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1.
Cocuk Enfeksiyon Dergisi ; 16(2):77-86, 2022.
Article in English | EMBASE | ID: covidwho-2010458

ABSTRACT

Objective: Multisystem inflammatory syndrome (MIS-C) in children is a newly defined and serious health problem that develops after SARS-CoV-2 infection. Our aim is to report epidemiological, clinical, laboratory and radiological features of children with MIS-C. Material and Methods: Forty patients who applied to our hospital from October 2020 to February 2021 and met the MIS-C criteria were included in the study. Patients with gastrointestinal involvement (GIS), cardiac involvement and Kawasaki Disease (KD)-like MIS-C were examined clini-cally and laboratory. Results: The mean age of the patients was 8.2 ± 4.2 years and male patients were in the majority (70%). The most common symptoms were fe-ver (100%) and fatigue (90%). Gastrointestinal symptoms were present in 71%, cardiac involvement in %40, Kawasaki-like patients in %52.5, shock symptoms in 59%. Elevated levels of C-reactive protein, D-dimer, and ferritin were found in 100%, 97.5%, and 67.5% of the patients, respec-tively. Patients with cardiac involvement had higher mean age and lower lymphocyte levels. Shock findings were higher in patients with KD-like MIS-C. Also, INR and ferritin levels were higher in KD-like MIS-C patients (p= 0.028). The mean platelet count (p= 0.004) and albumin levels were lower (p= 0.048) in shock group. Conclusion: MIS-C is a hyperinflammatory syndrome with cardiac, GIS, and lung involvement. Cardiac findings were not common in patients presenting with KD-like MIS-C, but a poor prognosis was observed in KD-like MIS-C patients. Patients with cardiac involvement were older and their lymphocyte count was lower.

2.
Frontiers in Pediatrics ; 10, 2022.
Article in English | EMBASE | ID: covidwho-2009892

ABSTRACT

Background: With the rapid surge of SARS-CoV-2 Omicron variant, we aimed to assess parents' perceptions of the COVID-19 vaccines and the psychological antecedents of vaccinations during the first month of the Omicron spread. Methods: A cross-sectional online survey in Saudi Arabia was conducted (December 20, 2021-January 7, 2022). Convenience sampling was used to invite participants through several social media platforms, including WhatsApp, Twitter, and email lists. We utilized the validated 5C Scale, which evaluates five psychological factors influencing vaccination intention and behavior: confidence, complacency, constraints, calculation, and collective responsibility. Results: Of the 1,340 respondents, 61.3% received two doses of the COVID-19 vaccine, while 35% received an additional booster dose. Fify four percentage were unwilling to vaccinate their children aged 5–11, and 57.2% were unwilling to give the additional booster vaccine to children aged 12–18. Respondents had higher scores on the construct of collective responsibility, followed by calculation, confidence, complacency, and finally constraints. Confidence in vaccines was associated with willingness to vaccinate children and positively correlated with collective responsibility (p < 0.010). Complacency about COVID-19 was associated with unwillingness to vaccinate older children (12–18 years) and with increased constraints and calculation scores (p < 0.010). While increasing constraints scores did not correlate with decreased willingness to vaccinate children (p = 0.140), they did correlate negatively with confidence and collective responsibility (p < 0.010). Conclusions: The findings demonstrate the relationship between the five antecedents of vaccination, the importance of confidence in vaccines, and a sense of collective responsibility in parents' intention to vaccinate their children. Campaigns addressing constraints and collective responsibility could help influence the public's vaccination behavior.

3.
Annals of the Rheumatic Diseases ; 81:979-980, 2022.
Article in English | EMBASE | ID: covidwho-2009204

ABSTRACT

Background: Pediatric Infammatory Multisystemic Syndrome associated to SARS-CoV2 (PIMS) happens 4 to 6 weeks after SARS-CoV2 infection1-2. Its early diagnostic recognition as well as its early management is important to avoid cardiac complications related to this pathology. Objectives: To highlight a frequent symptom in PIMS and improve its therapeutic care. Methods: The JIR Cohort database, an international registry collecting data on patients with pediatric infammatory diseases, was consulted to include patients between 03/15/20 and 12/31/2021. Results: Of the 140 patients in whom a diagnosis of PIMS was retained, we present a series of 38 patients (27%) who presented at diagnosis or during evolution, febrile torticollis or painful cervical involvement. These patients were on average 8.2 years old (0.6-15.2). The proportion of boys was 14 out of 38 (37%). Twenty-four patients out of 33 (73%) were hospitalized in intensive care. Ten patients out of 38 (26%) underwent cervical imaging, 5 (50%) had abnormalities such as collection or infltration of the soft tissues. At the therapeutic level, 27/38 patients (71%) received corticosteroid therapy, 33/38 (87%) immunoglobulins, and 26/38 (68%) antibiotic therapy. Conclusion: PIMS is a pathology with signifcant clinical heterogeneity and severe consequences in case of delay in therapeutic management. In this epidemic context, it is important to consider PIMS in any patient with febrile torticollis, especially if he does not respond to antibiotics.

