Idiopathic pulmonary hemosiderosis in a 9-year-old girl.
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.
Department of Normal and Clinical Anatomy, Pomeranian Medical University, Szczecin, Poland. Ekamienska@poczta.onet.pl
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E
Kamienska
T
T
Urasinski
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A
Gawlikowska-Sroka
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B
Glura
A
A
Pogorzelski
D002648:Child; D005260:Female; D006486:Hemosiderosis / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology; D006801:Humans; D008171:Lung Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology