Study protocol of a national multicentre prospective evaluation study assessing the validity and impact of the Dutch Paediatric Early Warning Score (PEWS) in the Netherlands.

Introduction: Early recognition of clinical deterioration and timely intervention are important to improve morbidity and mortality in paediatric care. The Paediatric Early Warning Score (PEWS) is a scoring system aiming to identify hospitalised children at risk for deterioration. Currently, there is a large heterogeneity of PEWS systems in the Netherlands, with a considerable number remaining unvalidated or self-designed.

A Systematic Approach to Evaluate Sudden Unexplained Death in Children.

Objective: To evaluate in the Netherlands the national outcomes in providing cause of and insights into sudden and unexplained child deaths among children via the Postmortem Evaluation of Sudden Unexplained Death in Youth (PESUDY) procedure.

Study Design: Children aged 0-18 years in the Netherlands who died suddenly were included in the PESUDY procedure if their death was unexplained and their parents gave consent. The PESUDY procedure consists of pediatric and forensic examination, biochemical, and microbiological tests; radiologic imaging; autopsy; and multidisciplinary discussion.

Development of the national Dutch PEWS: the challenge against heterogeneity and implementation difficulties of PEWS in the Netherlands.

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.

[How do we help children who visit an emergency department frequently?].

Dutch research demonstrated that almost 5% of the pediatric population at an emergency department (ED) of an university hospital are so-called frequent flyers (FFs). These FFs account for more than 20% of all pediatric visits. Three-quarter of these FFs have (multiple) comorbidities.

Effective low-dose sirolimus regimen for kaposiform haemangioendothelioma with Kasabach-Merritt phenomenon in young infants.

Aims: Management of kaposiform haemangioendotheliomas (KHE) with Kasabach-Merritt phenomenon is challenging in young infants who are subjected to developmental pharmacokinetic changes. Sirolimus, sometimes combined with corticosteroids, can be used as an effective treatment of KHE. Simultaneously, toxicities such as interstitial pneumonitis related to the use of sirolimus may be fatal.

Risk stratification to improve Pediatric Early Warning Systems: it is all about the context.

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.

Pediatric Early Warning System Scores: Lessons to be Learned.

The objective was to evaluate the use of a pediatric early warning system (PEWS) score in Dutch general and university hospitals, 4 years after the introduction of a national safety program in which the implementation of a PEWS was advised. An electronic cross-sectional survey was used. All general and university hospitals (  = 91) with a pediatric department in The Netherlands were included in the study.

Clinician Perceptions of an Early Warning System on 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.

Understanding fetal factors that contribute to preterm birth: Sjögren-Larsson syndrome as a model.

Aim: Preterm birth is the world's leading cause of neonatal death. Unfortunately, the pathophysiology of preterm birth remains poorly understood. Sjögren-Larsson syndrome is a rare, neurometabolic disorder caused by a fatty aldehyde dehydrogenase deficiency.

Protein-losing enteropathy in camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome.

Background: Camptodactyly-arthropathy-coxa vara-pericarditis (CACP, OMIM: #208250) syndrome is a rare autosomal recessive disease that can be difficult to recognise not only because of its wide clinical variability but also because of its clinical resemblance to juvenile idiopathic arthritis (JIA). PRG4 is the only gene so far known to be associated with CACP syndrome. Children with CACP syndrome lack the glycoprotein lubricin due to recessive mutations in PRG4.

Validation of a Paediatric Early Warning Score: first results and implications of usage.

Unlabelled: Timely recognition of deterioration of hospitalised children is important to improve mortality. We developed a modified Paediatric Early Warning Score (PEWS) and studied the effects by performing three different cohort studies using different end points. Taking unplanned Paediatric Intensive Care Unit admission as end point and only using data until 2 h prior to end point, we found a sensitivity of 0.

Multimodal imaging of the macula in hereditary and acquired lack of macular pigment.

Purpose: Macular pigment (MP) deficit has been described in macular teleangiectasia type 2 (MTA; acquired MP loss) and in Sjögren-Larsson syndrome (SLS; hereditary MP deficiency). Central blue light-induced fundus autofluorescence (FAF) and blue light fundus reflectance (BLR) are thought to reflect MP distribution. This study was performed to describe the macular morphology in SLS and MTA by multimodal imaging to further investigate the causes of FAF and BLR changes in these disorders.

Sjögren-Larsson syndrome in clinical practice.

This review article gives a state-of-the-art synopsis of current pathophysiological concepts in Sjögren-Larsson syndrome (SLS) mainly based upon original research data of the authors in one of the world's largest clinical SLS study cohorts. Clinical features are discussed in order of appearance, and diagnostic tests are set out to guide the clinician toward the diagnosis SLS. Furthermore, current and future treatment strategies are discussed to render a comprehensive review of the topic.

Patients with Sjögren-Larsson syndrome lack macular pigment.

Purpose: Sjögren-Larsson syndrome (SLS), an autosomal recessive hereditary disorder with congenital ichthyosis, spastic diplegia or tetraplegia, and mental retardation, reveals a characteristic macular dystrophy with intraretinal crystals and foveal pseudocysts. Ophthalmic symptoms in SLS are reduced visual acuity and photophobia. This article reports the deficiency of macular pigment as a novel finding in this peculiar, congenital maculopathy.

Speech-language performance in Sjögren-Larsson syndrome.

Objective: To describe speech-language pathology in patients with Sjögren-Larsson syndrome (SLS) in relation to their cognitive and motor impairment.

Design: Observational case series.

Methods: Cognitive functioning was assessed in 16 patients with SLS (nine males; seven females) using different neuropsychological tests.

Sjögren-Larsson syndrome: motor performance and everyday functioning in 17 patients.

Sjögren-Larsson syndrome (SLS) is an autosomal recessive neurometabolic disorder characterized by spasticity, learning disability, and ichthyosis. To our knowledge, there is no detailed report in the literature concerning the functional consequences of SLS. Therefore, we performed a cross-sectional study of motor performance and everyday functioning in 17 patients with this rare disorder.

Subclinical changes in the juvenile crystalline macular dystrophy in Sjögren-Larsson syndrome detected by optical coherence tomography.

Purpose: To study morphologic changes in the macula by optical coherence tomography (OCT) in patients with a crystalline macular dystrophy due to the autosomal recessive neurocutaneous Sjögren-Larsson syndrome (SLS).

Design: Retrospective observational case series.

Participants: Twenty-seven eyes of 14 patients, mean age 14.