4.
Annals of the Rheumatic Diseases ; 81:978-979, 2022.
Article in English | EMBASE | ID: covidwho-2009189

ABSTRACT

Background: Across the globe, coronavirus disease 2019 (COVID-19) caused by SARS-CoV-2 appears to affect paediatric population in a milder and non-threatening way, when compared to adults. However, since April 2020 case reports of previously healthy children presenting with unremitting fever, biologic infammatory syndrome and cardiac dysfunction have been emerging. This syndrome, which has been termed Pediatric Infammatory Multisystem Syndrome Temporally Associated with SARS-CoV-2 infection (PIMS-TS), represents a rare complication of COVID-19 in children1. Objectives: To describe the clinical, laboratory and imaging characteristics, course, management and outcomes of hospitalized children diagnosed with PIMS-TS in a Portuguese tertiary care hospital. Methods: A retrospective study including children (< 18 years) that attended our hospital from April 2020 to December 2021 was performed. All the children included fulflled the case defnition of PIMS-TS published by the Centre for Disease Control and Prevention. Sociodemographic and clinical data, laboratory markers and imaging fndings were collected. Results: A total of 19 children met the criteria for PIMS-TS, 68% male with a mean age at diagnosis of 8 years old (IQR 5.8-15). They were all caucasian, except for a mixed-race patient, and all previously healthy, except one patient who was obese. Twelve had recent infection by SARS-CoV-2 detected by reverse transcriptase (RT) PCR and 18 had positive IgG serology. All had fever at diagnosis, with a median duration of 6 days (IQR 5-6) and 89.5% had mucocutaneous, gastrointestinal and hematological attainment, respectively. Other affected systems were respiratory (73.7%), cardiovascular (63%), lymphoid organs (52.6%), musculoskeletal (47%), genito-urinary (31.6%) and neurological (26.3%). Laboratory fndings can be found in Table 1. Thirty-six percent were admitted in intensive care unit for a median duration of 8 days (IQR 4-9). 42.1% needed respiratory support, 87.5% with supplemental oxygen therapy, 62.5% with mechanical ventilation and 12.5% with non-invasive ventilation. All patients received intravenous (IV) immunoglobulin, 52.6% IV cor-ticosteroid (CS) pulses and 78.9% IV and oral CS. Other treatments included acetylsalicylic acid (n=18), heparin (n=8) and antibiotic therapy (n=19)-Table 3. Seventeen fully recovered and 2 had sequalae: one of them with coronary artery aneurysms and other exertional dyspnea. Conclusion: In this case series, there was a broad spectrum of clinical symptoms and disease severity, ranging from fever and systemic infammation to critical care admission with myocardial injury, shock, and development of coronary artery aneurysms. Despite short-term morbidity, there were no mortality cases, with most of them recovering without sequelae. All physicians providing clinical care to children should consider this rare but severe delayed syndrome in paediatric population.

5.
Annals of the Rheumatic Diseases ; 81:144, 2022.
Article in English | EMBASE | ID: covidwho-2009183

ABSTRACT

Background: In contrast to adults, children are less likely to develop serious disease upon infection with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) but are at increased risk for infammatory and autoimmune diseases linked to the virus (1). The reported incidence of multisystem infammatory syndrome in children (MIS-C) varied from 0.2 to 11.4/100,000 persons under 21 years (2,3). It is yet unknown whether MIS-C can recur after SARS-CoV-2 reinfection or COVID-19 vaccination. Objectives: To estimate the incidence and describe the spectrum of infam-matory and autoimmune diseases linked to SARS-CoV-2 infection and coronavirus (COVID-19) vaccination in pediatric patients from two neighbouring South Central European countries and regions, Slovenia and Friuli Venezia Giulia (FVG), Italy. Methods: We performed a multi-centre prospective cohort study of all children and adolescents (under 18 years) newly diagnosed with MIS-C or other inflammatory/autoimmune diseases linked to SARS-CoV-2 infection, who were admitted to the pediatric tertiary care hospitals in Slovenia or FVG, Italy during the period from January 1, 2020, to December 31, 2021. These hospitals serve a combined population of 587,053 children and adolescents. Only patients who had positive anti-SARS-CoV-2 antibodies and/or positive SARS-CoV-2 PCR test within 3 months prior to disease onset were considered for estimating the disease incidence. We obtained the number of patients with serious adverse events (SAE) after COVID-19 vaccination and the number of patients with severe COVID-19 in the same population. This study was conducted as a part of the EU interregional Italy-Slovenia project CATTEDRA (Cross border cooperation for innovative diagnosis of rare diseases in paediatrics). Results: 192 children and adolescents were diagnosed with infammatory and autoimmune diseases linked to SARS-CoV-2 (Figure 1). Median age at diagnosis was 11. 9 years (IQR 7. 6-14.7). All included patients were White. Incidence of MIS-C was one in 921 children and adolescents after SARS-CoV-2 infection and one in 5870 of all children and adolescents. Cumulative incidence of MIS-C since the start of the pandemic was 17/100,000 children and adolescents. Until December 31, 2021, 92,139 children and adolescents (15.7 %) received at least one dose of COVID-19 vaccine. Three patients presented with infammatory/autoimmune disease after COVID-19 vaccination, including 2 patients with MIS-C and one patient with myositis. All 3 had evidence of recent SARS-CoV-2 infection in form of positive anti-N SARS-CoV-2 antibodies. In the same period, 15 children and adolescents were hospitalised with severe COVID-19. Seven patients from our cohort were vaccinated against COVID-19 median 8 months after MIS-C and further 6 patients had a SARS-CoV-2 reinfection 3-14 months after MIS-C. None of them experienced SAE or recurrence of MIS-C. COVID-19=coronavirus disease, FVG=Friuli Venezia Giulia region in Italy, MIS-C=multisystem infammatory syndrome in children, SARS-CoV-2=severe acute respiratory syndrome coronavirus 2 Conclusion: MIS-C was the most common manifestation and its incidence in this predominantly white population was higher than previously reported. Based on our limited experience, MIS-C does not seem to recur after SARS-CoV-2 reinfection or COVID-19 vaccination, however long-term data are lacking. Autoimmune diseases were much more common after SARS-CoV-2 infection than after COVID-19 vaccination. Hospitalisations due to MIS-C were seven times as frequent as hospitalisations due to severe COVID-19 in children.

