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Multisystem inflammatory syndrome in children (MIS-C) is a disease that made its mark in the early days of the COVID-19 pandemic due to the diverse course and symptoms affecting multiple body systems. It is a condition that develops in pediatric patients about 2-6 weeks after contact with a person infected with the SARS-CoV-2 virus. In many instances, MIS-C has caused multiple organ failure, with particularly severe complications involving the cardiovascular system and manifesting as hypotension, various cardiac arrhythmias, myocarditis or coronary artery lesions resembling those seen in Kawasaki disease. Currently, the incidence of MIS-C is about 1-3 per 1000 children, with a decreasing trend in recent years due to the introduction of immunization against the SARS-CoV-2 virus for children as young as 6 months. In our paper, we present the case of a patient with a severe course of MIS-C with numerous cardiovascular and neurological complications, in whom the symptoms of the disease were managed by administering biological treatment. We also present a review of the literature on the subject, which shows how many different facets this disease can have and that physicians still need to remain alert, as there are cases of severe MIS-C, especially in unvaccinated patients.
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Background: an accurate assessment of the immunity against SARS-CoV-2 can facilitate a better understanding and management of not only the recent coronavirus but similar pathogens as well. Objective: the aim of this study was to evaluate T-cell immunity with reference to antibody titers in a group of pediatric patients with autoimmune arthritides utilizing the widely known Interferon-γ Release Assay (IGRA). Materials and Methods: This study was conducted in the cohort of 55 children suffering from Juvenile Idiopathic Arthritis (JIA). This research analyzed the SARS-CoV-2 T-cell response measured by a specific quantitative IGRA, followed by a serological ELISA test measuring the presence and quantity of IgG, IgM, and IgA antibodies in serum. Results: The cellular response to SARS-CoV-2 measured by the IGRA test significantly correlated with the antibody titers, IgA (p < 0.00003, R = 0.537), IgG (p < 0.0001, R = 0.668), and IgG nucleocapsid protein (NCP) (p < 0.003, R = 0.0399), with no correlation with IgM levels. The antibody levels in patients receiving biological agents were significantly lower compared to the rest of the cohort (p = 0.0369), while traditional disease-modifying antirheumatic drugs had no such effect. Limitations: the main limitation of the research is the small sample size, mostly due to the specific cohort of patients and the lack of a healthy control. Conclusions: IGRA appears to be a viable tool in the accurate evaluation of T-cell responses to SARS-CoV-2, and serodiagnostics alone is not always sufficient in the assessment of immune responses.
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Background: As the SARS-CoV-2 virus remains one of the main causes of severe respiratory system infections, the Food and Drug Administration strongly advises the continuation of current vaccination programs, including the distribution of updated boosters, especially in high-risk groups of patients. Therefore, there is an unceasing need for further research on the safety and, no less importantly, the clinical effectivity of the vaccines, with an extra focus on cohorts of patients with underlying health problems. This study aimed to assess the efficacy of the SARS-CoV-2 vaccine in possibly immunocompromised children with rheumatic disease while utilizing the interferon-gamma release assay (IGRA) as a marker for COVID-19 immunity in the study follow-up. Methods: This prospective study was performed in a group of 55 pediatric patients diagnosed with juvenile idiopathic arthritis. Eight participants were immunized with the Comirnaty mRNA vaccine before the research commenced, while the rest of the group (n = 47) had not been vaccinated against SARS-CoV-2. At the study baseline, the cellular response to the virus antigen was measured using a specific quantitative IGRA in whole blood; subsequently, the anti-SARS-CoV-2 test was performed, marking the antibodies' levels in serum. Around four months after the enrollment of the last patient in the study, a follow-up survey regarding the events of COVID-19 infection within the cohort was conducted. Results: The study confirmed that all the vaccinated children developed specific T-cell (p = 0.0016) and humoral (p = 0.001 for IgA antibodies, p = 0.008 for IgG antibodies) responses to the inoculation, including those receiving biological treatment and those on conventional disease-modifying anti-rheumatic drugs. The study also showed the different patterns of immunity elicited both after infection and post-vaccination, with higher levels of antibodies and T-cell response after inoculation than after natural exposure to the pathogen. According to the follow-up survey, six children developed PCR-confirmed SARS-CoV-2 infection, whereas the additional 10 patients admitted to having COVID-like symptoms with no laboratory verification. Conclusions: SARS-CoV-2 vaccinations elicit valid immune responses in pediatric rheumatic patients. Including the assessment of T-cell immunity in the evaluation of inoculation-induced immunization can enhance the accuracy of sole humoral response assays.
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Macrophage activation syndrome (MAS) is one of the most severe complications of systemic juvenile idiopathic arthritis (sJIA). Around 10% of patients with sJIA exhibit systemic symptoms accompanied by macrophage activation syndrome (MAS), but it may occur subclinically in another 30-40%. In this article, we present a case of a 3-year-old girl diagnosed with sever MAS as an onset of sJIA complicated by disseminated intravascular coagulation (DIC). First symptoms of sJIA were observed about 5 months before setting the diagnose, and it was resembling urticaria. A comprehensive allergological diagnostics were conducted, but no cause for the skin changes was identified. A few weeks before admission to the hospital, the girl was presented with a high fever. During the hospital stay, viral, bacterial, and fungal infections were ruled out. However, the findings indicated significantly elevated markers of inflammation (ferritin, CRP, ESR) in the conducted tests. Meanwhile, swelling of the feet and ankle joints was also observed. Based on Ravelli criteria, we set the diagnosis of MAS in a course of sJIA. We implemented treatment with steroid pulses, followed by cyclosporine; however, her clinical condition did not improve. Despite intensive treatment, skin petechiae were observed twice, and laboratory tests revealed a very high INR along with an extremely low level of fibrinogen. The patient required multiple plasma transfusions and clotting factor administrations. Due to the severe condition of the girl, we initiated biological treatment with anakinra, after which the child's condition gradually improved. In this case, we want to present how dynamic and life-threatening the course of MAS can be. In the discussion, we are also comparing our approach and the applied treatment with the currently available knowledge.
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The available literature is scarce on the initial symptoms of arterial hypertension in children. Our study aimed to analyze the initial clinical profile of patients referred to the hospital with suspected hypertension and those diagnosed with hypertension for the first time during a hospitalization for other reasons. This study was a retrospective analysis of medical records in 471 patients. More than half of the patients showed no symptoms. The most common symptom reported was a headache-28% (132) of patients. The diagnosis of elevated blood pressure or hypertension was more frequent in asymptomatic patients (P = 0.001). Headaches were seen more often in healthy patients than in patients with hypertension. Newly diagnosed hypertension is mainly diagnosed in asymptomatic children. Moreover, the symptoms previously described in the literature as the most common did not prove to be predictive of hypertension in our study.
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BACKGROUND: Lipid disorders are one of the risk factors for cardiovascular diseases. The aim of the study was to estimate the lipid profile in early childhood in the population of Polish children born small for gestational age (SGA). MATERIALS AND METHODS: The study included 140 patients (93 SGA children and 47 controls) aged 5 to 11 years. All the subjects underwent a physical examination and blood laboratory tests for the glucose and lipid profiles. The SGA group was divided into subgroups, i.e., symmetrical and asymmetrical intrauterine growth restriction (IUGR). RESULTS: Blood sample analysis revealed higher levels of total cholesterol (SGA group 190.61 ± 24.66 mg/dL vs. controls 143.23 ± 23.90; p < 0.001). The analysis of particular cholesterol fractions showed significantly higher mean values of triglycerides and LDL cholesterol as well as lower mean values of HDL cholesterol in SGA children. Children in both groups did not differ significantly in terms of weight or body mass index. A statistically significantly higher glucose concentration was observed in SGA patients with the symmetrical type of IUGR. Analyzing the differences regarding metabolic factors, we obtained a statistically significant difference only in fasting glucose concentration (asymmetrical IUGR = 90.56 ± 10.21 vs. symmetrical IUGR = 98.95 ± 14.79; p < 0.001). CONCLUSIONS: Children born SGA, even those not suffering from overweight or obesity in their early childhood, have an abnormal lipid profile, which may contribute to the development of cardiovascular diseases in adulthood.
