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Chronic Granulomatous Disease - Europe PMC

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Last Updated: 20 July 2022

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

Tuberculosis is endemic in China, and the Bacillus Callmette-Gu00fcerin vaccine is administered to all of the infants, potentially contributing to BCG infection in patients with chronic granulomatous disease. BCG/TB infections in CGD patients can be lethal, and pneumonia is the most affected organ. In CGD, the aim was to determine the diagnosis of pulmonary BCG/TB infection. We screened 169 CGD patients and identified patients with pulmonary BCG/TB infection. BCG infection diagnosis was determined by the vaccination history, local infection diagnosis, acid-fast bacilli staining, specific polymerase chain reaction, and/or spoligotyping. BCG disease was identified earlier than TB. The first and most common feature among those patients with BCG was lymphadenopathy. The pulmonary infections were more severe in TB patients, with massive masses, acute lymphadenopathy, and extensive pulmonary fibrosis. BCG-in CGD patients were more common than TB, but less severe.

Source link: https://europepmc.org/article/MED/35817807


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

Purpose In a Chinese cohort, we aimed to reveal the clinical and immunological characteristics of variant type X91+ chronic granulomatous disease. X91 + CGD patients' clinical emergences and immunological phenotypes were found. Expression of Gp91 phox protein expression was determined using extracellular staining with the monoclonal antibody 7D5 and flow cytometry. Patients with CGD with X91+ CGD accounted for 8% of all patients with CGD. CGD patients with X91 + CGD had the median age of onset in the seven patients with X91+ CGD. The BCG vaccine was given to Six patients, and 50% of them had probable BCG infections. The stimulation index of patients with X91 + CGD varied widely from 1. 9 to 67. 3. CYBB mutations associated with X91 + CGD were most prevalent in second transmembrane or intracellular locations. Conclusions Variant type X91 + CGD can cause a variety of clinical signs.

Source link: https://europepmc.org/article/MED/35796921


Clinical features and outcomes of patients with chronic granulomatous disease in Taiwan.

Chronic granulomatous disease is a rare primary immunologic disease caused by impaired neutrophil killing of microbial pathogens and recurrent infections in chronic granulomatous disease. Pediatric patients with CGD from a medical center in Taiwan were admitted from January 1999 to Oct 2021, according to a study. CGD was published in nine pediatric patients with CGD with CYBB gene mutations, three autosomal recessive CGD with two NCF1 and one CYBA gene mutation were among nine X-linked CGD patients with two NCF1 and one CYBB gene mutation. Patients with XL-CGD had a younger age of onset and age of diagnosis than AR-CGD patients. At 2. 2 years ago, one male patient with XL-CGD was successfully treated with hematopoietic stem cell transplantation. Conclusion In Taiwanese CGD patients, staphylococcus aureus, Serratia marcescens, and Salmonella infections are all important. Early disease onset is noted among patients with XL-CGD. Prophylaxis and prophylactic anti-microbial drugs may have a role in reducing CGD patients' infection episodes.

Source link: https://europepmc.org/article/MED/35842407

* 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