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Chronic Granulomatous Disease - Springer Nature

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Last Updated: 20 July 2022

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Treatment with Polyethylene Glycol–Conjugated Fungal d-Amino Acid Oxidase Reduces Lung Inflammation in a Mouse Model of Chronic Granulomatous Disease

Chronic granulomatous disease is a primary immunodeficiency in which phagocytes are unable to produce reactive oxygen species owing to a defect in the nicotinamide phosphate oxidase complex. Moreover, antibiotic therapy may not help in reducing excessive inflammation due to the soluble presence of fungal cell wall u03b2-glucan. We used an in vitro non-viable Candida albicans gp91-phox knockout CGD mice in this research, as well as new Fusarium spp. Three experimental methods with the in vivo lung inflammation model were used to determine the reliability of the ROS-producing enzyme replacement therapy with PEG-fDAO. Although a more precise method is preferable, these results show that PEG-fDAO can be used to treat inflammation in CGD in vivo.

Source link: https://doi.org/10.1007/s10753-022-01650-z


Imaging characteristics of pulmonary BCG/TB infection in patients with chronic granulomatous disease

Tuberculosis is endemic in China, and the Bacillus Callmette Gutefcerin vaccine is administered to all infants, potentially resulting in BCG infection in patients with persistent granulomatous disease. BCG/TB infection in CGD patients can be lethal, and pneumonia is the most affected organ. We wanted to know the imaging of pulmonary BCG/TB infection in CGD. We screened 169 CGD patients and identified the patients with pulmonary BCG/TB infection. According to the vaccination history, local infection outbreak, acid-fast bacilli staining, specific polymerase chain reaction, and/or spoligotyping, the diagnosis of BCG infection was confirmed. BCG disease diagnosis was much earlier than TB. For those patients who had only BCG, lymphadenopathy was the first and most common feature. The pulmonary infections in TB patients were more severe, with large masses, acute lymphadenopathy, and extensive pulmonary fibrosis. BCG pneumonia infections were more prevalent than TB in CGD patients, but not as severe.

Source link: https://doi.org/10.1038/s41598-022-16021-9


Variant Type X91^+ Chronic Granulomatous Disease: Clinical and Molecular Characterization in a Chinese Cohort

Purpose In a Chinese cohort, we investigated the clinical and immunological characteristics of variant type X91+ chronic granulomatous disease. Methods were established The clinical manifestations and immunological phenotypes of patients with X91+ CGD were gathered. The expression of Gp91'phox protein was determined by extracellular staining with the monoclonal antibody 7D5 and flow cytometry. Patients with CGD with X91+ CGD accounted for 8 percent of all patients with CGD. CGD in the seven patients with X91+ CGD was four months old. Six patients were vaccinated with the BCG vaccine, and 50% of them had suspected BCG infections. Mycobacterium tuberculosis infection was prevalent in this area. Recurrent oral ulcers in two patients. The stimulation index of the patients with X91+ CGD ranged from 1. 9 to 67. 3. CYBB mutations associated with X91+ CGD were most prevalent in the second transmembrane or intracellular regions. Conclusions Variant type X91+ CGD may lead to a variety of clinical manifestations.

Source link: https://doi.org/10.1007/s10875-022-01324-3

* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions

* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions