COVID-19 Vaccination in Patients with Inborn Errors of Immunity Reduces Hospitalization and Critical Care Needs Related to COVID-19: a USIDNET Report

Background The CDC and ACIP recommend COVID-19 vaccination for patients with inborn errors of immunity (IEI). Not much is known about vaccine safety in IEI, and whether vaccination attenuates infection severity in IEI. Objective To estimate COVID-19 vaccination safety and examine effect on outcomes in patients with IEI. Methods We built a secure registry database in conjunction with the US Immunodeficiency Network to examine vaccination frequency and indicators of safety and effectiveness in IEI patients. The registry opened on January 1, 2022, and closed on August 19, 2022. Results Physicians entered data on 1245 patients from 24 countries. The most common diagnoses were antibody deficiencies (63.7%). At least one COVID-19 vaccine was administered to 806 patients (64.7%), and 216 patients received vaccination prior to the development of COVID-19. The most common vaccines administered were mRNA-based (84.0%). Seventeen patients were reported to seek outpatient clinic or emergency room care for a vaccine-related complication, and one patient was hospitalized for symptomatic anemia. Eight hundred twenty-three patients (66.1%) experienced COVID-19 infection. Of these, 156 patients required hospitalization (19.0%), 47 required ICU care (5.7%), and 28 died (3.4%). Rates of hospitalization (9.3% versus 24.4%, p < 0.001), ICU admission (2.8% versus 7.6%, p = 0.013), and death (2.3% versus 4.3%, p = 0.202) in patients who had COVID-19 were lower in patients who received vaccination prior to infection. In adjusted logistic regression analysis, not having at least one COVID-19 vaccine significantly increased the odds of hospitalization and ICU admission. Conclusion Vaccination for COVID-19 in the IEI population appears safe and attenuates COVID-19 severity

Comparison of common para-clinical and laboratory methods in Iran: for the diagnosis of Covid-19 disease

Introduction: Coronavirus Disease (COVID-19) caused by Severe Acute RespiratorySyndrome Coronavirus (SARS-CoV-2) was first discovered in China in late 2019 and spreadrapidly worldwide. This study aimed to correlate positive real time Reverse TranscriptasePolymerase Reaction (RT-PCR) results after one month of follow-up with laboratory findingsof the same patients at hospital admission to predict clinical outcome and diagnosis.Materials and Methods: We conducted a retrospective study on the laboratory findings of299 adult patients suspected of COVID-19. Patients were admitted to hospital from March21 to May 25, 2021 with final follow-up of one month for each patient. After one month offollow-up, 233 patients recovered; however, in 64 patients the symptoms worsened. For thesepatients RT-PCR was performed and some patients needed chest Computed Tomography(CT) imaging and were hospitalized. We extracted laboratory findings of these 64 patients andcorrelated the results of their RT-PCR with their laboratory findings.Results: Based on our findings, severe cases are middle-aged adults (P=0.001) withlymphopenia (P<0.001), decreased levels of white blood cells (WBCs) (P<0.001), and platelets(P=0.007) count along with elevated COVID-19 IgG antibody (P=0.002) and ErythrocyteSedimentation Rate (ESR) (P<0.001).Conclusion: RT-PCR is not necessary at admission; instead, some routine hematologyexaminations and serological tests can predict the prognosis of COVID- 19 disease.  Background: Coronavirus disease 2019 (COVID-19), a newly emerging virus, caused by severe acute respiratory syndrome coronavirus (SARS-CoV-2) was first discovered in China in late 2019 and has spread rapidly worldwide. This study aimed to correlate patients with positive real time reverse transcriptase polymerase reaction (RT-PCR) results after one month of follow-up with their laboratory findings at hospital admission to predict clinical outcome and diagnosis. Methods: We performed a retrospective study on the laboratory findings of 299 adult patients suspected with COVID-19. Patients were admitted to hospital from 21 March 2021 to 25 May 2021 with final follow-up of one month for each patient. Results: After one month of follow-up, 233 patients recovered but in 64 patients, the symptoms worsened. For these patients RT-PCR was performed and some patients needed chest computed tomography (CT) imaging and were hospitalized. We extracted laboratory findings of these 64 patients at hospital admission and correlate the results of their RT-PCR with their laboratory findings. Conclusions: Based on our results, severe cases are middle-aged adults (p = 0.001) with lymphopenia (p<0.001) and decreased levels of white blood cells (WBCs) (p<0.001) and platelets (p = 0.007) count along with elevated COVID-19 IgG antibody (p = 0.002) and erythrocyte sedimentation rate (ESR) (p<0.001). It is therefore suggested that RT-PCR is not necessary at admission; instead, some routine hematology examinations and serological tests can predict the prognosis of COVID- 19 disease

