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

Interest of high‑flow nasal cannula (HFNC) versus nasal continuous positive airway pressure (nCPAP) during the initial management of severe bronchiolitis in infants: a multicenter randomized controlled trial

International audienceIntroduction The aim of this study was to evaluate the long-term effects of the implementation of “good laboratory test practices” pro-cedure on blood test prescription, cost cutting laboratory test and patient outcome and red cells transfusion needs. In addition, we looked for factors associated with transfusion.Patients and methods This is a retrospective, single-center study.Considering that prescription of laboratory tests was excessive in our ICU, we developed in 2009 a procedure aiming at limiting assays and blood sampling. All adult patients over 18 years admitted in the ICU from January 1, 2008, to December 31, 2012, were consecutively included. For each patient, the following data were retrospectively obtained from medical records and institutional databases: demo-graphic and severity of illness parameters, the amount and costs of laboratory tests, daily blood volume drawn, number of red cells trans-fusions and ICU and in-hospital outcomes.Results A total of 3568 patients were included between January 1, 2008, and December 31, 2012. Patient’s characteristics were similar during the study with respect to overall severity and vital organ sup-port, except the need for vasoactive drugs which increased across time. After implementation of guidelines, the total number of labora-tory tests decreased from 78,406 in 2008 (18.1 tests/patient/day) to 27,514 in 2012 (6.4 tests/patient/day). 318,000€ were saved in 2012 on the five more expensive tests as compared to 2008. Average daily blood drawn volume decreased from 29.2 ± 13 ml/day in 2008 to 22.4 ± 10.1 ml/day (p < 0.001) in 2012 in the whole population. The decline was larger for transfused patients (33.8 ± 15.6 ml/day in 2008 to 23.6 ± 10.7 ml/day in 2012 p < 0.001). We observed a nonsignifi-cant reduction in PRBC administered after the institution of guidelines. Hemoglobin threshold for blood transfusion remained unchanged over time. There was a significant relationship between daily blood volume loss and blood transfusion [OR 1.012 per ml volume drawn CI (1.009–1.014), p < 0.001], but mechanical ventilation, renal replace-ment, invasive monitoring and use of vasoactive drugs were more potent independent factors for transfusion. ICU and in-hospital mor-tality did not change across time (p= 0.367 and p= 0.361)Conclusion Guidelines of laboratory tests prescription led to a sus-tained reduction in the number of laboratory tests and related costs, the average daily blood volume drawn, without changing the ICU and in-hospital mortality. Although there was a correlation between the amount of blood sampled and the transfusion of PRBC, the number of patients transfused and the amount of red blood cells transfused did not significantly decrease

High flow nasal cannula (HFNC) versus nasal continuous positive airway pressure (nCPAP) for the initial respiratory management of acute viral bronchiolitis in young infants: a multicenter randomized controlled trial (TRAMONTANE study)

International audiencePURPOSE:Nasal continuous positive airway pressure (nCPAP) is currently the gold standard for respiratory support for moderate to severe acute viral bronchiolitis (AVB). Although oxygen delivery via high flow nasal cannula (HFNC) is increasingly used, evidence of its efficacy and safety is lacking in infants.METHODS:A randomized controlled trial was performed in five pediatric intensive care units (PICUs) to compare 7 cmH2O nCPAP with 2 L/kg/min oxygen therapy administered with HFNC in infants up to 6 months old with moderate to severe AVB. The primary endpoint was the percentage of failure within 24 h of randomization using prespecified criteria. To satisfy noninferiority, the failure rate of HFNC had to lie within 15% of the failure rate of nCPAP. Secondary outcomes included success rate after crossover, intubation rate, length of stay, and serious adverse events.RESULTS:From November 2014 to March 2015, 142 infants were included and equally distributed into groups. The risk difference of -19% (95% CI -35 to -3%) did not allow the conclusion of HFNC noninferiority (p = 0.707). Superiority analysis suggested a relative risk of success 1.63 (95% CI 1.02-2.63) higher with nCPAP. The success rate with the alternative respiratory support, intubation rate, durations of noninvasive and invasive ventilation, skin lesions, and length of PICU stay were comparable between groups. No patient had air leak syndrome or died.CONCLUSION:In young infants with moderate to severe AVB, initial management with HFNC did not have a failure rate similar to that of nCPAP. This clinical trial was recorded in the National Library of Medicine registry (NCT 02457013)

