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先天性免疫缺陷患者 121 例 SARS-CoV-2 感染的结局:一项横断面研究。

Outcome of SARS-CoV-2 Infection in 121 Patients with Inborn Errors of Immunity: A Cross-Sectional Study.

机构信息

IPPMG-Universidade Federal Do Rio de Janeiro (UFRJ), Rua Professor Luis Cantanhede, 77 ap 101, Rio de Janeiro, RJ, 22245-040, Brazil.

IPPMG-Universidade Federal Do Rio de Janeiro (UFRJ), Rio de Janeiro, RJ, Brazil.

出版信息

J Clin Immunol. 2021 Oct;41(7):1479-1489. doi: 10.1007/s10875-021-01066-8. Epub 2021 Jun 23.

Abstract

PURPOSE

There is still scarce data on SARS-CoV-2 infection in patients with Inborn Errors of Immunity (IEI) and many unresolved questions. We aimed to describe the clinical outcome of SARS-CoV-2 infection in Brazilian IEI patients and identify factors influencing the infection.

METHODS

We did a cross-sectional, multicenter study that included patients of any age affected by IEI and SARS-CoV-2 infection. The variables studied were sex, age, type of IEI, comorbidities (number and type), treatment in use for IEI, clinical manifestations and severity of SARS-CoV-2 infection.

RESULTS

121 patients were included: 55.4% female, ages from six months to 74 yo (median age = 25.1 yo). Most patients had predominantly antibody deficiency (n = 53). The infection was mostly asymptomatic (n = 21) and mild (n = 66), and one child had multisystem inflammatory syndrome (MIS-C). We could not observe sex-related susceptibility, and there was a weak correlation between age and severity of infection. The number of comorbidities was higher in severe cases, particularly bronchiectasis and cardiopathy. There were no severe cases in hereditary angioedema patients. Six patients aged 2 to 74 years died, three of them with antibody deficiency.

CONCLUSION

The outcome was mild in most patients, but the Case Fatality Ratio was higher than in the general population. However, the type of IEI was not a determining factor for severity, except for complement deficiencies linked to milder COVID-19. The severity of SARS-CoV-2 infection seems to be more related to older age, a higher number of comorbidities and type of comorbidities (bronchiectasis and cardiopathy).

摘要

目的

关于先天免疫缺陷(IEI)患者的 SARS-CoV-2 感染仍缺乏数据,存在许多悬而未决的问题。我们旨在描述巴西 IEI 患者 SARS-CoV-2 感染的临床结果,并确定影响感染的因素。

方法

我们进行了一项横断面、多中心研究,纳入了任何年龄患有 IEI 和 SARS-CoV-2 感染的患者。研究的变量包括性别、年龄、IEI 类型、合并症(数量和类型)、用于 IEI 的治疗、SARS-CoV-2 感染的临床表现和严重程度。

结果

共纳入 121 例患者:55.4%为女性,年龄从 6 个月至 74 岁(中位数年龄=25.1 岁)。大多数患者主要为抗体缺陷(n=53)。感染大多为无症状(n=21)和轻度(n=66),一名儿童患有多系统炎症综合征(MIS-C)。我们未观察到与性别相关的易感性,年龄与感染严重程度之间存在弱相关性。严重病例的合并症数量较高,特别是支气管扩张症和心脏病。遗传性血管性水肿患者无严重病例。6 名 2 至 74 岁的患者死亡,其中 3 名为抗体缺陷患者。

结论

大多数患者的结果为轻度,但病死率高于一般人群。然而,IEI 类型不是严重程度的决定因素,除了与 COVID-19 轻度相关的补体缺陷。SARS-CoV-2 感染的严重程度似乎与年龄较大、合并症数量较多以及合并症类型(支气管扩张症和心脏病)有关。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/69db/8221557/a9a350e260c4/10875_2021_1066_Fig1_HTML.jpg

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