6.
Annals of the Rheumatic Diseases ; 81:973, 2022.
Article in English | EMBASE | ID: covidwho-2009156

ABSTRACT

Background: Coronavirus vaccines have been widely applied all over the world after the coronavirus pandemic. There are inactivated vaccines and mRNA vaccines in our country. There is no clear guideline for the vaccination programs of patients receiving immunosuppressive therapy, especially childhood. Objectives: For this reason, we wanted to evaluate the frequency of side effects developed after covid-19 vaccine in pediatric patients diagnosed with rheumatic disease using biological therapy and nonbiologic disease modifying drugs Methods: A total of 226 patients over the age of 12, who were followed up in the pediatric rheumatology clinic of the University of Health Sciences, Umraniye Training and Research Hospital, using biological therapy and were vaccinated against Covid-19, were included in the study. The standard questionnaire forms were flled in face-to-face after each administration for both vaccines. The patient, who had any serious side effects, was followed up in the hospital. Results: Of the 226 patients included in the study, 97 were male and 128 were female. Their mean age was 16.4 + 2.4. It was determined that 88.4% (n=200) of the patients had mRNA vaccine and 11.6% (n=26) had inactivated vaccine. 105 of the patients were using biologic drugs during vaccination. Of these, 63 (27.9%) patients were treated with anti-TNF drugs (46 adalimumab, 10 etanercept, 6 infix-mab), 32 patients were anti-il-1 (30 canakinumab, 2 anakirna), 4 patients were anti-il-6, 3 patients were anti-il-17, 3 patient was receiving abatacept and 1 patient was receiving rituximab. 121 patients were using DMARDs. Of these, 61 were using colchicine. 26.5% (n=60) of the patients had covid infection before vaccination. Side effects were observed in 180 of our patients. No side effects were observed in 46 patients. Pain at the injection site was the most common among them, 72.6% (n=164). Headache was seen in 16.8% (n=38) of the patients, myalgia in 18.1% (n=41), fever in 16.4% (n=37) and arthralgia in 6.6% (n=15). The frequency of serious adverse events was determined as 0.9%(n=2). Both patients were followed up in the ward. When the patients were compared in terms of covid infection and gender, there was no signifcant difference in the frequency of side effects. When the vaccines were compared, the incidence of side effects in the mRNA vaccine was statistically signifcantly higher (p=0.001). Pain at the injection site was signifcantly less frequent in the inactivated vaccine (p=0.004). In terms of drug distribution, there was no signifcant difference in the frequency of side effects between patients using biologic drugs and DMARDs. p=0.004) There was no statistically signifcant difference in the frequency of side effects when the patients using dmard were compared as colcicine and other dmards. Conclusion: In our study, we have shown that the use of vaccines in individuals with adolescent rheumatological diseases is safe and that the biological treatments used by the patients do not cause an increase in the risk of vaccine side effects. In addition;We have shown in our study that the side-effect profile of the inactivated vaccine used in our country is milder than the mRNA vaccine, and that it affects daily life less. Another result of our study was that anti-TNF drugs could cause a decrease in pain sensation due to the relationship of anti-TNF with pain pathways.

7.
Annals of the Rheumatic Diseases ; 81:337-338, 2022.
Article in English | EMBASE | ID: covidwho-2009134

ABSTRACT

Background: The study of the features of the course and mutual influence of the new coronavirus disease COVID-19 and various rheumatic diseases (RD) in children can still give us new lessons, warnings and fears. Objectives: To update the analysis in a retrospective study the course of covid-19 in children with RD based on the results of two years of the pandemic. To analyze the impact of COVID-19 on the course of RD in children. Methods: Retrospective analysis based on data from single center. The study included patients with RD and confirmed COVID-19 for 2 years (2020-2021). Results: Were registered 320 cases of COVID-19 in children with RD. 158 (49%) patients were asymptomatically infected, 162 (51%) had clinical symptoms. A detailed description of the groups is presented in Table 1. Clinical symptoms were fever (67%), anosmia (47%), rhinitis (34%), cough (19%), arthralgia/myalgia (16%), dyspepsia (5%), rash (2.5%), pneumonia (3%). In the majority of cases (98%), COVID-19 proceeded in mild to moderate severity. Hospitalization due to COVID-19 was required just in 5 cases. 2 children were admitted to the intensive care unit. First, an 11-year-old girl with sJIA with the history of recurrent episodes of MAS resolved by regular administration of canakinumab. 2nd, a 12-year-old girl with Sjogren's syndrome, who received Rituximab and 1 month later she developed the COVID-19 with MIS-like clinical picture, pneumonia with 25%CT lessions. Both cases have a favorable outcome. The clinical characteristics of the patients are presented in Table 1. COVID-19 didǹt affect the course of RD in the 86% of pts. However, 15% developed a fare of the RD with average of 3 months after COVID-19. In this group for 13 pts (girls mostly F/M-9/4, mean age 15 years [9;16]), COVID-19 triggered the new onset of RD (non-systemic JIA-4, Uveitis-1, non-bacterial osteomyelitis-3, systemic JIA-2, Scleroderma-2, Sjögren's syndrome-1. 12 from 13 these children had clinical symptoms on COVID-19. Whether this is Long-COVID syndrome or an independent RD is not known. Conclusion: Our study suggests that the new coronavirus infection in most cases in children with RD, had mild or asymptomatic course, regardless of therapy with immunosuppressive drugs and bDMARD, except of 1 observation with the previous therapy of Rituximab. Worsening of RD after coronavirus infection developed in 15% of cases, regardless of its clinical manifestations. In 13 patients, the RD were started just after COVID-19. The explosive increasing of the incidence of a new strain of COVID-19 for a past month may change the current results and conclusions.

