pdf

Environmental Risk Factors Associated With Pediatric Idiopathic Pulmonary Hemorrhage and Hemosiderosis in a Cleveland Community

Immune Alveolar Hemorrhage

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 enviromental insult to the developing lung in genetically predisposed persons may contribute to a higher morbidity in the younger patients.

Prognostic criteria in idiopathic pulmonary hemosiderosis in children.

A Few Good Men : A Marine With Hemoptysis and Diarrhea

Idiopathic pulmonary hemosiderosis (IPH) is an extremely rare cause of massive pulmonary hemorrhage in children. During the acute phase, death due to massive alveolar hemorrhage and subsequent severe respiratory failure. We report two cases of IPH children who developed hypoxemic respiratory failure and massive pulmonary hemorrhage. One case of a 10-year-old boy was treated with methylprednisolone pulse therapy (10mg/kg/d) for the first three days and followed by systemic steroid therapy, he successfully decannulated 10days later and discharged with a favorable quality of life. Another case of a 4year-old female child with Down's syndrome diagnosed as IPH for over one year and treated with oral corticosteroids for maintenance therapy. She sudden suffered severe hypoxemia with rapid falls in the hemoglobin level. We applied methylprednisolone pulse therapy (10mg/kg/d) for three days and other supportive therapies, the girl survived through complicated with oxygen dependence. We suggest that methylprednisolone pulse therapy provides a chance of recovery and survival for patients with IPH at the acute phase, even if accompanied by severe pulmonary hemorrhage.

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Idiopathic pulmonary hemosiderosis