Idiopathic pulmonary haemosiderosis in paediatric patients: How to make an early diagnosis

Luca Castellazzi, Maria Francesca Patria, Gemma Frati, Andrea Alessandro Esposito, Susanna Esposito

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

Background: Idiopathic pulmonary haemosiderosis (IPH) is a rare but potentially lethal condition in paediatric patients. This condition is considered an immune-mediated disorder, but its pathogenesis is still unknown. Idiopathic pulmonary haemosiderosis is characterized by the classical triad of haemoptysis, iron-deficiency anaemia, and diffuse parenchymal consolidation on chest radiology. Unfortunately, this triad of signs is not frequent in children at the onset of this disease, resulting in a delay in diagnosis and a negative outcome. Case presentation: This case report describes a 4-year-old girl who was admitted for an acute episode of lower respiratory tract infection associated with severe dyspnoea, polypnoea, and severe anaemia (haemoglobin levels, 5.9 g/dL). She had a history of previous similar episodes, with anaemia treated unsuccessfully with iron supplementation and managed through repeated blood transfusions in the acute phase. She did not experience haemoptysis. A computed tomography (CT) scan of the thorax showed ground-glass opacity suggestive of pulmonary haemorrhage. After other causes of intra-alveolar haemorrhage were excluded, IPH was confirmed by the presence of siderophages in bronchoalveolar lavage. Immunosuppressive corticosteroid treatment was immediately started with a good clinical response. Conclusion: This case highlights the fact that IPH should be suspected in children with recurrent lower respiratory tract infections who have a history of iron-deficiency anaemia who shows no signs of improvement with iron supplementation and may require repeated blood transfusions. The absence of haemoptysis does not exclude the diagnosis of IPH in children. An early and prompt diagnosis is recommended in order to start adequate immunosuppressive treatment.

Original languageEnglish
Article number86
JournalItalian Journal of Pediatrics
Volume42
Issue number1
DOIs
Publication statusPublished - Sep 20 2016

Fingerprint

Early Diagnosis
Hemoptysis
Pediatrics
Iron-Deficiency Anemias
Immunosuppressive Agents
Blood Transfusion
Respiratory Tract Infections
Anemia
Thorax
Iron
Hemorrhage
Immune System Diseases
Bronchoalveolar Lavage
Radiology
Dyspnea
Glass
Adrenal Cortex Hormones
Hemoglobins
Tomography
Idiopathic pulmonary hemosiderosis

Keywords

  • Haemoptysis
  • Idiopathic pulmonary haemosiderosis
  • Lower respiratory tract infections
  • Severe anaemia

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Idiopathic pulmonary haemosiderosis in paediatric patients : How to make an early diagnosis. / Castellazzi, Luca; Patria, Maria Francesca; Frati, Gemma; Esposito, Andrea Alessandro; Esposito, Susanna.

In: Italian Journal of Pediatrics, Vol. 42, No. 1, 86, 20.09.2016.

Research output: Contribution to journalArticle

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abstract = "Background: Idiopathic pulmonary haemosiderosis (IPH) is a rare but potentially lethal condition in paediatric patients. This condition is considered an immune-mediated disorder, but its pathogenesis is still unknown. Idiopathic pulmonary haemosiderosis is characterized by the classical triad of haemoptysis, iron-deficiency anaemia, and diffuse parenchymal consolidation on chest radiology. Unfortunately, this triad of signs is not frequent in children at the onset of this disease, resulting in a delay in diagnosis and a negative outcome. Case presentation: This case report describes a 4-year-old girl who was admitted for an acute episode of lower respiratory tract infection associated with severe dyspnoea, polypnoea, and severe anaemia (haemoglobin levels, 5.9 g/dL). She had a history of previous similar episodes, with anaemia treated unsuccessfully with iron supplementation and managed through repeated blood transfusions in the acute phase. She did not experience haemoptysis. A computed tomography (CT) scan of the thorax showed ground-glass opacity suggestive of pulmonary haemorrhage. After other causes of intra-alveolar haemorrhage were excluded, IPH was confirmed by the presence of siderophages in bronchoalveolar lavage. Immunosuppressive corticosteroid treatment was immediately started with a good clinical response. Conclusion: This case highlights the fact that IPH should be suspected in children with recurrent lower respiratory tract infections who have a history of iron-deficiency anaemia who shows no signs of improvement with iron supplementation and may require repeated blood transfusions. The absence of haemoptysis does not exclude the diagnosis of IPH in children. An early and prompt diagnosis is recommended in order to start adequate immunosuppressive treatment.",
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