Cardiac manifestations of MIS-C: cardiac magnetic resonance and speckle-tracking data

BackgroundCardiac involvement is central in MIS-C and represents the main cause of morbidity. In this study, we aimed to assess myocardial damage in patients with MIS-C using cardiac magnetic resonance (CMR) during the acute phase, as well as left ventricular and atrial longitudinal strain on admission, at discharge, and after 3 months.MethodsWe performed a single-center prospective cohort study and case–control study. Between September 2020 and February 2022, we enrolled 39 patients hospitalized for MIS-C at our center. We performed left ventricular and atrial longitudinal 2D strain analysis on admission and during follow-up; echocardiographic data were compared to a matched control population. Patients above 4 years old with increased troponin underwent CMR.ResultsOf 24 patients (mean age: 8.2 ± 4.9 years) who underwent CMR, 14 (58%) presented myocardial edema and 6 (25%) late gadolinium enhancement (LGE). LGE was associated with older age (p < 0.01), increased BMI (p = 0.03), increased ferritin levels (p < 0.001), lower left ventricular (LV) ejection fraction (p < 0.001), LV longitudinal strain (p = 0.004), left atrial (LA) strain (p = 0.05), and prolonged hospital stay (p = 0.02). On admission, LV ejection fraction, LV longitudinal strain, and LA strain were impaired, but each improved gradually over time; LVEF was the fastest to recover, while global LV longitudinal strain was still impaired as compared to controls after 3 months (p = 0.01).ConclusionOur study demonstrates that myocardial injury is present in a quarter of MIS-C patients, and impaired LA and LV myocardial deformation persist for at least several weeks after the acute phase. CMR and LV/LA strain could help us to individualize follow-up of MIS-C patients

Health-related quality of life of food-allergic children compared with healthy controls and other diseases

Background Food allergy is a potentially life-threatening disease, affecting up to 10% of the pediatric population. Objective The aim of our study was to assess the health-related quality of life (HRQL) of food-allergic patients compared with the general population and patients with other chronic diseases with dietary or allergic burden, in a cross-sectional study. Methods We recruited patients aged 8-17 years diagnosed with food allergy and matched healthy controls recruited in schools. We also included patients with asthma, inflammatory bowel disease, celiac disease, diabetes, obesity, and eating disorders. We used the CHQ-CF87 questionnaire for generic HRQL assessment. Food allergy HRQL was also assessed using specific questionnaires: Food Allergy Quality of Life Questionnaire (FAQLQ) and Food Allergy Independent Measure (FAIM). Results One hundred and thirty-five food-allergic children, 255 children with chronic diseases, and 463 healthy controls were included in the analyses. Food-allergic patients had a better HRQL than healthy controls in the Behavior (BE), Bodily Pain (BP), Family Activities (FA), and Mental Health (MH) domains and a worse HRQL in the General Health Perception (GH) domain (p = .048). Food-allergic patients exhibited a better HRQL than patients affected by other chronic diseases, notably diabetes. Although an epinephrine autoinjector had been prescribed to 87.4% of the food-allergic children, only 54.2% of them carried it at all times. Conclusion Food-allergic patients display overall good HRQL compared with the general population and those with other diseases with daily symptoms and treatments, in line with recent improvements in food allergy management

ROHHAD syndrome without rapid-onset obesity: A diagnosis challenge

peer reviewedBackgroundROHHAD syndrome (Rapid-onset Obesity with Hypothalamic dysfunction, Hypoventilation and Autonomic Dysregulation) is rare. Rapid-onset morbid obesity is usually the first recognizable sign of this syndrome, however a subset of patients develop ROHHAD syndrome without obesity. The prevalence of this entity is currently unknown. Alteration of respiratory control as well as dysautonomic disorders often have a fatal outcome, thus early recognition of this syndrome is essential.Material and methodsA retrospective, observational, multicenter study including all cases of ROHHAD without rapid-onset obesity diagnosed in France from 2000 to 2020.ResultsFour patients were identified. Median age at diagnosis was 8 years 10 months. Median body mass index was 17.4 kg/m2. Signs of autonomic dysfunction presented first, followed by hypothalamic disorders. All four patients had sleep apnea syndrome. Hypoventilation led to the diagnosis. Three of the four children received ventilatory support, all four received hormone replacement therapy, and two received psychotropic treatment. One child in our cohort died at 2 years 10 months old. For the three surviving patients, median duration of follow-up was 7.4 years.ConclusionROHHAD syndrome without rapid-onset obesity is a particular entity, appearing later than ROHHAD with obesity. This entity should be considered in the presence of dysautonomia disorders without brain damage. Likewise, the occurrence of a hypothalamic syndrome with no identified etiology requires a sleep study to search for apnea and hypoventilation. The identification of ROHHAD syndrome without rapid-onset obesity is a clinical challenge, with major implications for patient prognosis

Development of Core Outcome Measures sets for paediatric and adult Severe Asthma (COMSA)

