The etiology, clinical profile, and outcome of diffuse alveolar hemorrhage in children: a ten-year single-center experience

BACKGROUND: Diffuse alveolar hemorrhage (DAH) is a life-threatening syndrome that may be caused by numerous disorders. There is scant data on the etiology and characteristics of DAH in children. METHODS: We retrospectively reviewed the clinical records of patients admitted to a tertiary pediatric hospital with DAH over a 10-year period. The syndrome was classified into five groups according to different etiologies, and the characteristics and outcomes of patients were compared. RESULTS: A total of 74 children were included in the study. Idiopathic pulmonary hemosiderosis (IPH) was the most frequent cause (64.9%), followed by miscellaneous causes (infection and other conditions) (16.2%), immune-mediated disorders (9.5%), liver dysfunction (5.4%), and cardiovascular disorders (4.1%). The median age of the patients was 3.5 years (ranging from 1.5 to 7 years), and no difference was found in the proportion of fever, crackles, and pulmonary infiltrates among the five etiological groups. There was no difference in the proportion of blood transfusions among the groups. Cardiac catheterization was performed on 31 patients for whom the diagnostic workup was negative and were suspected of having IPH, and abnormal signs were observed and bronchial artery embolization (BAE) was performed in all those patients. The patients with IPH had the lowest mortality, while those with DAH secondary to liver failure had the highest mortality. Patients in BAE group had a shorter duration of corticosteroids and a lower relapse rate than non-BAE group. CONCLUSIONS: Idiopathic pulmonary hemosiderosis is a common etiology of DAH and has a good prognosis in children. Vascular abnormalities were observed in the patients with IPH. Further studies are needed to clarify the role of vascular malformations in IPH.