- Author: Galia D Napchan, MD; Chief Editor: Michael R Bye, MD more...
Further Outpatient Care
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- Periodic evaluation of growth, oxygen saturation, pulmonary and renal function, and hemography and chest radiography findings is appropriate in patients with pulmonary hemosiderosis.
- Long-term steroid use requires frequent monitoring for possible adverse effects.
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- Patients with Heiner syndrome should avoid milk and dairy products.
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- Respiratory failure requiring mechanical ventilatory support may occur after an episode of acute pulmonary hemorrhage.
- Chronic cor pulmonale with pulmonary hypertension secondary to pulmonary fibrosis has been described in a minority of patients with idiopathic pulmonary hemosiderosis (IPH) who have lived for an exceptionally long time after the onset of their disease.
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- The prognosis for the pulmonary hemosiderosis syndromes as a group is difficult to determine because of the infrequency of the diagnosis and the variability among cases and etiologies. Furthermore, no national database monitors children with PH.
- When focusing on idiopathic pulmonary hemosiderosis, the clinical course widely varies; however, the prognosis has always been regarded as poor, with a mean survival of 2.5-3 years after diagnosis. Death can occur acutely from massive hemorrhage or after progressive pulmonary insufficiency and right heart failure. The available therapeutic modalities are not associated with a better outcome.
- One study of 30 children with idiopathic pulmonary hemosiderosis listed the following prognostic criteria:
- The severity of the disease at its onset does not correlate with the survival.
- Females survive longer than males.
- Young age at the onset of disease seems to carry a less favorable prognosis.
- Common therapeutic modalities have not improved outcome.
- Another retrospective study of 15 children with idiopathic pulmonary hemosiderosis found that the presence of antineutrophil cytoplasmic antibodies (ANCA) or other autoantibodies signal poor prognosis. The same study, in which the mean duration of follow-up was 17.2 years (range, 10-36 y), reported a survival rate of 80%
- In pulmonary hemosiderosis associated with milk protein allergy, avoidance of dairy products is usually associated with complete remission.
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