Pulmonary hemosiderosis in children with Down syndrome: a national experience
2018
Pulmonary
hemosiderosisis a rare and complex disease in children. A previous study from the French RespiRare® network led to two important findings: 20% of the children presented with both pulmonary
hemosiderosisand Down syndrome (DS), and at least one tested autoantibody was found positive in 50%. This study investigates the relationships between pulmonary
hemosiderosisand DS. Patients younger than 20 years old and followed for pulmonary
hemosiderosiswere retrieved from the RespiRare® database. Clinical, biological, functional, and radiological findings were collected, and DS and non-DS patients’ data were compared. A total of 34 patients (22 girls and 12 boys) were included, among whom nine (26%) presented with DS. The mean age at diagnosis was 4.1 ± 3.27 years old for non-DS and 2.9 ± 3.45 years old for DS patients. DS patients tended to present a more severe form of the disease with an earlier onset, more dyspnoea at diagnosis, more frequent
secondary pulmonary hypertension, and an increased risk of fatal evolution. DS patients have a higher risk of developing pulmonary
hemosiderosis, and the disease seems to be more severe in this population. This could be due to the combination of an abnormal lung capillary bed with fragile vessels, a higher susceptibility to autoimmune lesions, and a higher risk of evolution toward pulmonary hypertension. A better screening for pulmonary
hemosiderosisand a better prevention of hypoxia in DS paediatric patients may prevent a severe evolution of the disease.
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