The clinical manifestations present in the affected patients are not severe. This syndrome is compatible with life, and it seems to be less aggressive than the HLA class I deficiencies. The patient had repeated bacterial infections, such as bronchitis with productive cough and otitis media from the age of five. Chest x-ray and computed tomography (CT) revealed disseminated bronchiectases. Sputum culture was positive for Haemophilus influenzae. Functional respiratory tests revealed severe mixed ventilatory disturbance. Clinical status improved after intravenous antibiotic therapy. He has required further admissions because of respiratory reinfections.
Diagnosis
Diagnostic recommendations
Additional Information
Therapeutic options
- Use of antibiotic and intravenous immunoglobulins.