Ventilatory management of critically ill children in the emergency setting, during transport and retrieval.

Critical illness in children is uncommon. The acute stabilization and resuscitation of critically ill children remains challenging to even the most experienced operator. Cardiorespiratory illness represents the largest subgroup of diseases causing critical illness and, thus adds a layer of complexity and additional challenge to the safe intubation and establishment of effective ventilation of this group of children. Children have unique physiological and anatomical differences to adults, and present the team involved in their resuscitation and stabilization with challenges exaggerated by critical illness. The consideration of pathophysiological implications of disease and the equipment available during transport and retrieval from the roadside or nonspecialist setting to pediatric intensive care allows the clinician involved in resuscitation, stabilization, and establishment of ventilation to employ targeted strategies to optimize ventilatory success. This review focuses on the types of ventilatory challenges that must be addressed when managing critically ill children in the local settings in which they present, and the resources available to optimize the outcome prior to and during transfer to a higher level of care.

Acute Kidney Injury in Pediatric Inflammatory Multisystem Syndrome Temporally Associated With Severe Acute Respiratory Syndrome Coronavirus-2 Pandemic: Experience From PICUs Across United Kingdom.

OBJECTIVES: To study the prevalence, evolution, and clinical factors associated with acute kidney injury in children admitted to PICUs with pediatric inflammatory multisystem syndrome temporally associated with severe acute respiratory syndrome coronavirus-2. DESIGN: Multicenter observational study. SETTING: Fifteen PICUs across the United Kingdom. PATIENTS: Patients admitted to United Kingdom PICUs with pediatric inflammatory multisystem syndrome temporally associated with severe acute respiratory syndrome coronavirus-2 between March 14, 2020, and May 20, 2020. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Deidentified data collected as part of routine clinical care were analyzed. All children were diagnosed and staged for acute kidney injury based on the level of serum creatinine above the upper limit of reference interval values according to published guidance. Severe acute kidney injury was defined as stage 2/3 acute kidney injury. Uni- and multivariable analyses were performed to study the association between demographic data, clinical features, markers of inflammation and cardiac injury, and severe acute kidney injury. Over the study period, 116 patients with pediatric inflammatory multisystem syndrome temporally associated with severe acute respiratory syndrome coronavirus-2 were admitted to 15 United Kingdom PICUs. Any-stage acute kidney injury occurred in 48 of 116 patients (41.4%) and severe acute kidney injury in 32 of 116 (27.6%) patients, which was mostly evident at admission (24/32, 75%). In univariable analysis, body mass index, hyperferritinemia, high C-reactive protein, Pediatric Index of Mortality 3 score, vasoactive medication, and invasive mechanical ventilation were associated with severe acute kidney injury. In multivariable logistic regression, hyperferritinemia was associated with severe acute kidney injury (compared with nonsevere acute kidney injury; adjusted odds ratio 1.04; 95% CI, 1.01-1.08; p = 0.04). Severe acute kidney injury was associated with longer PICU stay (median 5 days [interquartile range, 4-7 d] vs 3 days [interquartile range, 1.5-5 d]; p < 0.001) and increased duration of invasive mechanical ventilation (median 4 days [interquartile range, 2-6 d] vs 2 days [interquartile range, 1-3 d]; p = 0.04). CONCLUSIONS: Severe acute kidney injury occurred in just over a quarter of children admitted to United Kingdom PICUs with pediatric inflammatory multisystem syndrome temporally associated with severe acute respiratory syndrome coronavirus-2. Hyperferritinemia was significantly associated with severe acute kidney injury. Severe acute kidney injury was associated with increased duration of stay and ventilation. Although short-term outcomes for acute kidney injury in pediatric inflammatory multisystem syndrome temporally associated with severe acute respiratory syndrome coronavirus-2 appear good, long-term outcomes are unknown.

The role and scope of retrieval nurse practitioners in the UK.

