Systemic corticosteroids for the treatment of acute episodes of rhabdomyolysis in lipin-1-deficient patients.

Mutations in the LPIN1 gene constitute a major cause of severe rhabdomyolysis (RM). The TLR9 activation prompted us to treat patients with corticosteroids in acute conditions. In patients with LPIN1 mutations, RM and at-risk situations that can trigger RM have been treated in a uniform manner. Since 2015, these patients have also received intravenous corticosteroids. We retrospectively compared data on hospital stays by corticosteroid-treated patients vs. patients not treated with corticosteroids. Nineteen patients were hospitalized. The median number of admissions per patient was 21 overall and did not differ when comparing the 10 corticosteroid-treated patients with the 9 patients not treated with corticosteroids. Four patients in the non-corticosteroid group died during a RM (mean age at death: 5.6 years). There were no deaths in the corticosteroid group. The two groups did not differ significantly in the number of RM episodes. However, for the six patients who had RM and occasionally been treated with corticosteroids, the median number of RM episodes was significantly lower when intravenous steroids had been administered. The peak plasma creatine kinase level and the area under the curve were or tended to be higher in patients treated with corticosteroids-even after the exclusion of deceased patients or focusing on the period after 2015. The median length of stay (10 days overall) was significantly longer for corticosteroid-treated patients but was similar after the exclusion of deceased patients. The absence of deaths and the higher severity of RM observed among corticosteroid-treated patients could suggest that corticotherapy is associated with greater survival.

Antimicrobial stewardship programs in European pediatric intensive care units: an international survey of practices.

Antibiotic therapy represents one of the most common interventions in pediatric intensive care units (PICUs). This study aims to describe current antimicrobial stewardship programs (ASP) in European PICUs. A cross-sectional survey distributed to European pediatric intensive care physicians through the European Society of Neonatal and Pediatric Intensive Care (ESPNIC) Infection, Inflammation, and Sepsis Section, to members of the Spanish Society of Pediatric Intensive Care, of the Pediatric Reanimation and Emergency Care French Group, and to European physicians known to be involved in antimicrobial stewardship programs. Responses from 60 PICUs across 12 countries were analyzed. Fifty three (88%) stated that ASP was implemented. The main interventions considered as ASP were the pharmacokinetic monitoring of antimicrobials (n = 41, 77%) and the development of facility-specific clinical practice guidelines (n = 40, 75%). The most common team composition of antimicrobial stewardship program included a pediatric infectious disease physician, a pharmacist, and a microbiologist (n = 11, 21%). CONCLUSION:  Although ASP practices were reported to be widely implemented across European PICUs, this survey observed a large heterogeneity in terms of activities and modalities of intervention. WHAT IS KNOWN: • Antibiotic therapy represents one of the most common interventions in pediatric intensive care units. • The role and subsequent success of antimicrobial stewardship programs has largely been reported in the adult population but scarcely in the pediatric population. WHAT IS NEW: • Antimicrobial stewardship programs were reported to be widely implemented across European pediatric intensive care units. • We observed a large heterogeneity in terms of activities and modalities of intervention.

Prevalence and risk factors of discomfort in infants with severe bronchiolitis.

AIM: The aim of this study was to assess the prevalence of discomfort in infants with severe bronchiolitis supported by noninvasive ventilation and to identify its potential risk factors. METHODS: A single-centre retrospective observational study. Discomfort was assessed using the EDIN (Echelle de Douleur et d'Inconfort du Nouveau-né) scale. RESULTS: Ninety-one infants (median age 34 days [Interquartile IQR 19-55], 52 (57%) boys) were included in our study. Overall, no patient had a mean EDIN score higher than 8 on Days 1, 2 and 3. On Days 1 and 2, patients supported by bilevel positive airway pressure (BiPAP) had a higher EDIN score compared with other patients (3.3 [SD 2.5] versus 2.6 [SD 2.2] on Day 1 and 2.9 (SD 2.1) versus 2.3 (SD 2.2) on Day 2, both p < 0.001). CONCLUSION: Patients with severe bronchiolitis and supported by any type of noninvasive ventilation had a low degree of discomfort during the first 3 days of ICU stay. Patients requiring bilevel noninvasive ventilation appeared to have a higher degree of discomfort, while we found no correlation between the level of discomfort and the degree of respiratory distress.

Cerebral vasospasm in children with subarachnoid hemorrhage: frequency, diagnosis, and therapeutic management.

