Title |
Idiopathic pulmonary hemosiderosis in a 9-year-old girl
|
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Published in |
European Journal of Medical Research, December 2009
|
DOI | 10.1186/2047-783x-14-s4-112 |
Pubmed ID | |
Authors |
E Kamienska, T Urasinski, A Gawlikowska-Sroka, B Glura, A Pogorzelski |
Abstract |
Diffuse alveolar hemorrhage (DAH) is a rare and life-threatening condition characterized by hemoptysis, dyspnoea, alveolar infiltrates on chest radiograph and various degrees of anemia. It may occur either as a primary disease of the lungs or a secondary condition due to cardiac, systemic vascular, collagen or renal diseases. Idiopathic pulmonary hemosiderosis (IPH) is a separate form of DAH of unknown origin, associated in some cases with celiac disease. The estimated incidence of IPH in children is 0.24-1.23 cases per million, with a mortality rate as high as 50%. Only about 500 cases of this disease have been described in medical literature. We present a case of a 9-year-old girl diagnosed with IPH, which was confirmed by the presence of many hemosiderin-laden macrophages in bronchoalveolar lavage obtained by bronchofiberoscopy. Therapy with glucocorticoids was initiated with a partial and transient response. Azathioprine and a gluten-free diet were subsequently introduced. However, the girl still suffers from recurrent episodes of hemoptysis, dyspnea and anemia. |
Mendeley readers
Geographical breakdown
Country | Count | As % |
---|---|---|
Unknown | 19 | 100% |
Demographic breakdown
Readers by professional status | Count | As % |
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Researcher | 2 | 11% |
Professor > Associate Professor | 2 | 11% |
Other | 2 | 11% |
Student > Master | 2 | 11% |
Student > Doctoral Student | 1 | 5% |
Other | 5 | 26% |
Unknown | 5 | 26% |
Readers by discipline | Count | As % |
---|---|---|
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Unspecified | 1 | 5% |
Agricultural and Biological Sciences | 1 | 5% |
Biochemistry, Genetics and Molecular Biology | 1 | 5% |
Unknown | 5 | 26% |