Clinical and electrocardiographic correlates of myocardial dysfunction after COVID-19 in nonhospitalised patients in long-term follow-up. Data from the polish long-covid cardiovascular study.

Clinical evidence indicates that COVID-19 is a multiorgan disease that significantly impacts the cardiovascular system. However, little is known about the predictors of myocardial dysfunction after SARS-CoV-2 infection. Therefore, this research aimed to evaluate the clinical and electrocardiographic correlates of myocardial dysfunction after SARS-CoV-2 infection in nonhospitalised patients without previously diagnosed cardiovascular disease. This observational study included 448 patients selected from the database of 4142 patients in the Polish Long-Covid Cardiovascular study. All patients underwent a 12-lead electrocardiogram (ECG); 24-h Holter ECG monitoring, 24/7 ambulatory blood pressure monitoring, echocardiography, and cardiac magnetic resonance imaging. According to the results of diagnostic tests, patients were divided into two groups depending on the occurrence of myocardial dysfunction after COVID-19. Group 1-without myocardial dysfunction after COVID-19-consisted of 419 patients, with a mean age of 48.82 (SD ± 11.91), and Group 2 (29 patients)-with myocardial dysfunction after COVID-19, with a mean age of 51.45 (SD ± 12.92). When comparing the analysed groups, there were significantly more men in Group 2 (p = 0.006). QRS (corresponds to the time of ventricular contraction in an electrocardiographic examination) fragmentation (p = 0.031), arrhythmias (atrial fibrillation, supraventricular extrasystole, ventricular extrasystole) (p = 0.008), and male gender (p = 0.007) were independently associated with myocardial dysfunction after COVID-19. The study showed that myocardial damage after COVID-19 affects men more often and is independent of typical clinical factors and the severity of the disease course. The QRS fragmentation and arrhythmias observed in the ECG indicate the possibility of myocardial dysfunction in patients after COVID-19, which may be a valuable marker for physicians.

Assessment of autoantibodies in paediatric population with primary immunodeficiencies: a pilot study.

BACKGROUND: The correlation between primary immunodeficiencies (PIDs) and autoimmunity shows ethnic and geographical diversity. The aim of our study was to accumulate more data in paediatric PID population. METHODS: 58 children aged 1-17 and with PID (study group) and 14 age-matched immunocompetent individuals (control group) were included in the study. Serum levels of 17 different specific IgG antibodies against autoantigens were measured by means of a quantitative enzyme immunoassay. Immunoglobulin levels were analysed in relation to a detailed medical examination. RESULTS: Autoantibodies against one or more antigens were detected in the sera of 24.14% (n = 14) subjects in the study group. The most frequent were anti-thyroid peroxidase (anti-TPO) antibodies (n = 8; 13.8%). Anti-TPO antibody levels were elevated more often in PID patients with a positive family history of autoimmune diseases (p = 0.04). The screening for anti-deamidated gliadin peptide (DGP) and anti-tissue transglutaminase (tTG) antibodies in our series allowed identifying two previously undiagnosed cases of coeliac disease in PID patients. There was no statistically significant difference between the study and the control group in terms of the autoantibodies prevalence. CONCLUSIONS: This study provides data on the prevalence of autoantibodies in paediatric population diagnosed with PID. Selected autoantibodies (i.e. anti-tTG, anti-DGP) might be useful for the screening of PID to avoid the delay of diagnosis of an autoimmune disease.

Do COVID-19 Vaccinations Affect the Most Common Post-COVID Symptoms? Initial Data from the STOP-COVID Register-12-Month Follow-Up.

Around the world, various vaccines have been developed to prevent the SARS-CoV-2 virus infection and consequently the COVID-19 disease. However, many patients continue to report persistent symptoms after the acute phase. Since gathering scientific information on long COVID and post-COVID syndrome has become an urgent issue, we decided to investigate them in relation to the vaccination status of patients from the STOP-COVID registry. In this retrospective study, we analyzed data from the medical visit after contraction of COVID-19 and follow-up visits in the 3rd and 12th month after the disease. In total, 801 patients were included in the analysis. The most frequent complaints after 12 months included deterioration of exercise tolerance (37.5%), fatigue (36.3%), and memory/concentration difficulties (36.3%). In total, 119 patients declared that they had been diagnosed with at least one new chronic disease since the end of isolation, and 10.6% required hospitalization. The analysis of individual symptoms revealed that headache (p = 0.001), arthralgia (p = 0.032), and dysregulation of hypertension (p = 0.030) were more common in unvaccinated patients. Considering headache and muscle pain, people vaccinated after the disease manifested these symptoms less frequently. Subsequent research is needed to consider vaccines as a preventive factor for post-COVID syndrome.