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Doenças Cardiovasculares , Retardo do Crescimento Fetal , Feminino , Recém-Nascido , Humanos , Criança , Pré-Escolar , Doenças Cardiovasculares/etiologia , Idade Gestacional , Recém-Nascido Pequeno para a Idade Gestacional , Triglicerídeos , Colesterol , GlucoseRESUMO
Diffuse fasciitis with eosinophilia (EF) is a rare condition classified as a part of the connective tissue disorders. The clinical presentation of this condition can be diverse, however the main symptoms include symmetrical swelling and hardening of distal parts of limbs accompanied by peripheral eosinophilia. The diagnostic criteria are not specified. In inconclusions cases Magnetic Resonance Imaging (MRI) and skin to muscle biopsy may be useful. The pathogenesis and ethiology remain unknown, but extensive physical exertion, certain infectious factors, such as Borrelia burgdorferi, or medications may serve as a trigger. EF affects equally women and men, mainly in their middle age, however the disease can occur at any age. The standard therapy contents gluccocorticosteroids. As a second-line treatment, methotrexate is usually chosen. In this article we compare world reports of EF in paediatric patients with the cases of two adolescent male patients recently hospitalized in the Department of Paediatric Rheumatology.
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Eosinofilia , Fasciite , Pessoa de Meia-Idade , Adolescente , Humanos , Masculino , Feminino , Criança , Eosinofilia/complicações , Fasciite/diagnóstico por imagem , Fasciite/tratamento farmacológico , Pele/patologia , BiópsiaRESUMO
Introduction: To determine the impact of single nucleotide polymorphisms (SNPs) in MTHFR and MTRR genes on disease activity and the presence of MTX therapy adverse events in Polish children with juvenile idiopathic arthritis (JIA). Methods: SNP genotyping was performed using genomic DNA isolated from peripheral blood samples. Results: Patients with MTHFR rs1801133 CT/TT variant had higher values of inflammatory markers, number of joints with active arthritis, and JADAS-71 value at the baseline of MTX treatment. Children with MTRR rs1801394 AG/AA variant presented higher inflammatory marker values at JIA diagnosis. Conclusions: MTHFR rs1801133 and MTRR rs1801394 polymorphisms are associated with higher disease activity at the moment of JIA diagnosis.
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INTRODUCTION: The aim of this study was to assess the exposure to cardiovascular disease (CVD) risk factors in patients with juvenile idiopathic arthritis (JIA). Intima-media complex thickness (IMT), selected metabolic parameters and health behaviors were assessed in the course of the study. METHODS: The study included study group, which consisted of 45 patients with JIA and 37 healthy age- and sex-matched children in the control group. Analyses in both groups included anthropometric parameters, laboratory tests, IMT and a questionnaire on exposure to modifiable CVD risk factors. RESULTS: The study confirmed that CVD risk factors were present in both groups of patients. Significantly more children with JIA had abnormal BMI (p = 0.006) compared to the control group. Children in the study group were more likely to consume fruit regularly (p = 0.021) and less likely to consume fast food (p = 0.011) and sweetened beverages (p = 0.042) than children in the control group. Only 1 patient with JIA met criteria for ideal cardiovascular health. Dietary habits were not associated with IMT values, BMI, presence of joint pain or biochemical parameters in the study group. CONCLUSIONS: Patients with JIA are exposed to cardiovascular risk factors equally to their healthy peers. Ideal cardiovascular health should be pursued in the pediatric population with particular attention paid to patients with chronic diseases (i.e., JIA). The application of carotid artery IMT measurement in the assessment of CVD risk requires studies on a larger group of patients.
