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- Title
Idiopathic pulmonary haemosiderosis: an Oriental experience.
- Authors
Yao, T-C; Hung, I-J; Wong, K-S; Huang, J-L; Niu, C-K
- Abstract
<bold>Objectives: </bold>Idiopathic pulmonary haemosiderosis (IPH) is a rare but potentially lethal disorder. A retrospective analysis of documented cases of IPH in our hospital was conducted in order to study the clinical spectrum and radiographic features, and to explore therapeutic strategies.<bold>Methods: </bold>A retrospective chart review was carried out, collecting medical records of patients with pulmonary haemo-siderosis at Chang Gung Children's Hospital (CGCH), a tertiary children's hospital in northern Taiwan. Secondary causes of pulmonary haemosiderosis were excluded.<bold>Results: </bold>Five patients were diagnosed as having IPH over a 25-year period. The classical triad of IPH was found at initial presentation in only 2/5 patients. One patient had well-established pulmonary fibrosis, but no pulmonary symptoms. The clinical course of IPH was exceedingly variable, with a mean delay of 9 months before diagnosis was made. Bronchoalveolar lavage (BAL) confirmed IPH in 3/5 patients. Immunological abnormalities were noted in two patients, without progression to immune disorders during follow up. While using corticosteroids alone, 4/5 patients continued to have recurrent bleeding episodes. All five patients required immunosuppressive therapy for maintenance of a symptom-free period, but survived to a mean follow up of 2 years.<bold>Conclusions: </bold>Early definitive diagnosis and aggressive immunosuppressive therapy of IPH are imperative in order to avoid pulmonary fibrosis and mortality in IPH. A chest radiograph should be included in a serial work-up of unexplained anaemia in children. An examination using BAL can confirm IPH and high-resolution thoracic computed tomography scans are useful for early detection of pulmonary fibrosis.
- Subjects
HEMOSIDEROSIS; IRON metabolism disorders
- Publication
Journal of Paediatrics & Child Health, 2003, Vol 39, Issue 1, p27
- ISSN
1034-4810
- Publication type
journal article
- DOI
10.1046/j.1440-1754.2003.00066.x