Before and After: Attitude and Adverse Effects Induced by the First and Second Doses of mRNA BNT162b2 Vaccine among Healthcare Professionals in the First Weeks after Their Introduction in Poland.

BACKGROUND: In the last days of December 2020, the SARS-CoV-2 virus vaccine BNT162b2 (Comirnaty, Pfizer-BioNTech) was introduced, for the first time, for wide use in Poland. According to the vaccination schedule, healthcare workers were the first to receive the vaccine. The aim of this study was to analyse the attitudes of those who were determined to be vaccinated, with particular reference to their concerns, attitudes towards vaccination advocacy and sources of knowledge on vaccination, as well as the incidence of adverse reactions. METHODS: The study had a three-stage design. Respondents completed a self-administered questionnaire before receiving the 1st and 2nd vaccine doses and 2 weeks after receiving the 2nd dose. A total of 2247 responses were obtained (1340 responses in the first stage, 769 in the second and 138 in the third). RESULTS: The main source of knowledge on vaccination was the Internet (32%; n = 428). Of the respondents, 6% (n = 86) reported anxiety before the 1st dose of the vaccine, which increased to 20% (n = 157) before the 2nd dose. A declaration of willingness to promote vaccination among their families was made by 87% (n = 1165). Among adverse reactions after the 1st dose of the vaccine, respondents most frequently observed pain at the injection site (n = 584; 71%), fatigue (n = 126; 16%) and malaise (n = 86; 11%). The mean duration of symptoms was 2.38 days (SD 1.88). After the 2nd dose of vaccine, similar adverse reactions-pain at the injection site (n = 103; 75%), fatigue (n = 28; 20%), malaise (n = 22; 16%)-predominated among respondents. Those who declared having had a SARS-CoV-2 virus infection (p = 0.00484) and with a history of adverse vaccination reactions (p = 0.00374) were statistically more likely to observe adverse symptoms after vaccination. CONCLUSIONS: Adverse postvaccinal reactions are relatively common after Comirnaty vaccination but are usually mild and transient in nature. It is in the interest of public health to increase the knowledge of vaccine safety.

COVID-19 in unvaccinated patients with inborn errors of immunity-polish experience.

At the beginning of the Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) pandemic, patients with inborn errors of immunity (IEI) appeared to be particularly vulnerable to a severe course of the disease. It quickly turned out that only some IEI groups are associated with a high risk of severe infection. However, data on the course of Coronavirus Disease 2019 (COVID-19) in patients with IEI are still insufficient, especially in children; hence, further analyses are required. The retrospective study included 155 unvaccinated people with IEI: 105 children and 50 adults (67.7% and 32.3%, respectively). Male patients dominated in the study group (94 people, 60.6%). At least two comorbidities were found in 50 patients (32.3%), significantly more often in adults (56% vs. 21%). Adult patients presented significantly more COVID-19 symptoms. Asymptomatic and mildly symptomatic course of COVID-19 was demonstrated in 74.8% of the entire group, significantly more often in children (88.6% vs. 46%). Moderate and severe courses dominated in adults (54% vs. 11.4%). Systemic antibiotic therapy was used the most frequently, especially in adults (60% vs. 14.3%). COVID-19-specific therapy was used almost exclusively in adults. In the whole group, complications occurred in 14.2% of patients, significantly more often in adults (30% vs. 6.7%). In the pediatric group, there were two cases (1.9%) of multisystem inflammatory syndrome in children. Deaths were reported only in the adult population and accounted for 3.9% of the entire study group. The death rate for all adults was 12%, 15.4% for adults diagnosed with common variable immunodeficiency, 12.5% for those with X-linked agammaglobulinemia, and 21.4% for patients with comorbidity. The results of our study imply that vaccinations against COVID-19 should be recommended both for children and adults with IEI. Postexposure prophylaxis and early antiviral and anti-SARS-CoV-2 antibody-based therapies should be considered in adults with IEI, especially in those with severe humoral immune deficiencies and comorbidity.