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Artrite Juvenil , Doenças Cardiovasculares , Humanos , Criança , Doenças Cardiovasculares/etiologia , Doenças Cardiovasculares/complicações , Artrite Juvenil/complicações , Artrite Juvenil/metabolismo , Fatores de Risco , Espessura Intima-Media Carotídea , Fatores de Risco de Doenças Cardíacas , Medição de RiscoRESUMO
Introduction: The safety of COVID-19 vaccines in children with juvenile idiopathic arthritis (JIA) is the concern of patients and their parents and doctors in the current pandemic reality. The main objective of the study was to evaluate the safety of COVID-19 vaccine in patients with JIA. Method: A cohort study based on short clinical follow-up of 43 children with JIA was conducted in the years 2021-2022 in two centres of paediatric rheumatology in Poland. All patients received mRNA COVID-19 vaccine. The patients' data were collected using appropriate validated questionnaire. Disease activity was evaluated using Juvenile Arthritis Disease Activity Score 27-joint count (JADAS-27). Results: Ten (22.7%) children had COVID-19 infection before getting COVID-19 vaccine. After first dose of COVID-19 vaccine 25/43 (58.1%) patients presented typical adverse events: arm pain or oedema at the application side or weakness. Also, twenty five (58.1%) children had side effects after second dose of this vaccine, however the spectrum of the symptoms was wider (additionally: headache, fever, lymphadenopathy, arrhythmia). Thirteen out of 43 (30.2%) patients had active disease before and 8/43 (18.6%) after COVID-19 vaccination, while the degree of JADAS-27 activity was higher in the study group before COVID-19 vaccination (p = 0.047). Conclusions: Our study found out that children and adolescents with JIA with remission without treatment or on the long-term treatment-cDMARDs or even bDMARDs, can be safely vaccinated for COVID-19. Moreover, the study found that COVID-19 vaccination does not interfere with the JIA treatment and does not exacerbate symptoms of the disease and that vaccination protected against developing COVID-19 in children with JIA even on treatment.
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BACKGROUND: Children diagnosed with juvenile idiopathic arthritis (JIA) are thought to be more likely to develop cardiovascular disease in adulthood. The factors modulating the cardiovascular risk, involving exposure to secondhand smoking, sedentary lifestyle and abnormal body mass index, might have had a stronger impact during the COVID-19 pandemic. The lack of reliable prognostic markers for a higher probability of cardiovascular events might be solved by carotid intima-media thickness (cIMT) measurement. The paramount goal of the study was to assess its usefulness in JIA patients. MATERIALS AND METHODS: The results of cIMT measured by a single physician in 45 children diagnosed with JIA were compared to 37 age- and sex-matched healthy counterparts. The analysis also involved anthropometric parameters, laboratory tests, and a survey regarding lifestyle-related factors. RESULTS: Four JIA patients appeared to have cIMT above the 94th percentile. A positive correlation between erythrocytes sedimentation rate (ESR) and right carotid artery percentiles was found. Passive smoking increased the cardiovascular risk regardless of JIA. Doubling the daily screen time during the pandemic led to a significant reduction in children's physical activity. However, the number of enrolled subjects was not enough to make significant recommendations. CONCLUSIONS: cIMT measurements remain an interesting perspective for future cardiovascular screening of children with JIA. It has yet to be determined whether it should be considered in all JIA patients on a reliable basis.
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BACKGROUND: Adipose tissue is not only a storage place for fat, but also an endocrine organ, secreting bioactive molecules which influence body metabolism. Such molecules are known as adipocytokines. In the past years the coincidence between adipocytokines and fetal growth restriction disorders was found. The aim of the study was to estimate serum levels of adiponectin, leptin and resistin in children born small for gestational age, compared to children born at an appropriate size for gestational age. METHODS: The study consisted of 35 children aged seven to nine years, born SGA (small for gestational age) on term and 25 healthy children (14 girls, 11 boys), born with proper birthweight (AGA-appropriate for gestational age)-control group. RESULTS: Adiponectin and leptin levels were significantly higher in the SGA group compared to the AGA group (p = 0.023, p = 0.018 respectively). The resistin values were comparable in both groups of patients. There was a positive correlation between serum leptin concentration and current body weight in SGA group (r = 0.28; p = 0.108). In turn, adiponectin levels in this group of patients negatively correlated with actual body weight (r = -0.51; p = 0.002). The negative correlation between body mass index and plasma adiponectin levels was found only in children born SGA. SGA children had significantly higher values of diastolic blood pressure. There was negative correlation between serum adiponectin level and systolic blood pressure in SGA children. In the SGA group the phenomenon of catch-up growth was observed in 32 children. CONCLUSIONS: Children born SGA have abnormal adipose tissue biomarkers profiles.