8.
Annals of the Rheumatic Diseases ; 81:1736, 2022.
Article in English | EMBASE | ID: covidwho-2009026

ABSTRACT

Background: Familial Hemophagocytic lymphohistiocytosis (fHLH) categorized as FHL2 (PRF1), FHL3 (UNC13D), FHL4 (STX11), and FHL5 (STXBP2) encoding for Perforin, Munc13-4, Syntaxin11, and Syntaxin binding protein 2, respectively. There is limited information available about the clinical and mutational spectrum of FHL patients in Indian population. Objectives: To delineate clinical and laboratory features of late onset familial Hemophagocytic Lymphohistiocytosis. Methods: A 12-years-old well nourished sick looking boy, born to a non-consanguineous parents with normal birth, development and immunization history with uneventful past presented to us with 6 days history of high fever, cough, breathing difficulty and severe headache. He had occasional vomiting, abdominal pain, polyarthragia & chest pain from last 10 days. Mother also had given history of throat pain, backache & some non-specifc papular rashes over face before the onset of fever. His vitals were normal. Examination revealed faint diffuse fxed erythematous rash all over the body, pallor, icterus and hepatos-plenomegaly. Musculoskeletal examination was unremarkable. Lab evaluation revealed HB 8.9gm%, TLC 4700/cumm with neutrophils 40% and lymphocytes 56% with 8-9% activated lympocytes. Further evaluation showed low ESR 6mm/hr, fbrinogen 97mg% and albumin 2.2 gm% with elevated CRP 40mg/L, ferritin 2000ng/ml, LDH 658IU/L, SGPT 110IU/L, SGOT 221 IU/L, total bilirubin 6mg%, D-dimer 4355 ng:EFU/ml and Triglycerides 441mg%. His blood, urine, CSF and bone marrow cultures were sterile for endemic bacterial and viral infections in our area. His EBV PCR, CoVID RT PCR and CoVID antibody (Total & IgG) test were negative. His immunoglobulin leves were normal. HRCT Chest showed bilateral mild-moderate plural effusions, mild interstitial thickening in both the lower lobes, few fbrotic opacities & old areas of consolidation bilaterally. 2D echo showed mild pericardial effusion. Bone marrow examination showed Hypercellular marrow with iron depletion and occasional hemophagocytosis with CD8 T lymphocytes proliferation (55.2%) and double positive CD4 & CD8 (1.2%). He was initially commenced on supportive therapy, oxygen & intravenous antibiotics. In view of most probable non-infectious, non-malignant hemophagocytic lymphohistiocytosis, he was fnally given intravenous immunoglobulin (2gm/kg) and intravenous pulse methylprednisolone (30mg/kg). He responded well to above regimen within 3 days. He was discharged with tapering steroids over few weeks. Clinical exome by NGS revealed Homozygous Mutation in STXBP2 gene Intron 14, c.1280-1G>C (3' Splice Site) His parents has been counselled for hematopoietic stem cell transplantation and their decision is still pending. Results: We compared our patietnt with a reference to the largest Indian series of pediatric HLH1. Conclusion: Primary HLH type 5 can present frst time during childhood and adolescence. Any child presenting with unexplained HLH features should undergo genetic analysis irrespective of person's past and family history.

9.
Annals of the Rheumatic Diseases ; 81:1731, 2022.
Article in English | EMBASE | ID: covidwho-2008936

ABSTRACT

Background: Primary Sjögren's syndrome (pSS) is a chronic and progressive multisystem autoimmune disease which rarely onset in children and adolescents. Diagnostic delay in large part of patients are common due to the non-specifc and variable symptoms and the slow progression of disease. Objectives: To analyse demographic data, specifc extraglandular, salivary and ocular manifestations, laboratory parameters and therapy of pSS with juvenile onset. Methods: Retrospective study of all patients (pts) with pSS in single center. Results: pSS was verifed in 15 pts (6.7% were boys), which amounted to 23.8% of all pts with SS in our pediatric rheumatologic department. The median age of pSS onset was 8.0 y.o. [IQR 7.0;10.2]. The median of disease duration at the time of pSS verifcation was 2.75 years [2.2;5.6]. All patients had systemic manifestations at onset: constitutional abnormalities-33.3%, nonerosive polyarthritis-64.3%, polyarthralgias-26.7%, lymphadenopathy-73.3%, cutaneous involvement-53.3% (2-xerosis, 2-annular erythema, 1-erythema nodo-sum, 2-Raynaud phenomenon, 2-nonspecifc spotty rashes, 1-hemorrhagic rash). At the time of diagnosis 7 pts (46.7%) had isolated involvement of salivary glands, 8 pts (53.3%)-combined with involvement of lacrimal glands. The decrease in salivary gland function was recorded in 80% of cases, hypolacrimia-in 46.7%, 1 patient had isolated hypolacrimia. Recurrent parotitis was present in 6 pts (40.0%). At time of diagnosis pulmonary involvement had 20.0% of pts, 1 patient had renal tubular acidosis. 8 pts (53.3%) had various hematological disorders: anemia-in 3 pts (20.0%), leukopenia-in 6 (40.0%). ANA Hep-2 were detected in 100% pts (in titer 1/640-4, 1/1280-7, 1/2560-3, 1/20480-1, with mixed patterns in all pts: speckled + homogeneous-9 pts, speckled + homogeneous+cytoplasmic-6 pts), anti-Ro-in 12 pts (80.0%), anti-La-in 8 pts (53.3%), RF+-in 9 pts (60.0%). 6 pts (40.0%) had polyclonal hypergammaglob-ulinemia, max 42%. 2 pts (13.3%) had concomitant autoimmune non-rheumatic disease;1-cutaneous psoriasis, 1-autoimmune thyroiditis. The treatment of each patient was justifed by the main individual manifestations: 93.3% received glucocorticoids, 26.7%-methotrexate, 33.3%-hydroxychloroquine, 6.7%-mycophenolate mofetil. Treatment with biologics (B) was received by 13 (93.3%) pts (7-rituximab (RTM), 6-abatacept (ABA)) with a good response in 10 pts, including improvement in the function of the salivary and lacrimal glands in 7 pts. 1 patient received 2B-RTM and ABA sequentially due to the development of MAS 7 days after 1st RTM infusion. B was discontinued in 3 pts: 1 due to development of hemorrhagic vasculitis 2 days after the 1st RTM infusion, 1-COVID-19 with lung involvement (CT 3-4) 2 weeks after the 1st RTM infusion, 1-inefficiency of ABA during 15 months. Conclusion: In our pediatric rheumatologic department pts with pSS made up less than a quarter of all pts with SS. The diagnosis was verifed delayed in all pts, which can be explained by a wide range of nonspecifc manifestations at the onset. However, the manifestations of SS that were present at the time of diagnosis were brought under control on the background of complex therapy, including the prescription of B, with a good efficacy and safety profile of therapy.

