Early recognition of critically ill patients is of paramount importance to reduce pediatric mortality and morbidity. We created a risk stratification system combining vital parameters and predefined risk factors aimed at reducing the risk of unrecognized clinical deterioration compared with conventional Pediatric Early Warning Systems (PEWS). This single-center retrospective case cohort study included infants (gestational age ≥ 37 weeks) to adolescents (aged <18 years) with unplanned pediatric intensive care unit (PICU) admission between April 01, 2014, and February 28, 2018. The sensitivity in the 24 h prior to endpoint of the Pediatric Risk Evaluation and Stratification System (PRESS) was compared with that of the conventional PEWS and calculated as the proportion of study patients who received a high-risk score. Seventy-four PICU admissions were included. PRESS and PEWS sensitivities at 2 h prior to endpoint were 0.70 (95%CI 0.59 to 0.80) and 0.30 (95%CI 0.20 to 0.42) respectively (p < 0.001). Excluding patients with seizures, PRESS sensitivity increased to 0.75 (95%CI 0.64 to 0.85). Forty-nine patients (66%) scored positive on at least one high-risk factor, and “worried sign” was scored in 31 patients (42%). Conclusion: Risk stratification seems advantageous for a faster detection of clinical deterioration, providing opportunity for earlier intervention. What is Known: • Prompt detection of clinical deterioration is of essential importance to reduce morbidity and mortality. • Conventional Pediatric Early Warning Systems (PEWS) have limited sensitivity and a short window of detection of 1 to 2 h. What is New: • Risk stratification based on context factors allows earlier identification of patients at risk, well before deviation of vital signs. • Risk stratification combined with continuous monitoring of deteriorating trends in vital signs could lead to the development of next-generation warning systems achieving true patient safety.

BACKGROUND AND OBJECTIVES: The Pediatric Early Warning Score (PEWS) aims to improve early recognition of clinical deterioration and is widely used despite lacking evidence of effects on outcome measures such as hospital mortality. In this qualitative study, we aimed to study effects of both PEWS and the locally designed risk stratification system by focusing on professionals’ perception of their performance. We also sought to gain insight into the perceived effects of PEWS and the risk stratification system on patient safety and to unravel the underlying mechanisms. METHODS: A single-center cross-sectional observational study whereby 16 semistructured interviews were held with selected health care professionals focusing on perceived effects and underlying mechanisms. Interviews were transcribed verbatim and coded without using a predetermined set of themes. RESULTS: Coding from semistructured interviews demonstrated that perceived value was related to effects on different levels of Endsley and co-workers’ situational awareness (SA) model. PEWS mainly improved level 1 SA, whereas the risk stratification system also seemed to improve levels 2 and 3 SA. CONCLUSIONS: This study shows clear effects of PEWS on SA among professionals. It also points to the additional value of other risk factor stratification systems to help further improve PEWS functioning.

Is missing (Short communication)...

Recent diagnostic advances reveal that lymphatic disease in Noonan syndrome (NS) and other NS-like RASopathies often stems from central conducting lymphatic anomalies (CCLAs). The RAS/MAPK-ERK pathway plays a central role in lymphangiogenesis. Targeting this pathway with MEK-inhibitor trametinib has emerged as a promising therapeutic strategy for managing CCLAs in patients with NS-like RASopathies. This case series assessed the clinical outcomes of trametinib therapy in eight patients with NS-like RASopathies and CCLA, each offering unique insights into the therapeutic efficacy of MEK inhibition. In infants, a lower dose of 0.01 mg/kg/day and earlier discontinuation of trametinib therapy effectively alleviated the symptoms of congenital chylothorax and rescued the lymphatic phenotype, compared to similar published cases. Moreover, four patients aged >11 y showed a slower response and did not achieve complete symptomatic recovery. In conclusion, it is advised to consider trametinib therapy for patients with severe, therapy-refractory CCLA in patients with NS-like RASopathies. However, individual responses to trametinib therapy may vary, with some patients demonstrating more favorable outcomes than others. Further investigation into potential enhancers and suppressors of the lymphatic phenotype is necessary for more accurate treatment predictions. While these factors are likely genetic, we cannot rule out other intrinsic or physiological factors.

Background: For the early recognition of deteriorating patients several Pediatric Early Warning Score (PEWS) systems have been developed with the assumption that early detection can prevent further deterioration. Although PEWS are widely being used in hospitals in the Netherlands, there is no national consensus on which score to use and how to embed the score into a PEWS system. This resulted in a substantial heterogeneity of PEWS systems, of which many are unvalidated or self-designed. The primary objective of this study was to develop a pragmatic consensus-based PEWS system that can be utilized in all Dutch hospitals (University Medical Centers, teaching hospitals, and general hospitals). Methods: This study is an iterative mixed-methods study. The methods from the Core Outcome Measures in Effectiveness Trials (COMET) initiative were used and consisted of two Delphi rounds, two inventories set out to all Dutch hospitals and a focus group session with parents. The study was guided by five expert meetings with different stakeholders and a final consensus meeting that resulted in a core PEWS set. Results: The first Delphi round was completed by 292 healthcare professionals, consisting of pediatric nurses and physicians. In the second Delphi round 217 healthcare professionals participated. Eventually, the core PEWS set was been developed comprising of the parameters work of breathing, respiratory rate, oxygen therapy, heart rate and capillary refill time, and AVPU (Alert, Verbal, Pain, and Unresponsive). In addition, risk stratification was added to the core set with standardized risk factors consisting of [1] worried signs from healthcare professionals and parents and [2] high-risk treatment, with the option to add applicable local defined risk factors. Lastly, the three categories of risk stratification were defined (standard, medium, and high risk) in combination with standardized actions of the professionals for each category. Conclusion: This study demonstrates a way to end a country’s struggle with PEWS heterogeneity by co-designing a national Dutch PEWS system. Currently, the power of the system is being investigated in a large multi-center study in the Netherlands.