Idiopathic pulmonary haemosiderosis is a rare cause of diffuse alveolar haemorrhage of unknown aetiology. It occurs most frequently in children, has a variable natural history with repetitive episodes of diffuse alveolar haemorrhage, and has been reported to have a high mortality. Many patients develop iron deficiency anaemia secondary to deposition of haemosiderin iron in the alveoli. Examination of sputum and bronchoalveolar lavage fluid can disclose haemosiderin-laden alveolar macrophages (siderophages), and the lung biopsy shows numerous siderophages in the alveoli, without any evidence of pulmonary vasculitis, nonspecific/granulomatous inflammation, or deposition of immunoglobulins. Contrary to earlier reports, corticosteroids alone or in combination with other immunosuppressive agents may be effective for either exacerbations or maintenance therapy of idiopathic pulmonary haemosiderosis.

https://erj.ersjournals.com/content/erj/24/1/162.full.pdf

Idiopathic pulmonary haemosiderosis revisited

Utility of early versus late fiberoptic bronchoscopy in the evaluation of new pulmonary infiltrates following hematopoietic stem cell transplantation

The spectrum of pediatric interstitial lung disease (PILD) includes a diverse group of rare disorders characterized by diffuse infiltrates and disordered gas exchange. Children with these conditions typically present with tachypnea, crackles, and hypoxemia. Recent advances have been made in the identification of different types of PILD that are unique to infancy. More exciting has been the discovery of genetic abnormalities of surfactant function, now described in both children and adults. A systematic evaluation of the child presenting with diffuse infiltrates of unknown etiology is essential to the diagnosis. Most often, lung biopsy is required. Current treatment options remain less than satisfactory, and morbidity and mortality remain considerable.

Pediatric interstitial lung disease revisited.

Pulmonary hemorrhage and hemoptysis are uncommon in childhood, and the frequency with which they are encountered by the pediatric pulmonologist depends largely on the special interests of the center to which the child is referred. In those centers caring for children with cystic fibrosis or congenital heart disease, these will be by far the most common causes of hemoptysis. Other causes of hemoptysis are far less common, such as bleeding from localized lesions in the upper airway or tracheobronchial tree. Even less common is bleeding into the lungs as part of a systemic disease, usually with renal involvement (pulmonary-renal syndromes), such as systemic lupus erythematosis or Goodpasture's syndrome. Bleeding into the lungs in children with a bleeding diathesis probably only occurs in immunosuppressed children after transplantation. When no other cause is found for pulmonary hemorrhage, the presumed diagnosis is idiopathic pulmonary hemosiderosis. This review discusses the various causes of hemoptysis and pulmonary hemorrhage, and the appropriate investigations to aid in determining the correct diagnosis. The management and prognosis of idiopathic pulmonary hemosiderosis, based on cumulative experience from published reports, are considered in more detail.

Pulmonary hemorrhage/hemoptysis in children.

Between 1993 and 2000, 30 infants were hospitalized with acute pulmonary hemorrhage at Rainbow Babies and Children's Hospital in Cleveland. Most infants presented with severe pulmonary symptoms requiring intensive support, but a few infants had less severe hemorrhage. Three quarters of the patients required ventilator support and blood transfusions. Eleven patients had transitory hemoglobinuria. Five patients died, but infants who survived did well. There are currently no specific treatment modalities, although we have advised moving to a different home and avoiding environmental tobacco smoke. Subsequently, rebleeding from the lower respiratory tract has decreased from 5 of 7 infants to 1 in 21. On the basis of decreased subsequent fatal hemorrhage, high dose glucocorticoids seem to be of some value. Several patients revealed continued low-grade alveolar hemorrhage for months after their initial bleed, even after removal from their original home environments.

Clinical Profile of 30 Infants With Acute Pulmonary Hemorrhage in Cleveland

Time course of hemosiderin production by alveolar macrophages in a murine model

Meconium aspiration syndrome is a cause of significant morbidity and mortality in the perinatal period and has been implicated in the pathogenesis of airway dysfunction. In this study, we developed a murine model to evaluate the effects of meconium aspiration on airway physiology and lung cellular responses. Under light anesthesia, BALB/c mice received a single intratracheal instillation of meconium or physiological saline. Respiratory mechanics were measured in unrestrained animals and expressed as percent increase in enhanced pause to increasing concentrations of methacholine (MCh). Furthermore, we assessed the changes in cells and cytokines into the bronchoalveolar lavage fluid (BALF). We found meconium aspiration produced increased airway responsiveness to MCh at 7 days. These functional changes were associated with lymphocytic/eosinophilic inflammation, goblet cell metaplasia, and increased concentrations of IL-5 and IL-13 in the BALF. Our findings suggest meconium aspiration leads to alterations of airway function, lung eosinophilia, goblet cell metaplasia, and cytokine imbalance, thus providing the first evidence of meconium-induced airway dysfunction in a mouse model.

Meconium aspiration produces airway hyperresponsiveness and eosinophilic inflammation in a murine model.

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Time course of hemosiderin production by alveolar macrophages in a murine model

Meconium aspiration produces airway hyperresponsiveness and eosinophilic inflammation in a murine model.