10.
Annals of the Rheumatic Diseases ; 81:314-315, 2022.
Article in English | EMBASE | ID: covidwho-2008921

ABSTRACT

Background: Although the risk for severe COVID-19 progression in children is low, this may be aggravated by the underlying disease and/or immunosuppres-sive drugs. Objectives: We analyzed clinical data of COVID-19 cases among paediatric patients with rheumatic diseases reported to the BIKER registry. Methods: The main task of the German BIKER (Biologics in Pediatric Rheumatology) registry is to monitor the safety of biologics therapies in JIA. After the onset of the COVID-19 pandemic, the survey was expanded with a standardized form to proactively interview all participating centers about the occurrence, presentation, and outcome of SARS-CoV-2-infections in children with rheumatic diseases. Interviews were conducted with 68 centers initially weekly and later biweekly. Results: A total of 68 centres participated in the survey. Clinical data from 194 COVID-19 cases reported to the BIKER registry from 41 German and 1 Austrian pediatric rheumatology institutions between February 2020 and December 2021 were analyzed. Juvenile idiopathic arthritis (JIA, n=144) was the most common diagnosis followed by genetic autoinflammation (n=18;i.e. FMF, TRAPS, CAPS, HIDS, DADA2), systemic autoimmune diseases (n=11;i.e. SLE, dermatomyositis, vasculitis) and 16 with other rheumatic diseases (i.e. CRMO, Uveitis). 5 patients with no rheumatic disease were excluded. 104 (54%) patients were receiving conventional DMARDs, 81 (43%) received biologics, mainly TNF inhibitors (n=66 (35%)). Of the 189 rheumatic patients with SARS-CoV2 infection, 123 (63%) were female. The mean age was 12.4+/-4.4 years in females and 13.2+/-4.1 in males. The duration of SARS-Co2 infection associated symptoms was 13.8+/-15.3 days (max. 113 days), in 35 (43%) patients they lasted for > 12 days. 46 (24%) were asymptomatic. Patients with autoinflammation and systemic autoimmunopathies reported more symptoms such as fever, head and throat ache. 4 patients only complained about dyspnea. Only 3 patients were hospitalized and received Oxygen-supplementation. The only patients admitted to ICU, received ventilation but succumbed. This 3/-year-old patient, initially diagnosed with systemic JIA, developed fatal disease with intracranial edema and respiratory failure, as well as typical pulmonary texture changes. Prior to her SARS-CoV-2 infection, the patient was treated with MTX and low-dose steroids. Genetic testing revealed a so far unrecognized congenital immunodeficiency. In the total JIA cohort, treatment with corticosteroids, conventional DMARDs, biologics or combinations did not influence the number of reported symptoms or the favorable outcome of the cohort. However, the duration of symptoms was lower in the TNF-treated cohort (10.4+/-6.4 days vs. 15.7 +/-19.7 days). In the cohort with autoinflammation, fever was observed in 11 (61%). Those 6 who received IL-1-inhibitors did not show a different outcome than those 12 who did not. No case of PIMS/MISC in children with rheumatic diseases was reported. Conclusion: Except for one patient with congenital immunodefciency who died from her COVID-19 infection, no case of severe COVID-19 was reported in our cohort. At the time of infection, over 80% of patients in our cohort had been treated with conventional DMARDs and/or biologics. This did not appear to have a negative impact on the severity or outcome of SARS-CoV2 infection. Interestingly, no case of PIMS/MISC was observed.

