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Diffuse alveolar hemorrhage syndromes in children
Sarat C Susarla1, Leland L Fan
1Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, 1102 Bates Street, Houston, TX 77030, USA. ssusarla@bcm.edu
Insights
Diffuse alveolar hemorrhage in children is increasingly recognized, including pulmonary capillaritis. Diagnosis may require lung biopsy, especially when serology is negative, guiding aggressive treatment for immune-mediated lung disease.
Area of Science:
- Pediatric Pulmonology
- Immunology
- Critical Care Medicine
Background:
- Diffuse alveolar hemorrhage (DAH) is a rare but serious condition in children.
- Traditionally, idiopathic pulmonary hemosiderosis was the primary diagnosis, but the differential diagnosis is expanding.
- Newer understanding includes immune-mediated causes like pulmonary capillaritis.
Purpose of the Study:
- To review evolving concepts of DAH in children.
- To discuss an expanded differential diagnosis for DAH.
- To outline new diagnostic and treatment strategies for pediatric DAH.
Main Methods:
- Review of current literature on pediatric diffuse alveolar hemorrhage.
- Analysis of recent case reports and studies, including pediatric pulmonary capillaritis.
- Discussion of diagnostic modalities and therapeutic approaches.
Main Results:
- Pulmonary capillaritis, an immune-mediated DAH, is being increasingly reported in children.
- Serological markers for immune-mediated disorders were positive in only half of pediatric pulmonary capillaritis cases.
- Acute idiopathic pulmonary hemorrhage in infants remains of uncertain etiology.
Conclusions:
- The classification of DAH in children now includes conditions with and without pulmonary capillaritis.
- Lung biopsy is crucial for diagnosing DAH in children, particularly when cardiovascular causes are excluded and serology is negative.
- Immune-mediated lung diseases necessitate more aggressive pharmacologic interventions.
Purpose Of Review:
In this review we discuss the changing concepts of diffuse alveolar hemorrhage in children in terms of an expanded differential diagnosis and new approaches to diagnosis and treatment.
Recent Findings:
More commonly found in adults, pulmonary capillaritis, an immune-mediated form of diffuse alveolar hemorrhage often associated with systemic disease, has been recently reported in children. In a series of eight children with pulmonary capillaritis, serology for immune-mediated disorders was positive in only half. Acute idiopathic pulmonary hemorrhage is a unique condition of infants who present with acute pulmonary hemorrhage and respiratory failure. The hypothesis that acute idiopathic pulmonary hemorrhage is caused by toxigenic mold has not been proven, and its cause remains uncertain.
Summary:
Classification of diffuse alveolar hemorrhage in children has been revised to include those conditions with and without pulmonary capillaritis. As idiopathic pulmonary hemosiderosis, the classic form of diffuse alveolar hemorrhage in children, is a diagnosis of exclusion and children with pulmonary capillaritis may have negative serology, lung biopsy should be strongly considered in any child with diffuse alveolar hemorrhage without a cardiovascular cause. Generally, patients with immune-mediated lung disease require more aggressive pharmacologic intervention.
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