JAGN1 mutation with distinct clinical features; two case reports and literature review

Abstract Jagunal homolog 1 (JAGN1) has been recognized as an essential protein in neutrophil function. The mutated JAGN1 is responsible for immunodeficiency related to innate and humoral defense mechanisms. This deficiency impairs neutrophil development and function, leading to recurrent infections and facial dysmorphism as phenotypic consequences of severe congenital neutropenia (SCN). We report two siblings having the reported JAGN1 mutation with different clinical manifestations. Recurrent abscess formation unresponsive to antibiotic therapy, a history of delayed umbilical separation, frequent bacterial or fungal infection, dysmorphic face, failure to thrive, and other coexisting organ abnormalities should prompt physicians to syndromic immunodeficiencies involving neutrophils. Genetic investigations to elucidate the responsible mutation is critical as clinical management varies. Once the diagnosis is confirmed, a multi-disciplinary team should perform further workups to investigate other coexisting malformations and neurodevelopmental evaluation

COVID-19 Vaccination in Patients with Inborn Errors of Immunity Reduces Hospitalization and Critical Care Needs Related to COVID-19: a USIDNET Report

Background: The CDC and ACIP recommend COVID-19 vaccination for patients with inborn errors of immunity (IEI). Not much is known about vaccine safety in IEI, and whether vaccination attenuates infection severity in IEI. Objective: To estimate COVID-19 vaccination safety and examine effect on outcomes in patients with IEI. Methods: We built a secure registry database in conjunction with the US Immunodeficiency Network to examine vaccination frequency and indicators of safety and effectiveness in IEI patients. The registry opened on January 1, 2022, and closed on August 19, 2022. Results: Physicians entered data on 1245 patients from 24 countries. The most common diagnoses were antibody deficiencies (63.7%). At least one COVID-19 vaccine was administered to 806 patients (64.7%), and 216 patients received vaccination prior to the development of COVID-19. The most common vaccines administered were mRNA-based (84.0%). Seventeen patients were reported to seek outpatient clinic or emergency room care for a vaccine-related complication, and one patient was hospitalized for symptomatic anemia. Eight hundred twenty-three patients (66.1%) experienced COVID-19 infection. Of these, 156 patients required hospitalization (19.0%), 47 required ICU care (5.7%), and 28 died (3.4%). Rates of hospitalization (9.3% versus 24.4%, p < 0.001), ICU admission (2.8% versus 7.6%, p = 0.013), and death (2.3% versus 4.3%, p = 0.202) in patients who had COVID-19 were lower in patients who received vaccination prior to infection. In adjusted logistic regression analysis, not having at least one COVID-19 vaccine significantly increased the odds of hospitalization and ICU admission. Conclusion: Vaccination for COVID-19 in the IEI population appears safe and attenuates COVID-19 severity

COVID-19 Vaccination in Patients with Inborn Errors of Immunity Reduces Hospitalization and Critical Care Needs Related to COVID-19: a USIDNET Report

Background: The CDC and ACIP recommend COVID-19 vaccination for patients with inborn errors of immunity (IEI). Not much is known about vaccine safety in IEI, and whether vaccination attenuates infection severity in IEI. Objective: To estimate COVID-19 vaccination safety and examine effect on outcomes in patients with IEI. Methods: We built a secure registry database in conjunction with the US Immunodeficiency Network to examine vaccination frequency and indicators of safety and effectiveness in IEI patients. The registry opened on January 1, 2022, and closed on August 19, 2022. Results: Physicians entered data on 1245 patients from 24 countries. The most common diagnoses were antibody deficiencies (63.7%). At least one COVID-19 vaccine was administered to 806 patients (64.7%), and 216 patients received vaccination prior to the development of COVID-19. The most common vaccines administered were mRNA-based (84.0%). Seventeen patients were reported to seek outpatient clinic or emergency room care for a vaccine-related complication, and one patient was hospitalized for symptomatic anemia. Eight hundred twenty-three patients (66.1%) experienced COVID-19 infection. Of these, 156 patients required hospitalization (19.0%), 47 required ICU care (5.7%), and 28 died (3.4%). Rates of hospitalization (9.3% versus 24.4%, p < 0.001), ICU admission (2.8% versus 7.6%, p = 0.013), and death (2.3% versus 4.3%, p = 0.202) in patients who had COVID-19 were lower in patients who received vaccination prior to infection. In adjusted logistic regression analysis, not having at least one COVID-19 vaccine significantly increased the odds of hospitalization and ICU admission. Conclusion: Vaccination for COVID-19 in the IEI population appears safe and attenuates COVID-19 severity