11.
Annals of the Rheumatic Diseases ; 81:941-942, 2022.
Article in English | EMBASE | ID: covidwho-2008915

ABSTRACT

Background: Pediatric patients with a diagnosis of rheumatic disease are at increased risk for infectious diseases due to immune dysregulation arising fromthe disease itself, as well as immunoregulatory or immunosuppressive drugs they use. However, there are studies reporting that COVID-19 disease has a moderate course in children with rheumatic disease. Objectives: This study aims to determine the seroprevalence of SARS-CoV-2 in children with rheumatic disease during the COVID-19 pandemic, to evaluate the effects of immunosuppressive drugs and biological agents on seroprevalence of COVID. Methods: Between September 2020 and September 2021, patients aged 2-18 years with a diagnosis of and with a follow-up period of more than 6 months were included in the study. Patients were selected by 'simple random sampling' method. Anti-SARS-CoV-2 antibodies (IgG and IgA) against the S1 domain of the SARS-CoV-2 spike protein were investigated with a micro ELISA kit. Results: 170 patients, 92 (54.1%) girls, with a mean age of 12.16±4.18 years, were included. The median age at diagnosis was 7.59 (IQR 4.33-11.30) years, and the median follow-up time after diagnosis was 3.24 (IQR 1.87-5.99) years. Primary disease distribution was presented on Figure 1. The co-morbid diseases as obesity, hypertension and chronic kidney disease was present in 4.7%, 9.4% and 5.3% of patients, respectively. The distribution of medications was as follows;colchicine 101 patients (59.4%), Disease Modifying Anti-Rheumatism Drugs 49 (28.8%), steroid 25 (14.7%), and biological agents 28 (16.5%). Anti-SARS-CoV-2 Ig G antibody was positive in 40 (23.5%) of the patients. Demographic characteristics and treatment-related variables were similar between SARS-CoV-2 IgG seropositive and seronegative patients (Table 1). Thirty-eight (22.4%) of the patients had close contact with an individual diagnosed with COVID-19. Thirty two (18.9%) patients underwent PCR testing for SARS-CoV-2;only 4 (2.4%) had positive. Of these 32 patients, 28.1% were positive for IgG. IgG seropositivity was observed in 3 (75.0%) of 4 patients with PCR positivity. Conclusion: We found the SARS-CoV-2 seroprevalence of 23.5% in children with a diagnosis of rheumatic disease. It seems that primary rheumatological diagnosis does not affect COVID seropositivity in children.

12.
Annals of the Rheumatic Diseases ; 81:1729, 2022.
Article in English | EMBASE | ID: covidwho-2008891

ABSTRACT

Background: Multisystem Infammatory Syndrome in Children (MISC) is a hyper-infammatory state with similarities to Kawasaki Disease, 4 to 6 weeks after Covid-19 infection1. Literature describes a 11:1 Relative Risk for Asian children versus Caucasians2. Since the start of the pandemic, 17,699 children under 12 years were infected with Covid-193. Objectives: To describe presentation and short term outcomes, for a cohort of children with MIS-C at the sole Children's Hospital in Singapore. Methods: Demographic and clinical/lab data were collected from children diagnosed with MIS-C accrording to the WHO criteria4 at KK Woman's and Children's Hospital, Singapore. Nonparametric descriptive statistics were used to describe and analyse data. Results: Eleven patients were diagnosed with MIS-C between October 2021 and Jan 2022. Seven (64%) were male and 4 (36%) were Chinese, with median age at presentation was 8.08 years (IQR 4.54-9.79). All patients had positive COVID-19 serology at the time of diagnosis. Median duration of fever prior to diagnosis was 5 days (IQR 4-5);Nine (82%) had gastrointestinal symptoms and median number of Kawasaki Disease (KD) features were 2 (IQR 2-3.5);common manifestations were conjunctivitis (90%), red lips (55%) and rash (36%). Of note, 8 (70%) patients had KD type peeling on follow-up. No BCGitis was found during acute phase. Seven (64%) were admitted to higher dependency care. Table 1, all patient received IVIG and IV steroids;6 (55%) as pulse (30mg/kg/day) therapy. Patient 8, additionally received Anakinra. Median duration of admission was 6 days (IQR 5-13). One patient developed complications post therapy and was re-admitted to hospital for hematochezia. Treatment involved stopping Enoxaparin and Prednisone. Aspirin was resumed as soon as bleeding ceased. Laboratory characteristics and outcomes are denoted in Table 1. All patients had a monophasic course during the median of 10 weeks (IQR 8-11.5) of follow-up. Conclusion: 1.Asian prevalence of MIS-C is not as high as that reported from the West. Similarities in presentation as to age and gender were noted. 2.Most of our MIS-c patients developed periungual peeling at follow up, similarly to Kawasaki Disease. 3.Different from our typical KD population, no BCG site infammation was found.

13.
Annals of the Rheumatic Diseases ; 81:1414-1415, 2022.
Article in English | EMBASE | ID: covidwho-2008823

ABSTRACT

Background: Behçet's disease (BD) is an systemic infammatory vasculitis characterized by recurrent oral aphthae and genital ulcers. In the course of the disease, skin, eye, musculoskeletal, nervous system and gastrointestinal system involvements can be detected. Objectives: To evaluate the clinical, laboratory and radiological fndings of pedi-atric cases diagnosed with BD. Methods: Fifty patients (0-20 years old) who were followed up with the diagnosis of BD at the Pediatric Rheumatology outpatient clinic of Uludag University Faculty of Medicine between January 2011 and July 2021 were included in our study. The patients were diagnosed according to the diagnostic criteria of the International BD Study Group. Results: Twenty-four (48%) of the patients were male and 26 (52%) were female. The mean age at the diagnosis of patients was 9 ±4.55 years (10.75±4.55 years in boys, 12.35±3.65 years in girls). Twenty patients (36.3%) had a family history of Behçet's Disease. Oral aphthae were present in 96% (n=48) patients, while genital ulcers were in 32% (n=16) (Table 1). Of the nine patients with uveitis, 6 had panu-veitis, 2 had anterior uveitis and 1 had posterior uveitis. There was no difference in the distribution of symptoms according to the gender of the patients. HLA-B51 allel was found in 39 (78%) and ANA was positive in 14 patients (28%). Immunological tests showed that serum immunoglobulins were low in 11 (32.3%) of 34 patients. Low IgG levels were detected in 6 patients, low IgM levels were in 3, and low IgA levels were in 2. Thrombus was presented in three cases (thrombus in the right ventricle in one case, in the intracranial transverse sigmoid sinus and left jugular vein in two cases). The most commonly used drug for aphthae was colchicine (n=45, 82%). The use of biological agents according to patient manifestations is shown in Table 2. In the follow-up, clinical fndings improved in 35 patients (70%) Complete improvement was detected only with biological agents in 8 patients with uveitis. One patient was operated due to the development of complicated cataracts secondary to uveitis. Three patients were diagnosed with Covid-19, one of them was followed without treatment, while two of them were treated with favipiravir at home. Three patients with Covid-19 infection were using only colchicine treatment. Conclusion: Behçet's disease is rare in childhood. Although it is not common, life-threatening complications can be observed. To reduce morbidity and complications, physicians should be aware of manifestations and rare clinical pictures of the BD.

