Idiopathic pulmonary haemosiderosis: An Oriental experience
Identifieur interne : 002100 ( Main/Exploration ); précédent : 002099; suivant : 002101Idiopathic pulmonary haemosiderosis: An Oriental experience
Auteurs : T-C Yao [Taïwan] ; I-J Hung [Taïwan] ; K-S Wong [Taïwan] ; J-L Huang [Taïwan] ; C-K Niu [Taïwan]Source :
- Journal of Paediatrics and Child Health [ 1034-4810 ] ; 2003-01.
English descriptors
- Teeft :
- Abnormality, Acta paediatr, Anaemia, Antineutrophil cytoplasmic antibodies, Blood transfusions, Bronchoalveolar lavage, Cgch, Chang, Chang gung hospital, Chest radiograph, Classical triad, Clinical course, Clinical presentations, Corticosteroid, Corticosteroid therapy, Cows milk protein, Dyspnoea, Early detection, Extrapulmonary diseases, Female fever, Gastric aspirates, Gung, Haemoptysis, Haemorrhage, Haemosiderosis, Idiopathic, Immune disorders, Immunological, Immunological abnormalities, Immunosuppressive, Immunosuppressive therapy, Initial presentation, Insidious onset, Iron deficiency anaemia, Iron supplements, Iron therapy, Macrophage, Milk precipitins, Northern taiwan, Paediatr, Pediatr, Prednisolone, Present study, Pulmonary fibrosis, Pulmonary haemorrhage, Pulmonary haemosiderosis, Pulmonary symptoms, Radiograph, Rast, Tertiary hospital.
Abstract
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.
Url:
DOI: 10.1046/j.1440-1754.2003.00066.x
Affiliations:
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Le document en format XML
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<term>Blood transfusions</term>
<term>Bronchoalveolar lavage</term>
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<term>Chang gung hospital</term>
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<term>Corticosteroid therapy</term>
<term>Cows milk protein</term>
<term>Dyspnoea</term>
<term>Early detection</term>
<term>Extrapulmonary diseases</term>
<term>Female fever</term>
<term>Gastric aspirates</term>
<term>Gung</term>
<term>Haemoptysis</term>
<term>Haemorrhage</term>
<term>Haemosiderosis</term>
<term>Idiopathic</term>
<term>Immune disorders</term>
<term>Immunological</term>
<term>Immunological abnormalities</term>
<term>Immunosuppressive</term>
<term>Immunosuppressive therapy</term>
<term>Initial presentation</term>
<term>Insidious onset</term>
<term>Iron deficiency anaemia</term>
<term>Iron supplements</term>
<term>Iron therapy</term>
<term>Macrophage</term>
<term>Milk precipitins</term>
<term>Northern taiwan</term>
<term>Paediatr</term>
<term>Pediatr</term>
<term>Prednisolone</term>
<term>Present study</term>
<term>Pulmonary fibrosis</term>
<term>Pulmonary haemorrhage</term>
<term>Pulmonary haemosiderosis</term>
<term>Pulmonary symptoms</term>
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<front><div type="abstract" xml:lang="en">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.</div>
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