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Characterization of Lung Disease in Autosomal Dominant Hyper IgE Syndrome
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A4797 - Characterization of Lung Disease in Autosomal Dominant Hyper IgE Syndrome
Author Block: K. Bogdanovski1, A. K. Urban2, D. Darnell3, J. Peterson2, P. Welch2, A. F. Freeman3, K. N. Olivier1; 1Laboratory of Chronic Airway Infection, The National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, United States, 2Clinical Research Directorate/Clinical Monitoring Program, Leidos Biomedical Research, Inc., NCI Campus at Frederick, Frederick, MD, United States, 3Laboratory of Clinical Immunology and Microbiology, The National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, United States.
Rationale: Autosomal Dominant Hyper IgE Syndrome (AD-HIES: STAT 3 deficiency) is a primary immunodeficiency characterized by high serum IgE, eczema, connective tissue and vascular abnormalities, and recurrent sinopulmonary infections. Pulmonary complications have been the main source of hospitalizations and mortality. This study reports the pulmonary profile of patients diagnosed with AD-HIES that are followed at the National Institutes of Health.
Methods: A cross-sectional assessment of lung disease in AD-HIES patients was performed for this study. Data were reviewed from the clinical history of 144 patients followed at the NIH in an IRB-approved natural history study to examine pulmonary function, frequency of pulmonary complications through radiographic reports (bronchiectasis, pneumatoceles/cavities, or no remarkable infections), and frequency of characteristic micro-organisms (Pseudomonas aeruginosa, Staphylococcus aureus, nontuberculous mycobacteria, and Aspergillus species) within sputum and bronchoalveolar lavage samples. The data were abstracted from the most recent reports and samples detailed within the NIH clinical database.
Results: The FEV1 % predicted used to categorize the severity of obstructive lung disease, indicated that 48% were normal, 20% were mild, 7% were moderate, 10% were moderate severe, 7.5% were severe, and 7% were very severe. Chest CT scan reports demonstrated that 67% had some degree of bronchiectasis, 27% had pneumatoceles/cavities, and 19% had no remarkable radiographic findings. The sputum and bronchoalveolar lavage samples yielded positive cultures for P. aeruginosa (30%), S. aureus (27%), nontuberculous mycobacteria (12%), and Aspergillus (35%).
Conclusions: Among patients diagnosed with AD-HIES, 12% had no evidence of lung disease (normal FEV1 % predicted, normal CT, no reported pathogens). These data suggest that the vast majority of AD-HIES patients experience some pulmonary complication or infection that can lead to increased hospitalization. The susceptibility of AD-HIES patients to these pulmonary events suggests more aggressive treatment and preventative measures should be taken to minimize the recurrence of severe pulmonary outcomes.
Author Block: K. Bogdanovski1, A. K. Urban2, D. Darnell3, J. Peterson2, P. Welch2, A. F. Freeman3, K. N. Olivier1; 1Laboratory of Chronic Airway Infection, The National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, United States, 2Clinical Research Directorate/Clinical Monitoring Program, Leidos Biomedical Research, Inc., NCI Campus at Frederick, Frederick, MD, United States, 3Laboratory of Clinical Immunology and Microbiology, The National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, United States.
Rationale: Autosomal Dominant Hyper IgE Syndrome (AD-HIES: STAT 3 deficiency) is a primary immunodeficiency characterized by high serum IgE, eczema, connective tissue and vascular abnormalities, and recurrent sinopulmonary infections. Pulmonary complications have been the main source of hospitalizations and mortality. This study reports the pulmonary profile of patients diagnosed with AD-HIES that are followed at the National Institutes of Health.
Methods: A cross-sectional assessment of lung disease in AD-HIES patients was performed for this study. Data were reviewed from the clinical history of 144 patients followed at the NIH in an IRB-approved natural history study to examine pulmonary function, frequency of pulmonary complications through radiographic reports (bronchiectasis, pneumatoceles/cavities, or no remarkable infections), and frequency of characteristic micro-organisms (Pseudomonas aeruginosa, Staphylococcus aureus, nontuberculous mycobacteria, and Aspergillus species) within sputum and bronchoalveolar lavage samples. The data were abstracted from the most recent reports and samples detailed within the NIH clinical database.
Results: The FEV1 % predicted used to categorize the severity of obstructive lung disease, indicated that 48% were normal, 20% were mild, 7% were moderate, 10% were moderate severe, 7.5% were severe, and 7% were very severe. Chest CT scan reports demonstrated that 67% had some degree of bronchiectasis, 27% had pneumatoceles/cavities, and 19% had no remarkable radiographic findings. The sputum and bronchoalveolar lavage samples yielded positive cultures for P. aeruginosa (30%), S. aureus (27%), nontuberculous mycobacteria (12%), and Aspergillus (35%).
Conclusions: Among patients diagnosed with AD-HIES, 12% had no evidence of lung disease (normal FEV1 % predicted, normal CT, no reported pathogens). These data suggest that the vast majority of AD-HIES patients experience some pulmonary complication or infection that can lead to increased hospitalization. The susceptibility of AD-HIES patients to these pulmonary events suggests more aggressive treatment and preventative measures should be taken to minimize the recurrence of severe pulmonary outcomes.
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