Diagnostic Challenges in Patients with Inborn Errors of Immunity with Different Manifestations of Immune Dysregulation.

Inborn errors of immunity (IEI), formerly known as primary immunodeficiency disorders (PIDs), are inherited disorders caused by damaging germline variants in single genes, which result in increased susceptibility to infections and in allergic, autoimmune, autoinflammatory, nonmalignant lymphoproliferative, and neoplastic conditions. Along with well-known warning signs of PID, attention should be paid to signs of immune dysregulation, which seem to be equally important to susceptibility to infection in defining IEI. The modern diagnostics of IEI offer a variety of approaches but with some problems. The aim of this review is to discuss the diagnostic challenges in IEI patients in the context of an immune dysregulation background.

Serum Allergen-Specific IgE among Pediatric Patients with Primary Immunodeficiency.

Background: Allergy is a clinical condition that reflects a deviated function of the immune system. The purpose of this study was to evaluate serum allergen-specific IgE (sIgE) along with clinical manifestations of allergy in patients with diagnosed primary immunodeficiency (PID). Methods: 72 patients, aged 1−17 years, diagnosed with PID and hospitalized between July 2020 and February 2021 were included in the study. Blood samples were obtained by venipuncture. sIgE (30 allergens), blood eosinophil count, as well as total IgE and IgG were measured and assessed in relation to a detailed medical examination. Results: Serum sIgE was detected in the blood of 50% of the patients in the study group, which significantly correlated (p < 0.0001) with clinical symptoms of allergy. During the period of the study, 61.1% of the patients showed symptoms of allergy, with 77.27% of them having tested positive for sIgE. The total IgE level was elevated in 18.06% of the patients and correlated with clinical symptoms of allergy (p = 0.004). An elevated total IgE level was not observed in children receiving immunoglobulin replacement therapy. Conclusion: The study showed that serum sIgE and total IgE together might be a plausible diagnostic tool for PID patients. However, for patients receiving immunoglobulin replacement therapy, the assessment of total IgE is not useful.

Assessment of COVID-19 Incidence and the Ability to Synthesise Anti-SARS-CoV-2 Antibodies of Paediatric Patients with Primary Immunodeficiency.

BACKGROUND: Data regarding the course of SARS-CoV-2 infection in children with primary immunodeficiency (PID) is insufficient. The purpose of the study was to evaluate the morbidity and clinical course of COVID-19 and the ability to produce anti-SARS-CoV-2 IgG antibodies in children with PID. METHODS: In this retrospective study, medical records of 99 patients aged 0-18 were evaluated. The patients were divided into three groups: PID group (68.69%), control group (19.19%) and patients with ongoing or previous paediatric inflammatory multisystem syndrome (12.12%). Data such as morbidity, clinical outcome, and IgG anti-SARS-CoV-2 antibody titres were assessed. RESULTS: A confirmed diagnosis of SARS-CoV-2 infection has been established in 26.47% of patients with PID. Among patients with PID infected with SARS-CoV-2, only three cases were hospitalised. Mortality in the PID group was 0%. Throughout an observation period of 1 year, 47.06% of patients with PID were tested positive for the anti-SARS-CoV-2 antibody. CONCLUSIONS: In the study group, in most cases the disease had a mild and self-limiting course. Remarkably, even though IgG deficiency was the most prevalent form of PID in the study group, the patients were able to respond satisfactorily to the infection in terms of anti-SARS-CoV-2 IgG.

Variants of ATP1A3 in residue 756 cause a separate phenotype of relapsing encephalopathy with cerebellar ataxia (RECA)-Report of two cases and literature review.