BACKGROUND: The present study explores the frequency, diagnostic approach, and therapeutic management of cerebral vasospasm in a cohort of children with moderate-to-severe traumatic and nontraumatic subarachnoid hemorrhage (SAH). METHODS: This was a single-center retrospective study performed over a 10-year period, from January 2010 to December 2019. Children aged from one month to 18 years who were admitted to the pediatric or adult intensive care unit with a diagnosis of SAH were eligible. Cerebral vasospasm could be suspected by clinical signs or transcranial Doppler (TCD) criteria (mean blood flow velocity > 120 cm/s or an increase in mean blood flow velocity by > 50 cm/s within 24 h) and then confirmed on cerebral imaging (with a reduction to less than 50% of the caliber of the cerebral artery). RESULTS: Eighty patients aged 8.6 years (3.3-14.8 years, 25-75th centiles) were admitted with an initial Glasgow Coma Scale score of 8 (4-12). SAH was nontraumatic in 21 (26%) patients. A total of 14/80 patients (18%) developed cerebral vasospasm on brain imaging on day 6 (5-10) after admission, with a predominance of nontraumatic SAH (12/14). The diagnosis of cerebral vasospasm was suspected on clinical signs and/or significant temporal changes in TCD monitoring (7 patients) and then confirmed on cerebral imaging. Thirteen of 14 patients with vasospasm were successfully treated using a continuous intravenous infusion of milrinone. The Pediatric Cerebral Performance Category score at discharge from the intensive care unit was comparable between children with vasospasm (score of 2 [1-4]) vs. children without vasospasm (score of 4 [2-4]) (p = 0.09). CONCLUSIONS: These findings indicate that cerebral vasospasm exists in pediatrics, particularly after nontraumatic SAH. The use of TCD and milrinone may help in the diagnostic and therapeutic management of cerebral vasospasm.

Clinical applications of transcranial Doppler in non-trauma critically ill children: a scoping review.

BACKGROUND: Many applications of transcranial Doppler (TCD) as a diagnosis or monitoring tool have raised interest in the last decades. It is important that clinicians know when and how to perform TCD in this population, what parameter to assess and monitor and how to interpret it. OBJECTIVE: This review aims to describe the emerging clinical applications of TCD in critically ill children excluding those suffering from trauma. METHODS: Databases Web of Science, Cochrane and PubMed were searched in May 2020. We considered all publications since the year 2000 addressing the use of TCD as a prognostic, diagnostic or follow-up tool in children aged 0 to 15 years admitted to intensive care or emergency units, excluding neonatology and traumatic brain injury. Two independent reviewers selected 82 abstracts and full-text articles from the 2011 unique citations identified at the outset. RESULTS: TCD provides crucial additional information at bedside about cerebrovascular hemodynamics. Many clinical applications include the diagnosis and management of various medical and surgical neurologic conditions (central nervous system infections, arterial ischemic stroke, subarachnoid hemorrhage and vasospasm, brain death, seizures, metabolic disease, hydrocephalus) as well as monitoring the impact systemic conditions on brain perfusion (hemodynamic instability, circulatory assistance). CONCLUSION: To conclude, TCD has become an invaluable asset for non-invasive neuromonitoring in critically ill children excluding those suffering from trauma. However, the scope of TCD remains unclearly defined yet and reference values in critically ill children are still lacking.

Traumatic brain injury in children with thoracic injury: clinical significance and impact on ventilatory management.

PURPOSE: This study aims to describe the epidemiology and management of chest trauma in our center, and to compare patterns of mechanical ventilation in patients with or without associated moderate-to-severe traumatic brain injury (TBI). METHODS: All children admitted to our level-1 trauma center from February 2012 to December 2018 following chest trauma were included in this retrospective study. RESULTS: A total of 75 patients with a median age of 11 [6-13] years, with thoracic injuries were included. Most patients also had extra-thoracic injuries (n = 71, 95%) and 59 (79%) had TBI. A total of 52 patients (69%) were admitted to intensive care and 31 (41%) were mechanically ventilated. In patients requiring mechanical ventilation, there was no difference in tidal volume or positive end-expiratory pressure in patients with moderate-to-severe TBI when compared with those with no-or-mild TBI. Only one patient developed Acute Respiratory Distress Syndrome. A total of 6 patients (8%) died and all had moderate-to-severe TBI. CONCLUSION: In this small retrospective series, most patients requiring mechanical ventilation following chest trauma had associated moderate-to-severe TBI. Mechanical ventilation to manage TBI does not seem to be associated with more acute respiratory distress syndrome occurrence.