BACKGROUND: The first independent paediatric retrieval nurse practitioners (RNP) in the UK and Europe were appointed in 2006 in one hospital Trust. Since then, many have been appointed around the UK but the range of responsibility and scope of the role is unknown as well as possible geographical variability. AIM: The aim of this paper is to explore the role and scope of paediatric retrieval nurse practitioners in the United Kingdom (UK). METHOD: A two part questionnaire was sent to all RNPs identified by the regional retrieval/transport centres. Information was sought about the role and scope of RNPs and how the role may have changed since appointment. RNPs were asked to self-assess their own ability in a variety of technical and leadership skills. CONCLUSION: The majority of respondents felt their role had expanded since appointment. RNPs reported that they would now lead the team for any critically ill infant or child where previously they were retrieving stable or high dependency patients. Other expansions of their role included being on a middle grade tier of the medical rota, asked to undertake aeromedical transfers, providing the withdrawal of care at referring centres and non-medical independent prescribing. RELEVANCE TO PRACTICE: This survey has revealed the changing role and scope of RNPs in the UK but most importantly, the increased responsibility of the roles. Over 80% of RNPs rated their leadership skills between proficient and expert on Benner's Novice to Expert Continuum (Benner, 1984).

Can critical care transport be safely reduced in children intubated during emergency management of status epilepticus in the United Kingdom: a national audit with case-control analysis.

OBJECTIVE: This study describes the baseline clinical characteristics, predictors of successful extubation at referring hospitals and short-term outcomes of children intubated for status epilepticus and referred to United Kingdom (UK) paediatric critical care transport teams (PCCTs). DESIGN: Multicentre audit with case-control analysis, conducted between 1 September 2018 and 1 September 2020. SETTING: This study involved 10 UK PCCTs. PATIENTS: Children over 1 month of age intubated during emergency management for status epilepticus (SE), referred to UK PCCTs. Patients with trauma, requiring time-critical neurosurgical intervention or those with a tracheostomy were excluded. INTERVENTIONS: No interventions were implemented. MEASUREMENTS AND MAIN RESULTS: Out of the 1622 referrals for SE, 1136 (70%) were intubated at referral. The median age was 3 years (IQR 1.25-6.54 years). Among the intubated children, 396 (34.8%) were extubated locally by the referring team, with 19 (4.8%) requiring reintubation. Therefore, the overall rate of successful extubation was 33% (377/1136). There was significant variation between PCCTs, with local extubation rates ranging from 2% to 74%. Multivariable analyses showed region/PCCT, contributing diagnosis, acute changes on CT, preceding encephalopathy and type of continuous sedation (midazolam) used postintubation were significantly associated with transfer to a critical care unit. CONCLUSION: This study highlights wide regional variation in early extubation practices. Regions with high successful extubation rates have established extubation guidelines from PCCTs. Successful extubation represents critical care transports that have been avoided.

Brown-Vialetto-Van Laere syndrome, a ponto-bulbar palsy with deafness, is caused by mutations in c20orf54.

Brown-Vialetto-Van Laere syndrome is a rare neurological disorder with a variable age at onset and clinical course. The key features are progressive ponto-bulbar palsy and bilateral sensorineural deafness. A complex neurological phenotype with a mixed picture of upper and lower motor neuron involvement reminiscent of amyotrophic lateral sclerosis evolves with disease progression. We identified a candidate gene, C20orf54, by studying a consanguineous family with multiple affected individuals and subsequently demonstrated that mutations in this gene were the cause of disease in other, unrelated families.

Understanding Covid and the associated post-infectious hyper-inflammatory state (PIMS-TS) in children.

When SARS-CoV2 infection was first reported from China, very few children had severe lung or systemic disease. Approximately six weeks after the first adult cases were reported in the United Kingdom, a small subgroup of children of largely non-white backgrounds, presented with severe hyper-inflammatory disease, most likely associated with Covid. The possible reasons for this ethnic predilection are explored.

Management of severe bronchiolitis: impact of NICE guidelines.