BACKGROUND: Variants in ATP1A3 cause well-known phenotypes-alternating hemiplegia of childhood (AHC), rapid-onset dystonia-parkinsonism (RDP), cerebellar ataxia, areflexia, pes cavus, optic atrophy, sensorineural hearing loss (CAPOS), and severe early infantile epileptic encephalopathy. Recently, there has been growing evidence for genotype-phenotype correlations in the ATP1A3 variants, and a separate phenotype associated with variants in residue 756-two acronyms are proposed for the moment-FIPWE (fever-induced paroxysmal weakness and encephalopathy) and RECA (relapsing encephalopathy with cerebellar ataxia). MATERIALS AND METHODS: Herein, we are describing two new pediatric cases with a p.Arg756His change in the ATP1A3 gene. Both patients have had more than one episode of a neurological decompensation triggered by fever with severe hypotonia and followed by ataxia. Thirty-three cases from literature were analyzed to define and strengthen the genotype-phenotype correlation of variants located in residue 756 (p.Arg756His, p.Arg756Cys, p.Arg756Leu). CONCLUSIONS: Patients with a ATP1A3 variant in residue 756 are characterized by recurrent paroxysmal episodes of neurological decompensations triggered by fever, with severe hypotonia, ataxia, dysarthria, symptoms from the orofacial area (dysphagia, drooling) as well as with altered consciousness. Recovery is slow and usually not full with the persistent symptoms of cerebellar ataxia, dysarthria, dystonic and choreiform movements.

Assessment of weight and height of patients with primary immunodeficiency disorders and group of children with recurrent respiratory tract infections.

BACKGROUND: Primary immunodeficiences (PIDs) are a group of chronic, serious disorders in which the immune response is insufficient. In consequence, it leads to an increased susceptibility to infections. Up to date, there are about 350 different disorders classified in that group. There are also patients suffering from recurrent respiratory tract infections (RRTI), however that group doesn't present any abnormalities in terms of conducted immunological tests. Many factors, including medical, can have an impact on physical development of a child. Data such as birth weight and length, also weight, height, BMI during admission to the hospital were collected from 195 patients' medical histories from their hospitalization at Clinical Immunology and Paediatrics Ward of J. Gromkowski Hospital in Wroclaw. Investigated groups included patients with PIDs, RRTI and a control group of healthy children. Our purpose was to evaluate the physical growth of children with PID and children with RRTI by assessment of their height and weight. All of parameters were evaluated using centile charts, suitable best for the Polish population. RESULTS: The lowest mean birth weight and height was found among the PIDs patients group. Children with PIDs during hospitalization had statistically relevant lower mean weight than the control group and almost 18% of them had their height situated below 3rd percentile. The statistically relevant differences have been found between them and RRTI group in terms of weight, height and nutritional status. The statistically significant difference was detected between the nutritional status of PID and control group. CONCLUSIONS: There is a higher percentage of PID patients with physical growth abnormalities in comparison to healthy children. Our findings indicate a need for further investigation of immune system irregularities and their influence on physical growth of children.

Whole-body cryotherapy - promising add-on treatment of depressive disorders. / Krioterapia ogólnoustrojowa ­ obiecujaca forma potencjalizacji leczenia zaburzen depresyjnych.

OBJECTIVES: New, effective biological interventions for treatment of depressive episodes and recurrent depression are still needed. Whole-body cryotherapy (WBC), which is a treatment using cryogenic temperature, is a novel therapeutic modality in neurology and rheumatology. The objective of this study was to determine the efficacy and safety of WBC as an add-on treatment for depressive episode. METHODS: 30 adults diagnosed with depressive episode were recruited to an observational, prospective study. 21 participants (17 women, 81%), mean age 46.1 (±16.7), completed the whole study procedure. The Hamilton Depression Rating Scale and the Beck Depression Inventory were used to assess the severity of depressive symptoms. Additionally, quality of life and anhedonia were assessed with the WHOQoL-BREF and the SHAPS. Participants undertook 10, 2-minute (from - 110 C to - 135 degrees C) WBC sessions within two weeks. RESULTS: Patients after WBC sessions showed significant improvement in the form of a reduction in total scores in scales assessing depressive symptoms: the HDRS (p< 0.00001) between T1 (16.94±4.3) and T4 (4.50±4.2) and the BDI-II (T1: 13.48±4.6; T4: 6.14±6.7, p<0.03), lower anhedonialevelon SHAPS (p =0.011) and higher quality of lifein thefollowing domains: physical health (p =0.024), psychological health (p =0.016) and environmental domain (p =0.003). Pre/post comparison of self-report well-being measured by the VAS scale showed a significant increase (p< 0.00001). It was shown that WBC have no effect on the level of cytokines, NO, hsCRP, ESR and TAS in blood (p> 0.05). CONCLUSIONS: WBC proved to be an effective, safe, and tolerable add-on intervention in patients with depressive episode. Further randomized controlled trials should be conducted.