A multicenter randomized controlled trial of a 3-L/kg/min versus 2-L/kg/min high-flow nasal cannula flow rate in young infants with severe viral bronchiolitis (TRAMONTANE 2)

High-flow nasal cannula (HFNC) therapy is increasingly proposed as first-line respiratory support for infants with acute viral bronchiolitis (AVB). Most teams use 2 L/kg/min, but no study compared different flow rates in this setting. We hypothesized that 3 L/kg/min would be more efficient for the initial management of these patients

Assessment and management of iatrogenic withdrawal syndrome and delirium in pediatric intensive care units across Europe:An ESPNIC survey

Introduction: Analgesia and sedation are essential for the care of children in the pediatric intensive care unit (PICU); however, when prolonged, they may be associated with iatrogenic withdrawal syndrome (IWS) and delirium. We sought to evaluate current practices on IWS and delirium assessment and management (including non-pharmacologic strategies as early mobilization) and to investigate associations between the presence of an analgosedation protocol and IWS and delirium monitoring, analgosedation weaning, and early mobilization. Methods: We conducted a multicenter cross-sectional survey-based study collecting data from one experienced physician or nurse per PICU in Europe from January to April 2021. We then investigated differences among PICUs that did or did not follow an analgosedation protocol. Results: Among 357 PICUs, 215 (60%) responded across 27 countries. IWS was systematically monitored with a validated scale in 62% of PICUs, mostly using the Withdrawal Assessment Tool-1 (53%). The main first-line treatment for IWS was a rescue bolus with interruption of weaning (41%). Delirium was systematically monitored in 58% of PICUs, mostly with the Cornell Assessment of Pediatric Delirium scale (48%) and the Sophia Observation Scale for Pediatric Delirium (34%). The main reported first-line treatment for delirium was dexmedetomidine (45%) or antipsychotic drugs (40%). Seventy-one percent of PICUs reported to follow an analgosedation protocol. Multivariate analyses adjusted for PICU characteristics showed that PICUs using a protocol were significantly more likely to systematically monitor IWS (odds ratio [OR] 1.92, 95% confidence interval [CI] 1.01–3.67) and delirium (OR 2.00, 95% CI 1.07–3.72), use a protocol for analgosedation weaning (OR 6.38, 95% CI 3.20–12.71) and promote mobilization (OR 3.38, 95% CI 1.63–7.03). Conclusions: Monitoring and management of IWS and delirium are highly variable among European PICUs. The use of an analgosedation protocol was associated with an increased likelihood of monitoring IWS and delirium, performing a structured analgosedation weaning and promoting mobilization. Education on this topic and interprofessional collaborations are highly needed to help reduce the burden of analgosedation-associated adverse outcomes.</p

Place de l’analyse des gènes du surfactant dans la démarche diagnostique des pneumopathies interstitiellesdiffuses de l’enfant et l’adulte

