Clinical Study of 28 Cases of Paediatric Idiopathic Pulmonary Haemosiderosis
Identifieur interne : 001646 ( Main/Exploration ); précédent : 001645; suivant : 001647Clinical Study of 28 Cases of Paediatric Idiopathic Pulmonary Haemosiderosis
Auteurs : Xiaobo Zhang [République populaire de Chine] ; Libo Wang [République populaire de Chine] ; Aizhen Lu [République populaire de Chine] ; Mingzhi Zhang [République populaire de Chine]Source :
- Journal of Tropical Pediatrics [ 0142-6338 ] ; 2010.
Abstract
Objective: To summarize the clinical characteristics of idiopathic pulmonary haemosiderosis (IPH) to explore the aetiopathogenesis, risk factors, diagnosis and experiences in therapy of IPH. Methods: The documents of 28 IPH cases, who were hospitalized in Children’s Hospital of Fudan University between February 1989 and June 2009 were reviewed. Results: (i) fifteen cases were males and 13 were females, and 88.5% of the cases had first onset under the age of 10 years; (ii) the triad occurred in 57.1% cases; (iii) radiographic features of IPH including diffuse alveolar-type infiltrates, ground glass attenuation, interstitial reticular and micronodular patterns; (iv) haemosiderin-laden macrophages were found in 60.7% of the cases;(v) the trend of positive correlation was found between the severity of ventilatory restrictive pattern and the disease courses (r = 0.229, p = 0.237); and (vi) glucocorticosteroids can control the symptoms. Conclusion: (i) the clinical presentations are not classical. If long-term anaemia exists without reason, this case must be considered; (ii) corticosteroid can control the symptom; and (iii) IPH may be associated with the imbalance of immune system.
Url:
DOI: 10.1093/tropej/fmq010
Affiliations:
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<front><div type="abstract">Objective: To summarize the clinical characteristics of idiopathic pulmonary haemosiderosis (IPH) to explore the aetiopathogenesis, risk factors, diagnosis and experiences in therapy of IPH. Methods: The documents of 28 IPH cases, who were hospitalized in Children’s Hospital of Fudan University between February 1989 and June 2009 were reviewed. Results: (i) fifteen cases were males and 13 were females, and 88.5% of the cases had first onset under the age of 10 years; (ii) the triad occurred in 57.1% cases; (iii) radiographic features of IPH including diffuse alveolar-type infiltrates, ground glass attenuation, interstitial reticular and micronodular patterns; (iv) haemosiderin-laden macrophages were found in 60.7% of the cases;(v) the trend of positive correlation was found between the severity of ventilatory restrictive pattern and the disease courses (r = 0.229, p = 0.237); and (vi) glucocorticosteroids can control the symptoms. Conclusion: (i) the clinical presentations are not classical. If long-term anaemia exists without reason, this case must be considered; (ii) corticosteroid can control the symptom; and (iii) IPH may be associated with the imbalance of immune system.</div>
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