2013
DOI: 10.1586/eci.12.98
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Pulmonary manifestations of chronic granulomatous disease

Abstract: Chronic granulomatous disease (CGD) is an inherited disorder, characterized by defects in superoxide-generating NADPH oxidase of phagocytes. The genetic defects in CGD induce failure to activate the respiratory burst in the phagocytes, leading to severe recurrent infections and unexplained prolonged inflammatory reactions that may produce granulomatous lesions. A noble advance in curative therapy for CGD is hematopoietic stem cell transplantation. Since the most common site of involvement in CGD is the lung, t… Show more

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Cited by 51 publications
(42 citation statements)
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“…In a USA cohort, Aspergillus species were the most common isolated pathogens, in both pneumonia (41%) and lung abscesses (23%) [2]. Other less frequent infectious manifestations are pleural effusion, bronchiectasis, bronchitis, atelectasis, and mediastinal and hilar lymphadenopathy [10]. Chronic lung findings of Case  1 were related to recurrent and inadequately treated infections leading to his last admission with severe pneumothorax.…”
Section: Discussionmentioning
confidence: 99%
See 1 more Smart Citation
“…In a USA cohort, Aspergillus species were the most common isolated pathogens, in both pneumonia (41%) and lung abscesses (23%) [2]. Other less frequent infectious manifestations are pleural effusion, bronchiectasis, bronchitis, atelectasis, and mediastinal and hilar lymphadenopathy [10]. Chronic lung findings of Case  1 were related to recurrent and inadequately treated infections leading to his last admission with severe pneumothorax.…”
Section: Discussionmentioning
confidence: 99%
“…Inflammatory pulmonary manifestations such as granuloma formation, pneumonitis, and fibrosis may be seen in CGD patients [10]. Pulmonary granulomatous disease is diagnosed histologically with a negative culture and may be confused with mycobacterial infection [3].…”
Section: Discussionmentioning
confidence: 99%
“…This leads to severe recurrent infections and unexplained prolonged inflammatory reactions that may produce granulomatous lesions mainly in the lung. The pulmonologists (pediatrics or adult) need to be able to recognize different lung manifestations of CGD for a best diagnosis [22]. …”
Section: Discussionmentioning
confidence: 99%
“…CT findings include consolidation, ground-glass opacities, tree-inbud pattern, centrilobular or random nodules, bronchiectasis, septal thickening, air trapping, or scarring. Complications such as abscess formation, empyema, or lung fibrosis may also be found and are quite unique to CGD where contiguous spread to the chest wall and associated osteomyelitis of ribs and vertebrae may occur (Towbin and Chaves 2010;Mahdaviani et al 2013). …”
Section: Discussionmentioning
confidence: 99%