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Idiopathic pulmonary haemosiderosis: An Oriental experience
Author(s) -
Yao TC,
Hung IJ,
Wong KS,
Huang JL,
Niu CK
Publication year - 2003
Publication title -
journal of paediatrics and child health
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.631
H-Index - 76
eISSN - 1440-1754
pISSN - 1034-4810
DOI - 10.1046/j.1440-1754.2003.00066.x
Subject(s) - medicine , retrospective cohort study , bronchoalveolar lavage , chest radiograph , pulmonary fibrosis , siderosis , pediatrics , medical record , pneumonia , surgery , lung
Objectives: 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. Methods: A retrospective chart review was carried out, collecting medical records of patients with pulmonary haemosiderosis at Chang Gung Children's Hospital (CGCH), a tertiary children's hospital in northern Taiwan. Secondary causes of pulmonary haemosiderosis were excluded. Results: 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. Conclusions: 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.