Clinical significance of BPI-ANCA in patients with cystic fibrosis: a single center prospective study.

Recurrent pulmonary exacerbation due to infection and inflammation remain the major cause of mortality and morbidity in patients with cystic fibrosis (CF). Increased levels of BPI-ANCA have been linked to Pseudomonas colonization and pulmonary exacerbations in patients with CF. The majority of these studies were done in Europe, and it is unclear whether similar findings are true in CF patients who lives in United States.

Prevalence of Bactericidal/Permeability-Increasing Protein Autoantibodies in Cystic Fibrosis Patients: Systematic Review and Meta-Analysis.

There have been varying reports on the prevalence of antineutrophil cytoplasmic antibodies with bactericidal/permeability-increasing protein (BPI-ANCA) specificity in cystic fibrosis (CF) patients. These autoantibodies are believed to develop in response to infection and colonization, especially with . The aim of this review was to estimate the overall prevalence of BPI-ANCA in CF patients.

When to suspect atypical cystic fibrosis.

Not all patients with cystic fibrosis have abnormal sweat chloride levels, severe lung disease, or failure to thrive. These 2 cases remind us to think "outside the box".

Treatment of allergic bronchopulmonary aspergillosis (ABPA) in CF with anti-IgE antibody (omalizumab).

Allergic bronchopulmonary aspergillosis (ABPA) results from IgE induced pulmonary response to aspergillus species. Recognition and management of ABPA is challenging in cystic fibrosis (CF) patients because changes in symptoms, lung function and chest radiograph are similar to that seen in CF related pulmonary infection. Standard therapy for ABPA includes systemic steroids and adjunctive use of antifungal agents.

Bronchial anomalies in VACTERL association.

VACTERL association is an acronym made of associated defects including vertebral anomalies, anal atresia, cardiac, tracheal-esophageal fistula, and renal/radial limb anomalies. Tracheal bronchus is a condition characterized by ectopic location of the right upper lobe bronchus at the mid to distal trachea. This condition is associated with congenital anomalies and has been reported in one previous case of VACTERL.