Mechanisms underlying genetic susceptibility to multisystem inflammatory syndrome in children (MIS-C)

Chou, Janet; Platt, Craig D.; Habiballah, Saddiq; Nguyen, Andrew V.; Elkins, Megan; Weeks, Sabrina; Peters, Zachary; Day‐Lewis, Megan; Novak, Tanya; Armant, Myriam; Williams, Lucinda; Rockowitz, Shira; Sliz, Piotr; Williams, David A.; Randolph, Adrienne G.; Geha, Raif S.; Almutairi, Abduarahman; Jaber, Faris; Banzon, Tina; Roberts, Jay; Halyabar, Olha; Lo, Mindy S.; Kahn, Stacy A.; Henderson, Lauren A.; Lee, Pui Y.; Son, Mary Beth; Cheng, Leah

doi:10.1016/j.jaci.2021.06.024

Cited by 102 publications

(135 citation statements)

References 30 publications

(36 reference statements)

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“…Although the present patient had no past medical history and was otherwise considered a healthy adult, he was overweight (BMI 26.3 kg/m 2 ), and obesity has been associated with severe COVID-19 [ 29 , 30 ]. In addition, obesity also contributes to increased host meta-inflammation, and the adipocytes are known to express inflammatory cytokines such as interleukin 6, tumor necrosis factor-alpha, and acute phase reactants such as CRP [ 31 ].…”

Section: Discussionmentioning

confidence: 99%

Multisystem Inflammatory Syndrome Associated with SARS-CoV-2 Infection in an Adult: A Case Report from the Maldives

et al. 2021

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The multisystem inflammatory syndrome in adults (MIS-A) is a novel syndrome observed during COVID-19 outbreaks. This hyper-inflammatory syndrome is seen predominantly in children and adolescents. The case of an adult from the Maldives who had asymptomatic SARS-CoV-2 infection three weeks before presenting to the hospital with fever, rash, and shock is presented. De-identified clinical data were retrospectively collected to summarize the clinical progression and treatment during hospitalization and the six-month follow-up. SARS-CoV-2 infection was confirmed by RT-PCR. Other laboratory findings included anemia (hemoglobin: 9.8 g/dL), leukocytosis (leukocytes: 20,900/µL), neutrophilia (neutrophils: 18,580/µL) and lymphopenia (lymphocytes: 5067/µL), and elevated inflammatory markers, including C-reactive protein (34.8 mg/dL) and ferritin (2716.0 ng/dL). The electrocardiogram had low-voltage complexes, and the echocardiogram showed hypokinesia, ventricular dysfunction, and a pericardial effusion suggestive of myocardial dysfunction compromising hemodynamics and causing circulatory shock. These findings fulfilled the diagnostic criteria of MIS-A. The case was managed in the intensive care unit and required non-invasive positive pressure ventilation, inotropes, and steroids. With the new surges of COVID-19 cases, more cases of MIS-A that require the management of organ failure and long-term follow-up to recovery are anticipated. Clinicians should therefore be vigilant in identifying cases of MIS-A during the pandemic.

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Section: Discussionmentioning

confidence: 99%

Multisystem Inflammatory Syndrome Associated with SARS-CoV-2 Infection in an Adult: A Case Report from the Maldives

et al. 2021

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“…As for immune dysregulation disorders, COVID-19 has been reported in 62 patients (10%), the majority of whom were affected by autoimmune polyendocrine syndrome type 1 (APS-1, n = 27, 44%) [ 3 , 4 , 5 ▪ , 10 , 13 , 14 , 16 , 21 , 22 , 37 , 40 , 56 , 57 , 70 ]. The rate of ICU admission was 28% and CFR 15% ( n = 9).…”

Section: Overview Of Reports Of Coronavirus Disease 2019 In Patients With Inborn Errors Of Immunitymentioning

confidence: 99%

“…Eleven patients have CTLA4 haploinsufficiency or lipopolysaccharide-responsive and beige-like anchor protein (LRBA) deficiency (18%) and two succumbed (CFR 18%) [ 5 ▪ , 14 – 16 , 56 ]. Three patients had XIAP mutations, and 2/2 of these patients died post-HSCT [ 4 , 5 ▪ , 10 ].…”

