Valeur pronostique de l'élastase fécale chez les enfants mucoviscidosiques

Objectif - Évaluer chez des enfants mucoviscidosiques, l'influence de la fonction pancréatique externe, jugée par le résultat de l'élastase fécale, sur l'évolutivité de la maladie. Population et méthodes - travail rétrospectif. 231 enfants mucoviscidosiques, 62 suivis au Centre de Ressources et de Compétences de la Mucoviscidose (CRCM) du Centre Hospitalo-Universitaire d'Angers, 84 suivis à celui de Rennes ont été étudiés et 85 à celui de Nantes. Les 25 enfants suffisants pancréatiques externes (SPE) des 3 centres ont été comparés aux 50 enfants insuffisants pancréatiques externes (IPE) du même centre les plus proches en âge. Les caractéristiques de la maladie et les statuts nutritionnel, digestif et respiratoire ont été comparées entre les 2 groupes. Résultats - L'incidence de l'IPE était de 80%. Parmi les SPE, un était homozygote pour la mutation [delta]F508, 60% étaient hétérozygotes pour cette mutation et 36% n'étaient pas porteurs. Parmi les patients insuffisants pancréatiques, 52 % étaient homozygotes pour la mutation [delta]F508, 42% étaient hétérozygotes et 8% n'était pas porteur de cette mutation (p<0,001). De façon statistiquement significative, les SPE, comparés aux IPE, avaient un nombre de selles moins important (1,3 +- 0,6 vs 2,2 +- 1 par jour, p <0,01), un meilleur score de Brasfield (5 +- 4 vs 8+- 6, p<0,05), un z score de taille (102 +- 6 vs98 +- 6 p<0,01), un z score de BMI pour l'âge ( 99+- 12 vs 92 +- 8 p<0,001 ), un Volume Expiratoire Maximal Seconde (94 +- 15 vs 79 +- 20%, p<0,01) et une capacité vitale (95+- 13 vs 85 +- 16 p<0,05) pour l'âge moins abaissés. Conclusion - La SPE (taux d'élastase fécale supérieur à 200 mcg/g de selles), est associée à un meilleur état digestif, nutritionnel et respiratoire.ANGERS-BU Médecine-Pharmacie (490072105) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Serelaxin for infant heart failure in congenital dilated cardiomyopathy

Serelaxin has been studied in trials in adults with acute heart failure, but not in children. We report the first compassionate use of serelaxin in an infant. A 6-month-old girl with dilated cardiomyopathy was placed on extracorporeal membrane oxygenation following cardiac arrest unresponsive to medical treatment. Extracorporeal membrane oxygenation weaning failed despite maximal ino-dilator therapy. During the 48-hour infusion of serelaxin, we observed marked improvement in brain natriuretic peptide, left ventricular systolic function, and dilatation. The patient was successfully weaned from extracorporeal membrane oxygenation 24 hours later. The child died after a second extracorporeal membrane oxygenation run owing to sepsis

Comparison of inspiratory effort with three variable-flow nasal continuous positive airway pressure devices in preterm infants: a cross-over study

Objective: Patient's work of breathing may vary between different neonatal nasal continuous positive airway pressure (NCPAP) devices. Therefore, we aimed to compare the inspiratory effort of three variable-flow NCPAP delivery systems used in preterm infants. Design: Cross-over study. Patients/setting: From June 2015 to August 2016, 20 preterm infants weighing ≤2500 g requiring NCPAP for mild respiratory distress syndrome were enrolled. Interventions: Each patient was successively supported by three randomly assigned variable-flow NCPAP systems (MedinCNO, Infant Flow and Servo-i) for 20 min while maintaining the same continuous positive airway pressure level as the patient was on before the study period. Main outcome measures: Patients' inspiratory effort was estimated by calculating the sum of the difference between maximal inspiratory and baseline electrical activity of the diaphragm (∆EAdi) for 30 consecutive breaths, and after normalising this obtained value for the timing of the 30 breaths. Results: Physiological parameters (oxygen saturation measured by pulse oximetry, respiratory rate, heart beat, transcutaneous partial pressure CO2) and oxygen requirements remained identical between the three NCPAP systems. Although a wide variability in inspiratory effort could be observed, there were no statistically significant differences between the three systems for the sum of ∆EAdi for 30 breaths: CNO, 262 (±119) µV; IF, 352 (±262) µV; and SERVO-i, 286 (±126) µV, and the ∆EAdi reported on the timing of 30 breaths (sum ∆EAdi/s): CNO, 6.1 (±2.3) µV/s; IF, 7.9 (±4.9) µV/s; SERVO-i, 7.6 (±3.6) µV/s. Conclusion: In a neonatal population of preterm infants, inspiratory effort is comparable between the three tested modern variable-flow NCPAP devices.</p

Impact of ventilatory modes on the breathing variability in mechanically ventilated infants

Reduction of breathing variability is associated with adverse outcome. During mechanical ventilation, the variability of ventilatory pressure is dependent on the ventilatory mode. During neurally adjusted ventilatory assist (NAVA), the support is proportional to electrical activity of the diaphragm (EAdi), which reflects the respiratory center output. The variability of EAdi is, therefore, translated into a similar variability in pressures. Contrastingly, conventional ventilatory modes deliver less variable pressures. The impact of the mode on the patient's own respiratory drive is less clear. This study aims to compare the impact of NAVA, pressure-controlled ventilation (PCV), and pressure support ventilation (PSV) on the respiratory drive patterns in infants. We hypothesized that on NAVA, EAdi variability resembles most of the endogenous respiratory drive pattern seen in a control group

Pharmacokinetics, hemodynamic and metabolic effects of epinephrine to prevent post-operative low cardiac output syndrome in children.