14.
Annals of the Rheumatic Diseases ; 81:1131, 2022.
Article in English | EMBASE | ID: covidwho-2008798

ABSTRACT

Background: During the COVID-19 (coronavirus) pandemic, some provision of healthcare shifted to remote, technology-assisted appointments (telemedicine). Whilst parents/carers of children and young people with rheumatic conditions have reported benefts of telemedicine, concerns remain. Objectives: This patient and parent-led project sought to understand the views of parents/carers about telemedicine, identifying the benefts and limitations of remote technology-assisted appointments, and comparing views between Canadian and European cohorts. Methods: An online survey was developed, translated into multiple languages and shared via social media and patient organisations, targeted at parents of children and young people with rheumatic, autoimmune and autoinfammatory conditions. Fieldwork took place in April 2021 in Europe and May 2021 in Canada. Consent was provided during enrollment. Results: A total of 290 responses were received (133 Europe;157 Canada). Of these, 73% were female, median age 12. Over half of respondents (53%) in Europe reported travelling over an hour to in-person appointments with their paediatric rheumatologist, compared to a signifcantly higher proportion of respondents in Canada (87%). Consequently, in-person appointments represent a greater time burden amongst Canadian car-egivers, though both groups report appointments taking over three hours in total (51% Europe, 69% Canada). Prior to COVID-19, most had never had a telemedicine appointment (92% Europe, 95% Canada). Since March 2020, the majority (71% Europe, 82% Canada) had at least one telemedicine appointment. When asked about aspects of telemedicine, a greater proportion of respondents from Canada answered favourably compared to those from Europe with the majority reporting telemedicine appointments had saved them time, enabled them to have an appointment and that it made the appointment safer. However, most felt that their consultant could not properly assess their child (72% Europe, 78% Canada, P<.05). Overall respondents said they would prefer the next appointment to be in-person (82% Europe, 62% Canada, p<.05), although 31% from Canada were amenable to a combination of in-person and telemedicine-based care. Conclusion: There are advantages to telemedicine, notably saving time and making appointments accessible. Families from Canada tended to view telemed-icine more favourably than those from Europe, although the majority from both cohorts reported concerns about the ability to assess their child. There may be value in providing training to parents to enhance the accuracy of home-based assessments, particularly when the disease is stable. However, parents continue to report the value of in-person appointments.

15.
Cardiology in the young ; : 1-3, 2022.
Article in English | MEDLINE | ID: covidwho-2008234

ABSTRACT

Multisystemic inflammatory syndrome in children is an inflammatory condition with multiorgan dysfunction that manifest late in the course of Severe acute respiratory syndrome coronavirus 2 infection. We present a 12-year-old boy with a history of fever, vomiting, diarrhoea, and abdominal pain. He developed shock with ventricular dysfunction and pericardial effusion. He was diagnosed with multisystemic inflammatory syndrome in children and treatment with intravenous immunoglobulins, corticosteroids, and tocilizumab proved to be ineffective. Eventually, the patient responded to cyclosporin-A treatment. Multisystemic inflammatory syndrome in children has been treated with immunoglobulins and glucocorticoids and in refractory cases biologics and cyclosporin-A have been used. Intravenous and oral cyclosporin-A seems to be a safe and effective alternative treatment for refractory multisystemic inflammatory syndrome in children patients.

16.
Indian journal of pediatrics ; 2022.
Article in English | MEDLINE | ID: covidwho-2007260

ABSTRACT

Multisystem inflammatory syndrome in children (MIS-C) occurs secondary to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. A retrospective study, involving 6 tertiary-care centers in Haryana, was conducted to evaluate the clinical features, severity, laboratory findings, and outcomes of patients with MIS-C. Disease severity was graded (mild/moderate/severe) and presence of cardiac abnormalities noted. Patients with and without cardiac abnormalities and with and without severe disease were compared. Forty-eight children with MIS-C were included (median age - 9.5 y). Fever (100%), gastrointestinal (83.3%) and mucocutaneous (50%) symptoms were common. Only 16.7% patients had previous history of documented SARS-CoV-2 infection/contact. Severe disease and cardiac abnormalities were seen in 47.9% and 54.2% patients, respectively. NT-proBNP > 1286.5 pg/mL and thrombocytopenia (≤ 119500/µL) were significant risk factors for severe MIS-C. Forty-five patients (93.8%) recovered and 3 died. Median hospitalization duration was 7 d (5-9.5). MIS-C must be considered as a possibility in any febrile child, even if a positive epidemiological history is absent. High NT-proBNP and thrombocytopenia are significant risk factors for severe MIS-C. (Trial Registration: The study was registered with the Clinical Trials Registry, India (CTRI/2021/09/036491)).

