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Chronic granulomatous disease (CGD) is an inborn error of immunity caused by a defect in one of the components of the NADPH oxidase complex, which is responsible for generating reactive oxygen species (ROS) during the respiratory burst in phagocytes. The absence of ROS produced by NADPH oxidase in neutrophils and in macrophages leads to greater susceptibility to certain bacterial and fungal infections, and also to inflammatory manifestations due to a deregulated inflammatory response, which suggests that the ability to adequately regulate inflammatory signaling depends on ROS produced by NADPH oxidase. The disease course in patients with X-linked CGD is more severe, with recurrent invasive infections; in contrast, patients with non-classic CGD do not present invasive bacterial or fungal infections, but have more prominent inflammatory manifestations. The most frequent gastrointestinal manifestations are stomatitis, gingivitis, chronic diarrhea, liver abscesses that are similar to inflammatory bowel disease (IBD), and granulomas that can cause obstruction or stenosis in the esophagus, stomach or intestine. It has been observed that the deficiency of p40phox and ROS (non-classic CGD) are associated with greater susceptibility to colitis and the development of severe inflammation; therefore, it is presented that these proteins participate in the resolution of inflammation. In general, the inflammatory findings in CGD, including gastrointestinal manifestations, are seldom described. In international cohorts, manifestations that are similar to IBD are reported in up to 58% of patients with CGD; however, in the only Mexican cohort, its finding is described in only 4 out of 93 patients (4.3%). In this review, we summarize the gastrointestinal clinical findings of CGD, including infectious and inflammatory manifestations, emphasizing on the latter. [ABSTRACT FROM AUTHOR]

A temporary association between SARS-CoV-2 and late inflammatory systemic manifestations in previously healthy pediatric patients has recently been found that could be due to post-viral immunological reactions called Multisystem inflammatory syndrome in children (MIS-C). Descriptive, observational, transversal study it was carried out from May 29 to July 12, 2020 in a second level Hospital in the city of Tijuana, Baja California, Mexico. We included all children and adolescents (aged =18 years) who met the criteria for MIS-C according to the CDC. 6 patients were studied, in 5 (83%) reported exposure to COVID-19 within 4 weeks prior to the onset of symptoms, the median age was 5.3 years, male predominance of 5: 1, all of Hispanic ethnicity, without predominance of the purse group, with an average of 2.8 days of symptoms prior to admission. 100% had a fever of more than 24 hours of evolution, gastrointestinal symptoms 4 (66.6%), abdominal pain, vomiting, diarrhea and nausea, neurological symptoms in 33%, (irritability, seizures and headache). Upon admission, 2 patients presented manifestations similar to Kawasaki, both under 5 years of age. An alteration in the leukocyte count was observed in 100%, lymphopenia in 5 (83%), thrombocytopenia in 3 (50%). 66% required management of intensive pediatric therapy, no patient with respiratory symptoms upon admission but with abnormalities in CT of thorax in 33% (2) of consolidation type during its evolution. RT-PCR was performed in 100%, reported as positive in 5 (83.4%), the determination of IgG and IgM antibodies in 2 patients was positive. Intravenous immunoglobulin and steroid was administered at 6 (100%), 2 (33%) received tocilizumab. Average hospital stay was 6 days, mortality of 50% was observed in the first 24 hours of admission. MIS-C is a new entity related to SARS-CoV-2. That affects previously healthy children, this should be suspected in a feverish child associated with gastrointestinal manifestations, this for early management, especially of multisystemic failure secondary to the large inflammatory process. [ABSTRACT FROM AUTHOR]