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Adipocinas , Resistência à Insulina , Masculino , Recém-Nascido , Feminino , Humanos , Criança , Leptina , Resistina , Adiponectina , Idade Gestacional , Resistência à Insulina/fisiologia , Peso Corporal , Recém-Nascido Pequeno para a Idade Gestacional , Retardo do Crescimento FetalRESUMO
Severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) and its mechanisms have been thoroughly studied by researchers all over the world with the hope of finding answers that may aid the discovery of new treatment options or effective means of prevention. Still, over 2 years into the pandemic that is an immense burden on health care and economic systems, there seem to be more questions than answers. The character and multitude of immune responses elicited in coronavirus disease 2019 (COVID-19) vary from uncontrollable activation of the inflammatory system, causing extensive tissue damage and consequently leading to severe or even fatal disease, to mild or asymptomatic infections in the majority of patients, resulting in the unpredictability of the current pandemic. The aim of the study was to systematize the available data regarding the immune response to SARS-CoV-2, to provide some clarification among the abundance of the knowledge available. The review contains concise and current information on the most significant immune reactions to COVID-19, including components of both innate and adaptive immunity, with an additional focus on utilizing humoral and cellular responses as effective diagnostic tools. Moreover, the authors discussed the present state of knowledge on SARS-CoV-2 vaccines and their efficacy in cases of immunodeficiency.
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COVID-19 , Humanos , SARS-CoV-2 , Pandemias , Vacinas contra COVID-19 , Imunidade Adaptativa , Imunidade InataRESUMO
OBJECTIVE: To develop and externally validate a prediction model for new-onset chronic uveitis in children with juvenile idiopathic arthritis (JIA) for clinical application. METHODS: Data from the international Pharmachild registry were used to develop a multivariable Cox proportional hazards model. Predictors were selected by backward selection, and missing values were handled by multiple imputation. The model was subsequently validated and recalibrated in 2 inception cohorts: the UK Childhood Arthritis Prospective Study (CAPS) study and the German Inception Cohort of Newly diagnosed patients with juvenile idiopathic arthritis (ICON) study. Model performance was evaluated by calibration plots and C statistics for the 2-, 4-, and 7-year risk of uveitis. A diagram and digital risk calculator were created for use in clinical practice. RESULTS: A total of 5,393 patients were included for model development, and predictor variables were age at JIA onset (hazard ratio [HR] 0.83 [95% confidence interval (95% CI) 0.77-0.89]), ANA positivity (HR 1.59 [95% CI 1.06-2.38]), and International League of Associations for Rheumatology category of JIA (HR for oligoarthritis, psoriatic arthritis, and undifferentiated arthritis versus rheumatoid factor-negative polyarthritis 1.40 [95% CI 0.91-2.16]). Performance of the recalibrated prediction model in the validation cohorts was acceptable; calibration plots indicated good calibration and C statistics for the 7-year risk of uveitis (0.75 [95% CI 0.72-0.79] for the ICON cohort and 0.70 [95% CI 0.64-0.76] for the CAPS cohort). CONCLUSION: We present for the first time a validated prognostic tool for easily predicting chronic uveitis risk for individual JIA patients using common clinical parameters. This model could be used by clinicians to inform patients/parents and provide guidance in choice of uveitis screening frequency and arthritis drug therapy.
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Artrite Juvenil , Artrite Psoriásica , Uveíte , Criança , Humanos , Artrite Juvenil/complicações , Artrite Juvenil/epidemiologia , Artrite Juvenil/diagnóstico , Estudos Prospectivos , Uveíte/epidemiologia , Uveíte/etiologia , Uveíte/diagnóstico , PrognósticoRESUMO
Pediatric multi-system inflammatory syndrome temporally associated with COVID-19 (PIMS-TS) is a serious complication of a previous SARS-CoV-2 infection. The disease causes multiple organ failure, but in some patients, a more severe course of the disease is observed. The treatment is multidirectional and depends on the severity and course of the disease, as some patients do not respond to the recommended treatment. The aim of this study was to identify laboratory risk factors affecting the more severe course of the disease and resistance to standard therapy. It is a single-center retrospective study considering 51 patients with PIMS-TS. Clinical features, laboratory results, and additional imaging tests data were taken into account. Fifty-one patients with PIMS-TS were hospitalized within a 16-month observation period. In the studied group, 26/51 children (51%) were girls. The mean age of patients was 7 years. Sex of the patient was not a risk factor for changes in cardiovascular system or severe course of the disease. Sixteen patients (31.3%) required transfer to the intensive care unit. Children with initially higher concentrations of NT-proBNP, troponin, creatinine, triglycerides, C-reactive protein, procalcitonin, ferritin, D-dimers and lower hematocrit, platelet count, lymphocytes, and ejection fraction should be strictly observed as they have a higher risk of severe course of the disease. CONCLUSIONS: Laboratory parameters especially markers of myocardial damage, markers of inflammation, blood count, as wells as biochemical parameters are significant risk indicators of severe course of PIMS -TS and their concentration can be defined as predictor of disease severity. WHAT IS KNOWN: ⢠Pediatric multisystem inflammatory syndrome temporally associated with COVID-19 (PIMS-TS) is a serious complication of a previous SARS-CoV-2 infection in the group of pediatric patients ⢠Course of the disease may be severe, which may cause long-term complications and the need for longitudinal patient care. WHAT IS NEW: ⢠Children with higher concentrations of NT-proBNP, troponin, creatinine, TG, CRP, PCT, ferritin, D-dimers and lower hematocrit, PLT, lymphocytes, and EF have a higher risk of a severe course of the disease. ⢠Patients with high concentration of NT-proBNP, troponin, CRP, lactates, ferritin, D-dimers, creatinine and a lower concentration of PLT, albumin, leukocytes; lymphopenia, hyponatremia are at risk for intravenous immunoglobulin resistance.
