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.
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.
Authors: Anna Stainer; Alex Rice; Anand Devaraj; Joseph Luke Barnett; Jacqueline Donovan; Maria Kokosi; Andrew Gordon Nicholson; Tom Cairns; Athol Umfrey Wells; Elisabetta Augusta Renzoni Journal: BMC Pulm Med Date: 2019-10-24 Impact factor: 3.320
Authors: Chana I C Chin; Shirleen Loloyan Kohn; Thomas G Keens; Monique F Margetis; Roberta M Kato Journal: Orphanet J Rare Dis Date: 2015-08-20 Impact factor: 4.303