National audienceIntroductionLes pneumopathies interstitielles diffuses (PID) de l’enfant et l’adulte regroupent des pathologies hétérogènes et sévères. Une cause génétique est identifiée pour 2 à 20% des patients. Les gènes le plus souvent en cause sont ceux du complexe des télomérases, suivis des gènes du système du surfactant alvéolaire, qui sont communément associées à des formes pédiatriques de PID. Cette étude a pour but de rechercher des mutations des gènes du surfactant, sans a priori d’âge, dans une large cohorte prospective de PID.Matériel et méthodesLes patients ont été recrutés et inclus de façon prospective au sein de la filière de soins pour les maladies respiratoires rares RespiFIL. Les patients présentant des mutations des gènes du complexe des télomérases ont été exclus. Les exons et lesrégions introniques flanquantes des gènes SFTPA1, SFTPA2, SFTPB, SFTPC, ABCA3, NKX2-1 ont été séquencés. Les mutations retrouvées dans ces gènes ont été étudiées in silico et des tests fonctionnels sont en cours. Parallèlement, les données cliniques des patients ont été recueillies à l’aide d’une fiche standardisée.RésultatsEn 4 ans, 477 patients indépendants ont été inclus (190 enfants et 287 adultes). L’âge moyen au diagnostic de PID était de 33 ans (0-100), et le sexe ratio (hommes/femmes) de 1,47. La PID était familiale pour 22% des patients, et un antécédent personnel ou familial de cancer pulmonaire était retrouvé chez 44 patients. Une mutation dans l’un des gènes du système du surfactant a été identifiée chez 63 (13%) patients, dont 31 (16%) enfants et 32 (11%) adultes. Excepté les 2 mutations qui ont été identifiées dans SFTPB et retrouvées seulement chez l’adulte, tous les gènes étudiés ont été impliqués chez des adultes et des enfants.Discussion et conclusionContrairement aux idées reçues, les gènes du surfactant pulmonaire sont impliqués dans les PID à tout âge. Leur analyse semble donc indispensable à la démarche étiologique des PID, et ce sans a priori d’âge. La recherche systématique de mutations dans ces gènes, associée à une description précise du phénotype, permettra de progresser dans la description de ces maladies rares

Place de l’analyse des gènes du surfactant dans la démarche diagnostique des pneumopathies interstitiellesdiffuses de l’enfant et l’adulte

National audienceIntroductionLes pneumopathies interstitielles diffuses (PID) de l’enfant et l’adulte regroupent des pathologies hétérogènes et sévères. Une cause génétique est identifiée pour 2 à 20% des patients. Les gènes le plus souvent en cause sont ceux du complexe des télomérases, suivis des gènes du système du surfactant alvéolaire, qui sont communément associées à des formes pédiatriques de PID. Cette étude a pour but de rechercher des mutations des gènes du surfactant, sans a priori d’âge, dans une large cohorte prospective de PID.Matériel et méthodesLes patients ont été recrutés et inclus de façon prospective au sein de la filière de soins pour les maladies respiratoires rares RespiFIL. Les patients présentant des mutations des gènes du complexe des télomérases ont été exclus. Les exons et lesrégions introniques flanquantes des gènes SFTPA1, SFTPA2, SFTPB, SFTPC, ABCA3, NKX2-1 ont été séquencés. Les mutations retrouvées dans ces gènes ont été étudiées in silico et des tests fonctionnels sont en cours. Parallèlement, les données cliniques des patients ont été recueillies à l’aide d’une fiche standardisée.RésultatsEn 4 ans, 477 patients indépendants ont été inclus (190 enfants et 287 adultes). L’âge moyen au diagnostic de PID était de 33 ans (0-100), et le sexe ratio (hommes/femmes) de 1,47. La PID était familiale pour 22% des patients, et un antécédent personnel ou familial de cancer pulmonaire était retrouvé chez 44 patients. Une mutation dans l’un des gènes du système du surfactant a été identifiée chez 63 (13%) patients, dont 31 (16%) enfants et 32 (11%) adultes. Excepté les 2 mutations qui ont été identifiées dans SFTPB et retrouvées seulement chez l’adulte, tous les gènes étudiés ont été impliqués chez des adultes et des enfants.Discussion et conclusionContrairement aux idées reçues, les gènes du surfactant pulmonaire sont impliqués dans les PID à tout âge. Leur analyse semble donc indispensable à la démarche étiologique des PID, et ce sans a priori d’âge. La recherche systématique de mutations dans ces gènes, associée à une description précise du phénotype, permettra de progresser dans la description de ces maladies rares