Clinical study of 28 cases of paediatric idiopathic pulmonary haemosiderosis.

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.