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Case Reports
. 2009 Dec 7;14 Suppl 4(Suppl 4):112-5.
doi: 10.1186/2047-783x-14-s4-112.

Idiopathic pulmonary hemosiderosis in a 9-year-old girl

E Kamienska  1 , T Urasinski, A Gawlikowska-Sroka, B Glura, A Pogorzelski
Affiliations
Case Reports

Idiopathic pulmonary hemosiderosis in a 9-year-old girl

E Kamienska et al. Eur J Med Res. .

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.

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Figures

Figure 1
Figure 1
Bronchofiberoscopy showing increased vascularization of bronchial mucosa.
Figure 2
Figure 2
Posteroanterior chest radiography demonstrating pulmonary infiltrations during an acute phase of Idiopathic pulmonary hemosiderosis.
See this image and copyright information in PMC

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