BACKGROUND: Effectiveness studies with biological therapies for asthma lack standardised outcome measures. The COMSA (Core Outcome Measures sets for paediatric and adult Severe Asthma) working group sought to develop Core Outcome Measures (COM) sets to facilitate better synthesis of data and appraisal of biologics in paediatric and adult asthma clinical studies.METHODS: COMSA utilised a multi-stakeholder consensus process among patients with severe asthma, adult, and paediatric clinicians, pharmaceutical representatives and health regulators from across Europe. Evidence included a systematic review of development, validity, and reliability of selected outcome measures plus a narrative review and a pan-European survey to better understand patients' and carers' views about outcome measures. It was discussed using a modified GRADE Evidence to Decision framework. Anonymous voting was conducted using predefined consensus criteria.RESULTS: Both adult and paediatric COM sets include forced expiratory volume in 1 s (FEV1) as z scores, annual frequency of severe exacerbations and maintenance oral corticosteroid use. Additionally, the paediatric COM set includes the Paediatric Asthma Quality of Life Questionnaire, and Asthma Control Test (ACT) or Childhood-ACT while the adult COM includes the Severe Asthma Questionnaire and the Asthma Control Questionnaire-6 (symptoms and rescue medication use reported separately).CONCLUSIONS: This patient-centred collaboration has produced two COM sets for paediatric and adult severe asthma. It is expected that they will inform the methodology of future clinical trials, enhance comparability of efficacy and effectiveness of biological therapies, and help assess their socioeconomic value. COMSA will inform definitions of non-response and response to biological therapy for severe asthma.</p

Update on current views and advances on RSV infection (Review).

Respiratory syncytial virus (RSV) infection represents an excellent paradigm of precision medicine in modern paediatrics and several clinical trials are currently performed in the prevention and management of RSV infection. A new taxonomic terminology for RSV was recently adopted, while the diagnostic and omics techniques have revealed new modalities in the early identification of RSV infections and for better understanding of the disease pathogenesis. Coordinated clinical and research efforts constitute an important step in limiting RSV global predominance, improving epidemiological surveillance, and advancing neonatal and paediatric care. This review article presents the key messages of the plenary lectures, oral presentations and posters of the '5th workshop on paediatric virology' (Sparta, Greece, 12th October 2019) organized by the Paediatric Virology Study Group, focusing on recent advances in the epidemiology, pathogenesis, diagnosis, prognosis, clinical management and prevention of RSV infection in childhood

Genetic Basis of Myocarditis: Myth or Reality?

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Evaluation de la connaissance des recommandations de l'AFSSAPS concernant la prise en charge de la toux aigue du nourrisson auprès des médecins, familles et pharmacie de la région

NICE-BU Médecine Odontologie (060882102) / SudocSudocFranceF

Publishing in pandemic times: A bibliometric analysis of early medical publications on Kawasaki-like disease (MIS-C, PIMS-TS) related to SARS-CoV-2

International audienceIntroduction: At the end of April 2020, three European pediatric societies published an alert on a new hyperinflammatory disorder linked to SARS-CoV-2. This disease has alternatively been called Kawasaki-like disease, pediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 infection (PIMS-TS), and multisystem inflammatory syndrome in children (MIS-C). These alerts provide a clear starting point from which to study the early response of the medical and scientific community to a new disease in terms of scientific publications, and to compare the timeline of this response with levels of general public interest. To this aim, we conducted a bibliometric analysis of articles on this disease published between 1 April and 5 July 2020.Method: A literature search was performed using PubMed and in three preprint repositories. For each article, the name used for the disease in the title, the number of authors, the number of patients, the citations according to Google Scholar, the journal impact factor, and the Altmetric score were retrieved. Google search trends for the terms "Kawasaki" and "COVID," "COVID-19," and "coronavirus" were also retrieved, as was the number of Reuters news articles published on the topic. These data were compared longitudinally on a weekly basis. The quality of the reporting of the study was evaluated using the STROBE guidelines for observational studies with more than three patients and using the CARE guidelines for case reports of three or fewer patients.Results: Eighty-six articles were included, among which ten were preprints (three of which were subsequently published) and 49 were clinical articles (57%). A total of 857 patients were described. The median number of authors per article was five (range, 1-45), the median number of patients was four (1-186), the median number of citations was one (0-170), the median Altmetric score was 12 (0-7242), and the median journal impact factor was 3.7 (1-74.7). For the clinical articles, the median percentage of STROBE or CARE checklist items satisfied was 70% (IQR, 56.75-79.25; range, 40-90). Guideline adherence was significantly higher for observational studies than for case reports (median percentage of checklist items satisfied, 78.5% vs 61.5%; P<0.001); however, guideline adherence did not differ significantly between peer-reviewed and preprint articles (median percentage of checklist items satisfied, 57% vs 72%; P=0.205). The only statistically significant difference between clinical articles and other types of articles was the number of authors (median, 7 vs 2; P=2.53E-9). Fifty-seven of the 86 articles were authored by researchers from just three countries (the USA, 31; France, 14; and the UK, 12). The names most frequently used in the title were Kawasaki-like disease (n=37), followed by MIS-C (n=27), PIM-TS (n=14), and other names involving the term "inflammatory" (n=12). Google searches for related terms peaked between weeks 18 and 21, following the initial alerts and decreased rapidly thereafter. The number of Reuters articles on the subject was correlated with Google search trends (ρ: 0.86, 95% CI [0.59; 0.96]; P=0.00016), but the number of medical articles published was not (ρ: -0.54, 95% CI [-0.87; 0.14]; P=0.11). The first small case series was published less than 2 weeks after the initial alert; however, if all articles had been deposited as preprints when they were submitted to journals, the cumulative number of reported cases would have been 300% higher in week 18 (3 vs 1), 400% higher in week 19 (44 vs 11), 70% higher in week 20 (124 vs 73), and 54% higher in week 21 (129 vs 84).Conclusion: In a period of 9 weeks after the initial alerts from European pediatric societies, 85 medical articles were published, involving 856 patients (one case report was published before the alerts), allowing rapid dissemination of research information. However, general public interest followed the news cycle rather than scientific releases. The quality of the reporting, as assessed by adherence to STROBE or CARE guidelines, was adequate with more than two-thirds of checklist items satisfied. Learned societies play an important role in the early dissemination of up-to-date peer-reviewed information. Preprint deposition should be encouraged to accelerate the dissemination of research information