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COVID-19 , Proteína C-Reativa , COVID-19/complicações , Criança , Creatinina , Feminino , Ferritinas , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Lactatos , Masculino , Pró-Calcitonina , Estudos Retrospectivos , Fatores de Risco , SARS-CoV-2 , Síndrome , Síndrome de Resposta Inflamatória Sistêmica , Triglicerídeos , TroponinaRESUMO
Background Pediatric multisystem inflammatory syndrome temporally associated with COVID-19 (PIMS-TS) is a serious complication of a previous SARS-CoV-2 infection in the group of pediatric patients. Despite the fact that this disease affects only about 1 of 1000 children, it may be severe, and changes in the cardiovascular system may cause long-term complications and the need for longitudinal patient care. Methods It is a single-center retrospective study considering 51 patients with PIMS-TS. The aim of this study was to analyze patients with PIMS-TS, taking into account demographic data, clinical course, laboratory tests and cardiovascular system assessment (electrocardiography, echocardiography, etc.). Results From June 2020 to October 2021, 51 patients with PIMS-TS were hospitalized in our center. In the studied group, 26/51 children (51%) were girls. The mean age of patients was 7 years. Changes in electrocardiograms were found in 21/51 patients. Echocardiography revealed pericardial fluid in most patients. In addition, heart valve insufficiency was found. Changes in the coronary vessels in the form of dilatations and even aneurysms were found in 42 patients. Myocardial hypokinesis was visualized in more than 1/4 of the patients. Sixteen patients (31.3%) required transfer to the intensive care unit (ICU) due to severe hypotension. Laboratory tests revealed increased levels of markers of heart failure and enzymes of myocardial damage. Conclusions Changes in the cardiovascular system in the course of PIMS-TS are of various nature, but in most patients they require close cardiac supervision and long-term follow-up.
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COVID-19 , Sistema Cardiovascular , COVID-19/complicações , COVID-19/diagnóstico , Criança , Feminino , Humanos , Masculino , Estudos Retrospectivos , SARS-CoV-2 , Síndrome de Resposta Inflamatória Sistêmica/diagnósticoRESUMO
Raynaud phenomenon (RP) may be the first manifestation of a systemic connective tissue disease (SCTD). Early detection of dysfunction of small vessels called microangiopathy is essential for the diagnostic process. The focus of this single-center, retrospective study was to investigate the potential dependencies between microvascular image and laboratory markers measured in children with RP. The study analyzed the nail-fold video-capillaroscopy (NVC) findings and laboratory results of 81 children between the ages 6 and 17 who were referred to pediatric rheumatologist with a suspicion of SCTD. Out of 52 patients presenting with RP at the time of evaluation, abnormalities in capillary microscopic imaging were found in 34. NVC findings were then compared to levels of specific biomarkers in serum. Vitamin D3 serum levels have been significantly decreased in patients with RP (23.4 ng/mL ± 8.76 vs. 30.0 ng/mL ± 12.66, P = 0.0148). There were positive significant correlations between levels of vitamin D3 and acute-phase reactants in serum, such as C-reactive protein (P = 0.0292). Furthermore, free thyroxine levels (fT4) in patients with both RP (P = 0.0126) and micro-angiopathy (P = 0.05496) persisted in the lower range of the normal limit (< 1.0 ng/dL). Regular oral supplementation of vitamin D3 should be always considered in children with RP if deficiency is found. Additionally, low fT4 level (< 1.0 ng/dL) should be considered as an indication to perform NVC in patients suspected of SCTD even when they do not present RP.
