Lung parenchyma surgery in autosomal dominant hyper-IgE syndrome
AF Freeman, ED Renner, C Henderson… - Journal of clinical …, 2013 - Springer
Journal of clinical immunology, 2013•Springer
Abstract Purpose Autosomal dominant hyper-IgE syndrome (AD-HIES) due to heterozygous
STAT3 mutation is a primary immunodeficiency characterized by eczema, elevated serum
IgE, recurrent infections, and connective tissue and skeletal findings. Healing of pneumonias
is often abnormal with formation of pneumatoceles and bronchiectasis. We aimed to explore
whether healing after lung surgery is also aberrant. Methods We retrospectively analyzed
the medical records of 32 patients with AD-HIES who received lung surgery for the …
STAT3 mutation is a primary immunodeficiency characterized by eczema, elevated serum
IgE, recurrent infections, and connective tissue and skeletal findings. Healing of pneumonias
is often abnormal with formation of pneumatoceles and bronchiectasis. We aimed to explore
whether healing after lung surgery is also aberrant. Methods We retrospectively analyzed
the medical records of 32 patients with AD-HIES who received lung surgery for the …
Purpose
Autosomal dominant hyper-IgE syndrome (AD-HIES) due to heterozygous STAT3 mutation is a primary immunodeficiency characterized by eczema, elevated serum IgE, recurrent infections, and connective tissue and skeletal findings. Healing of pneumonias is often abnormal with formation of pneumatoceles and bronchiectasis. We aimed to explore whether healing after lung surgery is also aberrant.
Methods
We retrospectively analyzed the medical records of 32 patients with AD-HIES who received lung surgery for the management of pulmonary infections from 1960 to 2011. We collected information including patient demographics, STAT3 mutation status, clinical history, surgical and medical procedures performed, complications, related medical treatments, and outcomes.
Results
More than 50 % of lung surgeries had associated complications, with the majority being prolonged bronchopleural fistulae. These fistulae often led to empyemas that necessitated additional interventions including prolonged antibiotics, prolonged thoracostomy tube drainage and re-operations.
Conclusion
Lung surgery in AD-HIES patients is associated with high complication rates. STAT3 mutations likely lead to abnormalities in tissue remodelling that are further exacerbated by infection.
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