Lung function in hyper IgE syndrome

摘要

Pneumatoceles and bronchiectasis are secondary complications of respiratory infections in patients with hyper IgE syndrome (HIgES). We report on a patient with HIgES and recurrent pneumonias since 1 year of age, with progression to pneumatoceles and bronchiectasis and fatal outcome, with disseminated infection and suspected allergic bronchopulmonary aspergillosis, who was subjected to sequential spirometric assessment. We describe the early beginning of respiratory infections, difficulties in the management of pulmonary sequelae and the impact of an early functional diagnosis and follow-up of the patient regarding a disease for which lung function has not been fully described.