OBJECTIVE: To understand the impact of the National Institute for Health and Care Excellence (NICE) bronchiolitis guidelines on the management of children referred to paediatric intensive care unit (PICU) with bronchiolitis. DESIGN AND SETTING: Data were collected on all children referred to a regional PICU transport service with the clinical diagnosis of bronchiolitis during the winter prior to the NICE consultation period (2011-2012) and during the winter after publication (2015-2016). Management initiated by the referring hospital was assessed. RESULTS: There were 165 infants referred with bronchiolitis in epoch 1 and 187 in epoch 2. Nebuliser use increased from 28% in epoch 1 to 53% in epoch 2. Increased use of high-flow nasal cannula oxygen and reduction in continuous positive airway pressure use were observed. The use of antibiotics did not change between epochs. CONCLUSION: The use of nebulised therapies has increased in the management of severe bronchiolitis despite national guidance to the contrary.

Use of oral clonidine for sedation in ventilated paediatric intensive care patients.

OBJECTIVES: We aimed to document our experience with oral clonidine when used as a sedative in combination with intravenous morphine and lorazepam in a group of mechanically ventilated children with single-organ, respiratory failure. In particular, our objectives were to establish the relationship between oral dose, plasma concentration, and sedative effect, and second, to document the side-effect profile. DESIGN: Prospective, cohort study over a 72-h period. SETTING: Regional paediatric intensive care unit. PATIENTS AND PARTICIPANTS: Twenty-four children were enrolled (median age 3 months) of whom ten were excluded (six due to extubation before 72 h, three sedation failures, one protocol violation). MEASUREMENTS AND RESULTS: Plasma clonidine was measured using gas chromatography mass spectrometry, and sedation assessed using the COMFORT score. Using a dose of 3-5 microg/kg every 8 h, plasma concentrations appeared to plateau at approximately 41 h giving a mean value of 1.38 ng/ml (95% confidence interval 1.0-1.8). Adequate sedation was achieved during 82% (837/1022 h) of the study period; however, this decreased to 70.3% when analysed on an intention-to-treat basis. There was a concomitant overall decrease in the average hourly requirements for both morphine ( P = 0.02) and lorazepam ( P = 0.003). There were no documented episodes of bradycardia, hypotension or hyperglycaemia. CONCLUSIONS: Oral clonidine may be a safe and effective sedative in combination with morphine and lorazepam for young children with single-organ, respiratory failure. This agent may also exhibit opioid and benzodiazepine sparing effects in this patient group. A full pharmacokinetic study is warranted.

"Early" delayed sternal closure following pediatric cardiac surgery.

BACKGROUND: Delayed sternal closure is commonly used following pediatric cardiopulmonary bypass surgery for many reasons including support of the failing myocardium. We hypothesized that, as a result of improvements in perioperative care, sternal closure could be achieved at an earlier postoperative time than the 3 to 5 days typically reported in the literature. METHODS: Retrospective chart review of all bypass surgery (n = 585) performed in a single center over a 3-year period (2000-2002). RESULTS: We identified 66 children (11.3%), median age 5 days old, who underwent delayed sternal closure. In 60 of these patients, sternal closure was achieved at a median (interquartile) postoperative time of 21 hours (18 to 40 hours). The most common indication was inadequate hemostasis, although early sternal closure was also achieved in the subgroup with poor myocardial function as the primary indication at a median of 36 hours (21 to 44 hours). There was no noticeable hemodynamic, respiratory or metabolic compromise following sternal closure, although patients with poor myocardial function tended to have a lower mean blood pressure than those with inadequate hemostasis (ANOVA, p = 0.02). The overall mortality was 19.7% (13 of 66), with a median duration of ventilation and intensive care stay among survivors of 3.8 days (2.4 to 6.3 days) and 4.8 days (3.7 to 7.9 days), respectively. CONCLUSIONS: Delayed sternal closure is possible at an earlier stage than previously reported.