International audienceINTRODUCTION: The response to exogenous epinephrine (Ep) is difficult to predict given the multitude of factors involved such as broad pharmacokinetic and pharmacodynamic between-subject variabilities, which may be more pronounced in children. We investigated the pharmacokinetics and pharmacodynamics of Ep, co-administered with milrinone, in children who underwent open heart surgical repair for congenital defects following cardiopulmonary bypass, including associated variability factors. METHODS: Thirty-nine children with a high risk of low cardiac output syndrome were prospectively enrolled. Ep pharmacokinetics, hemodynamic and metabolic effects were analyzed using the non-linear mixed effects modeling software MONOLIX. According to the final model, an Ep dosing simulation was suggested. RESULTS: Ep dosing infusions ranged from 0.01 to 0.23 mug.kg-1.min-1 in children whose weight ranged from 2.5 to 58 kg. A one-compartment open model with linear elimination adequately described the Ep concentration-time courses. Bodyweight (BW) was the main covariate influencing clearance (CL) and endogenous Ep production rate (q0) via an allometric relationship: CL(BWi) = thetaCL x (BWi)3/4 and q0(BWi) = thetaq0 x (BWi )3/4. The increase in heart rate (HR) and mean arterial pressure (MAP) as a function of Ep concentration were well described using an Emax model. The effect of age was significant on HR and MAP basal level parameters. Assuming that Ep stimulated the production rate of plasma glucose, the increases in plasma glucose and lactate levels were well described by turnover models without any significant effect of age, BW or exogenous glucose supply. CONCLUSIONS: According to this population analysis, the developmental effects of BW and age explained a part of the pharmacokinetic and pharmacodynamics between-subject variabilities of Ep administration in critically ill children. This approach ultimately leads to a valuable Ep dosing simulation which should help clinicians to determine an appropriate a priori dosing regimen

In situ simulation training for parental presence during critical situations in PICU: an observational study

Family presence during invasive procedures or cardiopulmonary resuscitation (CPR) is a part of the family-centered approach in pediatric intensive care units (PICUs). We established a simulation program aiming at providing communication tools to healthcare professionals. The goal of this study was to evaluate the impact of this program on the stress of PICU professionals and its acceptance. An observational study of a simulation program, with questionnaire, was used to measure pre- and post-simulation stress and the degree of satisfaction of the participants. PICU of Geneva Children's Hospital, Switzerland. Forty simulations with four different simulation scenarios and various types of parental behavior, as imitated by professional actors, were completed during a 1-year period. Primary outcomes were the difference in perceived stress level before and after the simulation and the degree of satisfaction of healthcare professionals (nursing assistants, nurses, physicians). The impact of previous experience with family members during critical situations or CPR was evaluated by variation in perceived stress level. Overall, 201 questionnaires were analyzed. Perceived stress associated with parental presence decreased from a pre-simulation value of 6 (IQR, 4-7) to 4 (IQR, 2-5) post-simulation on a scale of 1-10. However, in 25.7% of cases, the individually perceived post-simulation stress level was higher than the pre-simulation one. Satisfaction of the participants was high with a median of 10 (IQR, 9-10) out of 10. Conclusions: A simulation program helps reduce PICU team emotional stress associated with the presence of family members during critical situations or CPR, and is welcomed by PICU team members. What is known: • Family presence during cardiopulmonary resuscitation (CPR) or critical situations is a part of the family-centered approach in pediatric intensive care. • The benefits for the family have been already demonstrated. However, this policy is still controversy among healthcare professionals. What is new: • A simulation program seeking to provide skills focused on family presence management in the PICU is useful to reduce stress and was well accepted by participants. • It might become an indispensable training intervention for the implementation of a PICU policy to allow family presence during CPR or other critical situations.</p

Early and Late Postoperative Tachyarrhythmias in Children and Young Adults Undergoing Congenital Heart Disease Surgery

The population of patients with congenital heart disease is constantly growing with an increasing number of individuals reaching adulthood. A significant proportion of these children and young adults will suffer from tachyarrhythmias due to the abnormal anatomy, the hemodynamic burden, or as a sequela of surgical treatment. Depending on the underlying mechanism, arrhythmias may arise in the early postoperative period (hours to days after surgery) or in the late postoperative period (usually years after surgery). A good understanding of the electrophysiological characteristics and pathophysiological mechanisms is therefore crucial to guide the therapeutic approach. Here, we synthesize the current state of knowledge on epidemiological features, risk factors, pathophysiological insights, electrophysiological features, and therapy regarding tachyarrhythmias in children and young adults undergoing reparative surgery for congenital heart disease. The evolution and latest data on treatment options, including pharmacological therapy, ablation procedures, device therapy decision, and thromboprophylaxis, are summarized. Finally, throughout this comprehensive review, knowledge gaps and areas for future research are also identified

Immunological assessment of pediatric multisystem inflammatory syndrome related to COVID-19

Recently, cases of multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19 have been reported worldwide. Negative RT-PCR testing associated with positive serology in most cases suggests a post-infectious syndrome. Because the pathophysiology of this syndrome is still poorly understood, extensive virological and immunological investigations are needed