PDF(Pubmed)
Abstract：
BACKGROUND: Patients with inborn errors of immunity (IEI) have a compromised or inappropriate immune response. Although they might be considered a high-risk group for severe SARS-CoV-2 infection, the reported impact of COVID-19 in these patients has been reassuring, while the differential susceptibility of distinct types of IEI remains unclear.
OBJECTIVE: We aimed to describe the findings and outcomes of our known patients with IEI who were diagnosed with COVID-19.
METHODS: In a retrospective study from March 2020 to February 2021, four centers in Mexico collected clinical, laboratory, and genetic data from pediatric and adult patients with known diagnoses of IEI who presented with COVID-19, based on compatible symptoms and positive SARS-CoV-2 testing or known household exposure.
RESULTS: We report 31 patients with known IEI from Mexico who presented with SARS-CoV-2 infection. Seventy-four percent were male, 52% were pediatric, and 81% survived. Their ages ranged from 5 months to 56 years, with a median of 17 years. Sixty-five percent had predominant antibody deficiencies, 48% were hospitalized, and 26% required ICU. Pediatric patients had a higher hospital admission rate than adults. Inpatient mortality was 40%, and ICU mortality rate was 63%. Forty-eight percent developed pneumonia, while 36% had evidence of hyperinflammation (4 adults and 7 children). Predominant laboratory features were lymphopenia and thrombocytopenia, seen in 70 and 44% of patients, respectively. The serum D-dimer median value was 2.6 (0.5-20.6) μg/mL, and the median highest ferritin value was 1015 (32-10,303) ng/mL. Intravenous immunoglobulin was used in 80% of patients. Other treatments included macrolides (39%) and corticosteroids (29%). Six patients died from secondary infection or uncontrolled systemic inflammation.
CONCLUSIONS: Although impaired immunity due to IEI may be a predisposing factor for severe COVID-19, most of our patients with IEI who acquired the SARS-CoV-2 infection developed a well-tolerated infection and survived, as have more than 80% of worldwide reported patients to date. An impaired immune or inflammatory response may be a predisposing factor for some and a protective factor for others. A systematic review of the literature could help identify those patients at risk of severe disease and complications. Healthcare-associated infections should be aggressively prevented.
OBJECTIVE: We aimed to describe the findings and outcomes of our known patients with IEI who were diagnosed with COVID-19.
METHODS: In a retrospective study from March 2020 to February 2021, four centers in Mexico collected clinical, laboratory, and genetic data from pediatric and adult patients with known diagnoses of IEI who presented with COVID-19, based on compatible symptoms and positive SARS-CoV-2 testing or known household exposure.
RESULTS: We report 31 patients with known IEI from Mexico who presented with SARS-CoV-2 infection. Seventy-four percent were male, 52% were pediatric, and 81% survived. Their ages ranged from 5 months to 56 years, with a median of 17 years. Sixty-five percent had predominant antibody deficiencies, 48% were hospitalized, and 26% required ICU. Pediatric patients had a higher hospital admission rate than adults. Inpatient mortality was 40%, and ICU mortality rate was 63%. Forty-eight percent developed pneumonia, while 36% had evidence of hyperinflammation (4 adults and 7 children). Predominant laboratory features were lymphopenia and thrombocytopenia, seen in 70 and 44% of patients, respectively. The serum D-dimer median value was 2.6 (0.5-20.6) μg/mL, and the median highest ferritin value was 1015 (32-10,303) ng/mL. Intravenous immunoglobulin was used in 80% of patients. Other treatments included macrolides (39%) and corticosteroids (29%). Six patients died from secondary infection or uncontrolled systemic inflammation.
CONCLUSIONS: Although impaired immunity due to IEI may be a predisposing factor for severe COVID-19, most of our patients with IEI who acquired the SARS-CoV-2 infection developed a well-tolerated infection and survived, as have more than 80% of worldwide reported patients to date. An impaired immune or inflammatory response may be a predisposing factor for some and a protective factor for others. A systematic review of the literature could help identify those patients at risk of severe disease and complications. Healthcare-associated infections should be aggressively prevented.