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Colecalciferol/deficiência , Doenças do Tecido Conjuntivo/sangue , Doença de Raynaud/sangue , Tiroxina/deficiência , Adolescente , Biomarcadores/sangue , Criança , Colecalciferol/sangue , Doenças do Tecido Conjuntivo/diagnóstico , Feminino , Humanos , Masculino , Angioscopia Microscópica , Doença de Raynaud/diagnóstico , Estudos Retrospectivos , Tiroxina/sangueRESUMO
Multisystem inflammatory syndrome in children is a newly described condition temporarily associated with severe acute respiratory syndrome coronavirus 2. The primary observations indicated coronavirus disease 2019 infection in children to be mild. However, recent reports have revealed a correlation between multisystem inflammatory symptoms and coronavirus infection. The manifestation of the disease is similar to Kawasaki disease, toxic shock syndrome and macrophage activation syndrome. Treatment includes intravenous corticosteroids, immunoglobulin and biological therapy (anakinra, tocilizumab, infliximab). In this article we compare world reports of multisystem inflammatory syndrome in children to case reports of two patients who were hospitalized recently at the Department of Paediatric Cardiology and Rheumatology. In the course of diagnostics they presented numerous symptoms which required differentiation from multisystem inflammatory syndrome in children.
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Small, noncoding sequences of ribonucleic acid called microRNAs (miRNAs, miR) are functioning as posttranscriptional regulators of gene expression. As they draw increasing attention of rheumatologists, there is a growing body of evidence concerning specific molecules that may affect the long-term care of patients with inflammatory arthritides. Findings involving children with juvenile idiopathic arthritis (JIA) are still limited though. The aim of the study was to browse the available data on microRNAs which may be utilized as potential biomarkers helpful in diagnosing and monitoring JIA patients. The review contains a brief summary on the most studied sequences: miR-16, miR-125a-5p, miR-146a, miR-155, and miR-223. It is complemented with other miRNAs possibly relevant for JIA (miR-145, miR-23b, miR-27a, and miR-204) and discussion on challenges for using miRNAs in pediatric rheumatology (particularly, issues regarding specificity of biomarkers and measurements involving synovial fluid).
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Artrite Juvenil/diagnóstico , Artrite Juvenil/genética , Biomarcadores , MicroRNAs/genética , Tomada de Decisão Clínica , Gerenciamento Clínico , Suscetibilidade a Doenças , Regulação da Expressão Gênica , HumanosRESUMO
AIM: The goal of the study was to assess the diagnostic and prognostic utility of survivin in patients with juvenile idiopathic arthritis (JIA). METHODS: Seventy children with JIA-59 newly diagnosed and 11 biologically treated (46 girls and 17 boys) aged 1.5-18 years and 29 healthy children as a control group, appropriately matched in terms of sex and age, were included in the study. The disease activity was established on the basis of the JADAS-27 criteria. The concentration of survivin was assessed by an ELISA test in serum and also 18 matched synovial fluid samples collected from patients with JIA. RESULTS: Children with JIA were divided according to the subtype of the JIA. In 65.7% of patients, oligoarthritis was diagnosed. The largest group comprised children of low disease activity (62.9%) according to JADAS-27. The serum concentration of survivin was significantly higher in children with JIA compared to the controls (p < 0.001). The concentration of survivin was higher among patients positive for anti-cyclic citrullinated peptide autoantibodies (ACPA) (p = 0.001). In all synovial fluid samples, the concentration of survivin was higher than in matched serum (p = 0.003). Serum survivin concentration was not significantly associated with radiological damage status or active synovitis assessed by joint ultrasonography. Survivin level was not significantly associated with disease duration time or treatment with TNF-α inhibitors in DMARD's non-responders. CONCLUSIONS: Survivin should be considered as a biomarker of joint inflammation helpful in the diagnosis of oligo- and polyarticular JIA and probably not dependent on treatment with TNF-α inhibitors.