Monitoring of Respiratory Muscle Function in Critically Ill Children.

OBJECTIVES: This review discusses the different techniques used at the bedside to assess respiratory muscle function in critically ill children and their clinical applications. DATA SOURCES: A scoping review of the medical literature on respiratory muscle function assessment in critically ill children was conducted using the PubMed search engine. STUDY SELECTION: We included all scientific, peer-reviewed studies about respiratory muscle function assessment in critically ill children, as well as some key adult studies. DATA EXTRACTION: Data extracted included findings or comments about techniques used to assess respiratory muscle function. DATA SYNTHESIS: Various promising physiologic techniques are available to assess respiratory muscle function at the bedside of critically ill children throughout the disease process. During the acute phase, this assessment allows a better understanding of the pathophysiological mechanisms of the disease and an optimization of the ventilatory support to increase its effectiveness and limit its potential complications. During the weaning process, these physiologic techniques may help predict extubation success and therefore optimize ventilator weaning. CONCLUSIONS: Physiologic techniques are useful to precisely assess respiratory muscle function and to individualize and optimize the management of mechanical ventilation in children. Among all the available techniques, the measurements of esophageal pressure and electrical activity of the diaphragm appear particularly helpful in the era of individualized ventilatory management.

Epidemiology of severe paediatric trauma following winter sport accidents.

AIM: This study describes the epidemiology of severe injuries related to winter sports (skiing, snowboarding and sledding) in children and assesses potential preventive actions. METHODS: A single-centre retrospective study performed at Pediatric or Adult Intensive Care Unit in the French Alps. All patients less than 15 years old, admitted to the Intensive Care Unit following a skiing, snowboarding or sledding accident from 2011 to 2018, were included. RESULTS: We included 186 patients (mean age 10.6 years and 68% were male); of which 136 (73%), 21 (11%) and 29 (16%) had skiing, snowboarding and sledding accidents, respectively. The average ISS (injury severity score) was 16. The major lesions were head (n = 94 patients, 51%) and intra-abdominal (n = 56 patients, 30%) injuries. Compared to skiing/snowboarding, sledding accidents affected younger children (7 vs 11 years, P < .001); most of whom did not wear a helmet (89% vs 8%, P < .001). Severity scores were statistically different amongst winter sports (ISS = 16 (IQR 9-24) for skiing, 9 (IQR 4-16) for snowboarding and 16 (IQR 13-20) for sledding accident, P = .02). CONCLUSION: Winter sports can cause severe trauma in children. Sledding accidents affect younger children that may benefit from wearing protective equipment.

Aeromedical Transport in Children: A Descriptive Analysis of 96 Cases.

OBJECTIVE: Pediatric aeromedical transport (AT) is highly challenging. We aim to describe a series of 96 cases of children requiring medical transport in terms of the indication, modalities, and related adverse events. METHODS: In this single-center retrospective study, we included air-transported patients less than 18 years old. RESULTS: During the study period, 96 patients, with median age of 120 months (range, 0-204), were air transported. The reason for AT was repatriation to the home country from another hospital in 65 (67.7%), whereas for 31 (32.3%) patients, it was for transport from a hospital to another for a special procedure. The main diagnosis was trauma surgery (n = 38; 39.5%). Fifteen (15.6%) patients were mechanically ventilated. No patient sustained serious adverse events during the flight. Patients were transported by an air ambulance or by a scheduled aircraft in 55.2% and 44.8%, respectively. The median flight distance was 822 km (range, 277-9878), and most of the ATs were intra-European (n = 41; 42.7%). CONCLUSION: This study describes an international experience in providing AT services for pediatric clinical cases. Among pediatric patients, the younger ones constitute a specific population, and most of them require mechanical ventilation. Therefore, the transport of these patients seems to be more appropriate in a dedicated air ambulance.

Relationship Between Diaphragmatic Electrical Activity and Esophageal Pressure Monitoring in Children.