Section: Overview Of Reports Of Coronavirus Disease 2019 In Patients With Inborn Errors Of Immunitymentioning

confidence: 99%

“…Despite shielding and being prioritized in vaccination campaigns in many countries, many patients with previously known inborn errors of immunity (IEIs) became infected with SARS-CoV-2 and hundreds of these cases have now been reported in the scientific literature. Moreover, a number of reports validated the hypothesis that individuals who became severely ill with SARS-CoV-2 – a primary infection for both children and adults – could suffer from a hitherto unknown defined error of immunity [ 1 ▪▪ , 2 ▪▪ , 3 , 4 , 5 ▪ , 6 ▪▪ , 7 ▪ , 8 ▪▪ ]. Based on these studies, a picture of the impact of COVID-19 in IEI is emerging.…”

Section: Introductionmentioning

confidence: 99%

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Coronavirus disease 2019 in patients with inborn errors of immunity: lessons learned

Bucciol

Tangye

Meyts

2021

Current Opinion in Pediatrics

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Purpose of review The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic has caused extreme concern for patients with inborn errors of immunity (IEIs). In the first 6 months of the pandemic, the case fatality rate among patients with IEIs resembled that of the general population (9%). This review aims at summarizing what we have learned about the course and outcome of coronavirus disease 2019 (COVID-19) in patients with different IEIs and what this can potentially teach us about the immune mechanisms that could confer protection or predisposition to severe disease. Recent findings A total of 649 patients with IEI and COVID-19 have been reported in the last year and a half, spanning all groups of the International Union of Immunological Societies classification of IEIs. For most patients, the underlying IEI does not represent an independent risk factor for severe COVID-19. In fact, some IEI may even be protective against the severe disease due to impaired inflammation resulting in less immune-mediated collateral tissue damage. Summary We review the characteristics of SARS-CoV-2 infection in a large number of patients with IEI. Overall, we found that combined immunodeficiencies, immune dysregulation disorders, and innate immune defects impairing type I interferon responses are associated with severe disease course.

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“… 137 T‐box transcription factor 21, a central coordinator of exhausted CD8+ T cell differentiation, as well as suppressor of cytokine signaling 1 (SOCS1), a negative regulator of type I and II interferons, may be the promising therapeutic target candidate for MIS‐C. 167 , 168 People recovered from COVID‐19 may develop posttraumatic stress disorder. 169 Augmented T helper 17 cell differentiation and cytokine response might be partially responsible COVID‐19‐associated central nervous system dysregulation, including Guillain‐Barré syndrome.…”

Section: Application Of Transcriptomics In Covid‐19 Pandemicmentioning

confidence: 99%

Application of omics technology to combat the COVID‐19 pandemic

Yang

Yan²,

Zhong³

2021

MedComm

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As of August 27, 2021, the ongoing pandemic of coronavirus disease 2019 (COVID‐19), caused by severe acute respiratory syndrome coronavirus 2 (SARS‐CoV‐2), has spread to over 220 countries, areas, and territories. Thus far, 214,468,601 confirmed cases, including 4,470,969 deaths, have been reported to the World Health Organization. To combat the COVID‐19 pandemic, multiomics‐based strategies, including genomics, transcriptomics, proteomics, and metabolomics, have been used to study the diagnosis methods, pathogenesis, prognosis, and potential drug targets of COVID‐19. In order to help researchers and clinicians to keep up with the knowledge of COVID‐19, we summarized the most recent progresses reported in omics‐based research papers. This review discusses omics‐based approaches for studying COVID‐19, summarizing newly emerged SARS‐CoV‐2 variants as well as potential diagnostic methods, risk factors, and pathological features of COVID‐19. This review can help researchers and clinicians gain insight into COVID‐19 features, providing direction for future drug development and guidance for clinical treatment, so that patients can receive appropriate treatment as soon as possible to reduce the risk of disease progression.

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Mechanisms underlying genetic susceptibility to multisystem inflammatory syndrome in children (MIS-C)

Cited by 102 publications

References 30 publications

Multisystem Inflammatory Syndrome Associated with SARS-CoV-2 Infection in an Adult: A Case Report from the Maldives

Multisystem Inflammatory Syndrome Associated with SARS-CoV-2 Infection in an Adult: A Case Report from the Maldives

Coronavirus disease 2019 in patients with inborn errors of immunity: lessons learned

Application of omics technology to combat the COVID‐19 pandemic

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