17.
Clinical Rheumatology ; 2022.
Article in English | EMBASE | ID: covidwho-2007160

ABSTRACT

Objective: In this study, it was aimed to evaluate the demographic, clinical and laboratory characteristics of MIS-C patients in our hospital, to share our treatment approach, and to assess the outcomes of short- and long-term follow-up. Methods: MIS-C patients who were admitted and treated in our hospital between July 2020 and July 2021 were evaluated. Demographic, clinical, laboratory, and follow-up data were collected from patient records retrospectively. Results: A total of 123 patients with MIS-C (median age, 9.6 years) were included the study. Nineteen (15.4%) were mild, 56 (45.6%) were moderate, and 48 (39%) were severe MIS-C. High CRP, ferritin, pro-BNP, troponin, IL-6, and D-dimer values were found in proportion to the severity of the disease (p < 0.001, p < 0.001, p < 0.001, p < 0.001, p = 0.005, p < 0.001), respectively. Two (1.6%) patients died. The mean follow-up period was 7.8 months. Valve failure, left ventricular dysfunction/hypertrophy, coronary involvement, and pericardial effusion were the most common cardiac pathologies in the short- and long-term follow-up of the patients. In the long-term follow-up, the most common reasons for admission to the hospital were recurrent abdominal pain (14.2%), cardiac findings (14.2%), pulmonary symptoms (8%), fever (7.1%), neuropsychiatric findings (6.2%) and hypertension (3.5%). Neuropsychiatric abnormalities were observed significantly more common in severe MIS-C patients at follow-up (p = 0.016). In the follow-up, 6.2% of the patients required recurrent hospitalization. Conclusion: MIS-C is a serious and life-threatening disease, according to short-term outcomes. In addition to the cardiac findings of patients with MIS-C, long-term outcomes such as neuropsychiatric findings, persistent gastrointestinal symptoms, fever and pulmonary symptoms should be monitored.Key Points• In MIS-C patients, attention should be paid not only to cardiac findings, but also to symptoms related to other systems.• Patients should be followed up in terms of neuropsychiatric findings, persistent gastrointestinal symptoms, fever and pulmonary symptoms that may occur during follow-up.

18.
Journal of Public Health in Africa ; 13:41-42, 2022.
Article in English | EMBASE | ID: covidwho-2006895

ABSTRACT

Introduction/ Background: Children have a lower susceptibility to SARS-CoV-2 infection than adults and experience less severe illness with 0.08% mortality. Since the emergence of new variants of concern, there are increased rates of infection across all age groups. This study aims to characterize COVID-19 among people under 18 years old in Tunisia. Methods: We conducted a national prospective study from March 02, 2020 to July 10, 2021. Data were collected by the National Observatory of New and Emerging Diseases via EPICOV and SARS-COV-2 database and concerned COVID-19 RT-PCR confirmed cases under 18 years. Viral mutations and variants in Tunisia, routinely monitored through a sequence-based surveillance using a representative sample of COVID- 19 patients in Tunisia, were also assessed. We did a descriptive analysis by gender, age group, region, outcome, and infecting sequence. We also calculated the cumulative incidence. Results: Out of 497,613 COVID-19 confirmed cases, 4.1% (n=20,434) were under 18. The average age was 12.5 +/- 5.1 years. Female were more affected than male (52.5% vs 47.5%). The cumulative incidence was 606,4/100,000 people under 18. The highest incidence rates occurred in weeks 1, 14 and 26 of 2021. Fifty-one children died from COVID-19, of which 67% were boys and 47% were under one year. Highest numbers were recorded during week 25 of 2021 (12%). Out of 208 samples sequenced, 41% (n=85) were VOC (84% Alpha, 15% Delta and 1% Beta). The Delta variant was mostly detected in Kairouan (62%). Impact: Our research describes COVID-19 among population under 18 years old since the beginning of the pandemic in Tunisia, which is important to know in order to orient preventive measures and adapt them according to the results. Conclusion: Our findings highlight that child COVID-19 is not uncommon, a majority being adolescents and with a high case-fatality rate. With the Delta variant, the control of cluster infections and preventive measures such as social distancing, hand wash, wearing mask and vaccination should be enhanced especially in family and school environment.

19.
Children Infections ; 21(2):51-56, 2022.
Article in Russian | EMBASE | ID: covidwho-2006683

ABSTRACT

Multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19 is a rare life-threatening immunopathological complication of COVID-19 that develops 1-6 weeks after the acute coronavirus infection. MIS-C is characterized by fever and multiorgan inflammation. We present a clinical case of a 10-year-old boy with skin lesions at the onset of MIS-C (erythematous malar rash, lacelike rash on the trunk and extremities and petechiae) with macrophage activation syndrome development and the early stage of primary Epstein-Barr virus infection (EBV infection) which required the exclusion of X-linked lymphoproliferative disease. This clinical case demonstrates the complexity of diagnosis in MIS-C with skin manifestations at the onset of the disease, especially with concurrent activation of other infections, particularly EBV infection.

20.
Current Medical Mycology ; 8(1):39-43, 2022.
Article in English | EMBASE | ID: covidwho-2006621

ABSTRACT

Background and Purpose: Disseminated fusariosis is an opportunistic infection caused by the hyaline fungus Fusarium spp. and occurs mainly in patients with leukemia. Case report: Two cases of disseminated fusariosis in pediatric patients are presented. Profound and prolonged neutropenia, fever, myalgia, and skin lesions in the legs were present in two girls with leukemia undergoing chemotherapy. In the first case, infection by Fusarium spp. was confirmed by anatomopathological findings, pathogen isolation, and polymerase chain reaction. In the second case, Fusarium solani infection was confirmed by mass spectrometry using blood cultures and skin lesion samples. Conclusion: It is important to consider disseminated fusariosis in high-risk patients who present with profound and prolonged neutropenia and persistent fever that does not resolve after broad-spectrum antibiotics to initiate antifungal therapy in a timely manner.

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