摘要：
背景：先天性免疫错误（IEI）患者的免疫反应受损或不适当。尽管他们可能被认为是严重SARS-CoV-2感染的高危人群，据报道，COVID-19对这些患者的影响令人放心，而不同类型的IEI的不同易感性仍不清楚。
目的：我们的目的是描述我们已知的被诊断为COVID-19的IEI患者的发现和结果。
方法：在2020年3月至2021年2月的一项回顾性研究中，墨西哥的四个中心收集了临床，实验室,以及根据一致症状和SARS-CoV-2阳性检测或已知家庭暴露，来自已知诊断为IEI的儿童和成人患者的遗传数据，这些患者出现了COVID-19。
结果：我们报告了31名来自墨西哥的已知IEI患者，他们出现了SARS-CoV-2感染。百分之七十四是男性,52%是儿科，81%幸存下来。他们的年龄从5个月到56岁，中位数为17岁。65%的人有明显的抗体缺乏,48%的人住院，26%需要ICU。儿科患者的入院率高于成人。住院患者死亡率为40%，ICU死亡率为63%。48％的人患有肺炎，而36％的人有炎症过度的证据（4名成人和7名儿童）。主要的实验室特征是淋巴细胞减少和血小板减少,在70％和44％的患者中看到，分别。血清D-二聚体中位值为2.6(0.5-20.6)μg/mL,和中位数最高铁蛋白值为1015（32-10,303）ng/mL。80％的患者使用静脉免疫球蛋白。其他治疗包括大环内酯类（39%）和皮质类固醇（29%）。6例患者死于继发感染或全身炎症失控。
结论：尽管IEI导致的免疫力受损可能是严重COVID-19的诱发因素，但我们大多数获得SARS-CoV-2感染的IEI患者都出现了耐受性良好的感染并存活，迄今为止，全球超过80％的患者报告。受损的免疫或炎性反应可能是一些人的诱发因素，而另一些人的保护因素。对文献的系统回顾可以帮助识别那些有严重疾病和并发症风险的患者。应积极预防医疗保健相关感染。
目的：我们的目的是描述我们已知的被诊断为COVID-19的IEI患者的发现和结果。
方法：在2020年3月至2021年2月的一项回顾性研究中，墨西哥的四个中心收集了临床，实验室,以及根据一致症状和SARS-CoV-2阳性检测或已知家庭暴露，来自已知诊断为IEI的儿童和成人患者的遗传数据，这些患者出现了COVID-19。
结果：我们报告了31名来自墨西哥的已知IEI患者，他们出现了SARS-CoV-2感染。百分之七十四是男性,52%是儿科，81%幸存下来。他们的年龄从5个月到56岁，中位数为17岁。65%的人有明显的抗体缺乏,48%的人住院，26%需要ICU。儿科患者的入院率高于成人。住院患者死亡率为40%，ICU死亡率为63%。48％的人患有肺炎，而36％的人有炎症过度的证据（4名成人和7名儿童）。主要的实验室特征是淋巴细胞减少和血小板减少,在70％和44％的患者中看到，分别。血清D-二聚体中位值为2.6(0.5-20.6)μg/mL,和中位数最高铁蛋白值为1015（32-10,303）ng/mL。80％的患者使用静脉免疫球蛋白。其他治疗包括大环内酯类（39%）和皮质类固醇（29%）。6例患者死于继发感染或全身炎症失控。
结论：尽管IEI导致的免疫力受损可能是严重COVID-19的诱发因素，但我们大多数获得SARS-CoV-2感染的IEI患者都出现了耐受性良好的感染并存活，迄今为止，全球超过80％的患者报告。受损的免疫或炎性反应可能是一些人的诱发因素，而另一些人的保护因素。对文献的系统回顾可以帮助识别那些有严重疾病和并发症风险的患者。应积极预防医疗保健相关感染。
