Question:

Which of the following is false, regarding idiopathic pulmonary haemosiderosis (IPH)?
1. IPH is a rare disease.
2. IPH is more common in adults.
3. IPH has a similar sex distribution in children.
4. IPH is characterized by accumulation of haemosiderin in the lungs.
5. The aetiology of IPH is unknown.





Answer:

The correct answer for the question "Which of the following is false, regarding idiopathic pulmonary haemosiderosis (IPH)?" is:

2. IPH is more common in adults.



Explanation
a) IPH is a rare disease [IPH is considered to be rare disorder with an incidence of 0.24 and 1.23 cases per million in selected populations].

b) IPH is more common in children [80% of cases occur in children with balanced sex distribution in childhood].

c) IPH has a similar sex distribution in children [80% of cases occur in children with balanced sex distribution in childhood and a slight male predominance in adults].

d) It is characterized by accumulation of haemosiderin in the lungs [IPH is characterised by abnormal accumulation of iron as haemosiderin in alveolar macrophages due to repeated episodes of intra-alveolar haemorrhage].

e) IPH is of unknown aetiology [The aetiology of the condition is unknown and can be lethal if left untreated].



From the manuscript:
Case Report of Idiopathic Pulmonary Haemosiderosis in a Child with recurrent chest infections
Radiology Case. 2011 Sep; 5(9):30-35


This article belongs to the Pediatric section.




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From the manuscript

Case Report of Idiopathic Pulmonary Haemosiderosis in a Child with recurrent chest infections

Free full text article: Case Report of Idiopathic Pulmonary Haemosiderosis in a Child with recurrent chest infections

Abstract
Idiopathic pulmonary haemosiderosis (IPH) is a rare condition that usually presents as a triad of haemoptysis, iron deficiency anaemia and pulmonary infiltrates. We report a case of IPH diagnosed in a 7 year old boy who had recurrent hospital admissions with severe chest infections and haemoptysis from his first few months of life. He was found to have microcytic hypochromic anaemia, diffuse infiltrate shadowing on his chest X-ray (CXR) and ground-glass opacification on his computed tomogram (CT). Perl`s Prussian blue staining of his bronchoalveolar lavage fluid revealed haemosiderin-laden macrophage infiltration. After exclusion of infective, cardiac, immunological and glomerular causes, he was diagnosed with idiopathic pulmonary haemosiderosis. He has since been treated intermittently with steroids, which have failed to control his symptoms fully.






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