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Clinical study of 28 cases of paediatric idiopathic pulmonary haemosiderosis
Xiaobo Zhang1, Libo Wang, Aizhen Lu
1Respiratory Department, Children's Hospital of Fudan University, Shanghai, China.
Insights
Idiopathic pulmonary haemosiderosis (IPH) often presents atypically, particularly in children. Glucocorticosteroids can manage symptoms, and IPH may involve immune system imbalances.
Area of Science:
- Pediatric Pulmonology
- Rare Diseases
- Immunology
Background:
- Idiopathic pulmonary haemosiderosis (IPH) is a rare condition characterized by recurrent pulmonary hemorrhage.
- Understanding its clinical spectrum is crucial for timely diagnosis and management.
Purpose of the Study:
- To summarize the clinical characteristics of IPH.
- To explore its aetiopathogenesis, risk factors, diagnosis, and therapeutic approaches.
Main Methods:
- Retrospective review of 28 IPH cases hospitalized between February 1989 and June 2009.
- Analysis of clinical presentations, radiographic findings, and treatment responses.
Main Results:
- Most cases (88.5%) presented before age 10; the classic triad occurred in 57.1%.
- Radiographic findings included alveolar infiltrates and interstitial patterns; haemosiderin-laden macrophages were common (60.7%).
- Glucocorticosteroids demonstrated efficacy in symptom control.
Conclusions:
- IPH may present non-classically, necessitating consideration in cases of unexplained chronic anemia.
- Corticosteroid therapy can effectively manage IPH symptoms.
- Immune system dysregulation may play a role in IPH aetiopathogenesis.
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.