OBJECTIVES: Mechanical ventilation is an essential life support technology, but it is associated with side effects in case of over or under-assistance. The monitoring of respiratory effort may facilitate titration of the support. The gold standard for respiratory effort measurement is based on esophageal pressure monitoring, a technology not commonly available at bedside. Diaphragmatic electrical activity can be routinely monitored in clinical practice and reflects the output of the respiratory centers. We hypothesized that diaphragmatic electrical activity changes accurately reflect changes in mechanical efforts. The objectives of this study were to characterize the relationship between diaphragmatic electrical activity and esophageal pressure. DESIGN: Prospective crossover study. SETTING: Esophageal pressure and diaphragmatic electrical activity were simultaneously recorded using a specific nasogastric tube in three conditions: in pressure support ventilation and in neurally adjusted ventilatory support in a random order, and then after extubation. PATIENTS: Children in the weaning phase of mechanical ventilation. INTERVENTIONS: The maximal swing in esophageal pressure and esophageal pressure-time product, maximum diaphragmatic electrical activity, and inspiratory diaphragmatic electrical activity integral were calculated from 100 consecutive breaths. Neuroventilatory efficiency was estimated using the ratio of tidal volume/maximum diaphragmatic electrical activity. MEASUREMENTS AND MAIN RESULTS: Sixteen patients, with a median age of 4 months (interquartile range, 0.5-13 mo), and weight 5.8 kg (interquartile range, 4.1-8 kg) were included. A strong linear correlation between maximum diaphragmatic electrical activity and maximal swing in esophageal pressure (r > 0.95), and inspiratory diaphragmatic electrical activity integral and esophageal pressure-time product (r > 0.71) was observed in all ventilatory conditions. This correlation was not modified by the type of ventilatory support. CONCLUSIONS: On a short-term basis, diaphragmatic electrical activity changes are strongly correlated with esophageal pressure changes. In clinical practice, diaphragmatic electrical activity monitoring may help to inform on changes in respiratory efforts.

Methemoglobinemia Following Monolinuron Ingestion: A Case Report in a Child.

CASE: We describe a case report of a 2-year-old boy presenting to the emergency department with cyanosis and agitation. There was no suggestive history of poisoning but parents reported the presence of an aquarium at the family dinner the night before, with an aquarium cleaner beside it. Physical examination at admission revealed central cyanosis without signs of respiratory distress. Oxygen saturation measured by pulse oximetry (SpO2) was at 80% in room air. Plasma level of methemoglobin was measured at 11.8%. After 6 hours, methemoglobin decreased spontaneously to 5% and pulse oximetry saturation reached 98% in room air. Consciousness, weakness, and behavior were concomitantly improved. Because of rapid and spontaneous improvement, no specific treatment such as methylene blue was administered. A mass spectrometry toxicological analysis was performed in a blood sample taken the day of admission. Screening procedure of pesticides based on liquid chromatography coupled with mass spectrometry identified monolinuron, a phenylurea herbicide. CONCLUSIONS: We recommend considering acquired methemoglobinemia after ingestion of industrial products and drugs in children with cyanosis, mental status alteration, and without respiratory distress symptoms.

Interfaces for noninvasive ventilation in the acute setting in children.

The use of noninvasive ventilation (NIV) is very specific in the acute setting as compared to its use in a chronic setting. In the Pediatric Intensive care Unit (PICU), NIV may be required around the clock and initiation has to be fast and easy. Despite the increasing use of non-invasive ventilation (NIV) and the larger choice of interfaces, data comparing the use of different interfaces for pediatric patients are scarce and recommendations for the most appropriate choice of interface are lacking. However, this choice in acute settings is crucial and a major contributor of the success of NIV. The aim of the present review was to describe the different types of interfaces available for children in the acute setting, their advantages and limitations, to highlight how to choose the optimal interface, and how to monitor the tolerance of the interface.

Parental perceptions of clown care in paediatric intensive care units.

AIM: The objective of this study was to report family satisfaction with regards to the presence of clowns in the paediatric intensive care unit (PICU). METHODS: This is a single-centre survey-based study, conducted over 4 months in a 12-bed third level PICU in a university hospital. All parents present at the bedside of their child during clowning were considered as potential participants. Eligible parents were approached by one of the two intensivists as investigators and asked to complete a survey within the 48 h following the clowns' intervention. RESULTS: Thirty-three parents consented to complete the survey. Median age of children was 14 months (15 days to 16 years) and median Pediatric Logistic Organ Dysfunction (PELOD) score was 1 (0-22). Twenty-four (72.7%) were considered as clinically stable while the clowns intervened. Twenty-eight parents (84.8%) and 27 (81.8%) considered that clowns had a positive effect on themselves and on their child, respectively. Clown care was considered as necessary in 19 cases (57.6%), optional in 13 (39.4%) and unnecessary in 1 (3.0%). The degree of parental satisfaction was not significantly associated with the child's clinical stability. CONCLUSION: We suggested that medical clowning in the PICU is well accepted by parents, regardless of severity of their child's condition. This study supports the adoption of medical clowning in PICUs as a patient- and family-centred care practice.