Chronic Granulomatous Disease in an Adult Recognized by an Invasive Aspergillosis

Kaufmann, Inès; Briegel, Josef; Heide, Verena van der; Choukèr, Alexander; Frey, Lorenz; Spiekermann, Karsten; Mayr, Doris; Thiel, Manfred

doi:10.1097/maj.0b013e31823484de

Cited by 6 publications

(3 citation statements)

References 14 publications

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“…Isolated PMNs (∼1 × 10 5 ) were incubated with dihydrorhodamine 123 (DHR123) (1 μM) (MoBiTec GmbH, Goettingen, Germany) in 0.5 mL HBSS buffer to detect the production of ROS as previously described (Kaufmann et al, 2012). After priming of the cells with TNF (10 ng/mL) for 5 min, PMNs were stimulated with fMLP (10 -7 M) for another 5 min.…”

Section: Methodsmentioning

confidence: 99%

Stress Related Shift Toward Inflammaging in Cosmonauts After Long-Duration Space Flight

Buchheim¹,

Matzel²,

Рыкова

et al. 2019

Front. Physiol.

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Space flight exerts a specific conglomerate of stressors on humans that can modulate the immune system. The mechanism remains to be elucidated and the consequences for cosmonauts in the long term are unclear. Most of the current research stems from short-term spaceflights as well as pre- and post-flight analyses due to operational limitations. Immune function of 12 cosmonauts participating in a long-duration (>140 days) spaceflight mission was monitored pre-, post-, and on two time-points in-flight. While the classical markers for stress such as cortisol in saliva where not significantly altered, blood concentrations of the endocannabinoid system (ECS) were found to be highly increased in-flight indicating a biological stress response. Moreover, subjects showed a significant rise in white blood cell counts. Neutrophils, monocytes and B cells increased by 50% whereas NK cells dropped by nearly 60% shortly after landing. Analysis of blood smears showed that lymphocyte percentages, though unchanged pre- and post-flight were elevated in-flight. Functional tests on the ground revealed stable cellular glutathione levels, unaltered baseline and stimulated ROS release in neutrophils but an increased shedding of L-selectin post-flight. In vitro stimulation of whole blood samples with fungal antigen showed a highly amplified TNF and IL-1β response. Furthermore, a significant reduction in CD4 + CD25 + CD27 low regulatory T cells was observed post-flight but returned to normal levels after one month. Concomitantly, high in-flight levels of regulatory cytokines TGF-β, IL-10 and IL-1ra dropped rapidly after return to Earth. Finally, we observed a shift in the CD8 + T cell repertoire toward CD8 + memory cells that lasted even one month after return to Earth. Conclusion: Long-duration spaceflight triggered a sustained stress dependent release of endocannabinoids combined with an aberrant immune activation mimicking features of people at risk for inflammation related diseases. These effects persisted in part 30 days after return to Earth. The currently available repertoire of in-flight testing as well as the post-flight observation periods need to be expanded to tackle the underlying mechanism for and consequences of these immune changes in order to develop corresponding mitigation strategies based on a personalized approach for future interplanetary space explorations.

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Section: Methodsmentioning

confidence: 99%

Stress Related Shift Toward Inflammaging in Cosmonauts After Long-Duration Space Flight

Buchheim¹,

Matzel²,

Рыкова

et al. 2019

Front. Physiol.

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“…Infection with Aspergillus fumigatus occurs in a majority of patients; C albicans is another prominent fungal pathogen. [535][536][537] A colitis similar to Crohn disease occurs in about 17% of patients. Granulomatous inflammation can lead to obstruction of the stomach, ureter, or esophagus in some patients.…”

Section: Defects Of the Respiratory Burst Chronic Granulomatous Disementioning

confidence: 99%

Practice parameter for the diagnosis and management of primary immunodeficiency

Bernstein¹,

Blessing-Moore²,

Khan³

et al. 2015

Journal of Allergy and Clinical Immunology

589

494

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The American Academy of Allergy, Asthma & Immunology (AAAAI) and the American College of Allergy, Asthma & Immunology (ACAAI) have jointly accepted responsibility for establishing the "Practice parameter for the diagnosis and management of primary immunodeficiency." This is a complete and comprehensive document at the current time. The medical environment is a changing environment, and not all recommendations will be appropriate for all patients. Because this document incorporated the efforts of many participants, no single individual, including those who served on the Joint Task Force, is authorized to provide an official AAAAI or ACAAI interpretation of these practice parameters. Any request for information about or an interpretation of these practice parameters by the AAAAI or ACAAI should be directed to the Executive Offices of the AAAAI, the ACAAI, and the Joint Council of Allergy, Asthma & Immunology. These parameters are not designed for use by pharmaceutical companies in drug promotion.

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“…4 There are several reports on the late onset of CGD. [5][6][7] Recurrent and intractable bacterial or fungal infections led to the suspicion of CGD in these patients. Although CGD usually occurs in children, this disorder should be considered for adults with recurrent and intractable infections.…”

Section: To the Editormentioning

confidence: 99%