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Objective: To measure scenario participant and faculty self-reported realism, engagement and learning for the low fidelity, in situ simulations and compare this to high fidelity, centre-based simulations., Methods: A prospective survey of scenario participants and faculty completing in situ and centre-based paediatric simulations., Results: There were 382 responses, 276 from scenario participants and 106 from faculty with 241 responses from in situ and 141 from centre-based simulations. Scenario participant responses showed significantly higher ratings for the centre-based simulations for respiratory rate (P = 0.007), pulse (P = 0.036), breath sounds (P = 0.002), heart sounds (P < 0.001) and patient noises (P < 0.001). There was a significant difference in overall rating of the scenario reality by scenario participants in favour of the centre-based simulations (P = 0.005); however, there was no significant difference when rating participant engagement (P = 0.11) and participant learning (P = 0.77). With the centre-based scenarios, nurses rated the reality of the respiratory rate (P < 0.001), blood pressure (P = 0.016) and abdominal signs (P = 0.003) significantly higher than doctors. Nurses rated the overall reality higher than doctors for the centre simulations (96.8% vs 84.2% rated as realistic, P = 0.041), which was not demonstrated in the in situ scenarios (76.2% vs 73.5%, P = 0.65)., Conclusion: Some aspects of in situ simulations may be less 'real' than centre-based simulations, but there was no significant difference in self-reported engagement or learning by scenario participants. Low fidelity, in situ simulation provides adequate realism for engagement and learning., (© 2017 The Authors Emergency Medicine Australasia published by John Wiley & Sons Australia, Ltd on behalf of Australasian College for Emergency Medicine and Australasian Society for Emergency Medicine.)

Objective: To prospectively compare the actual weights of Australian children in an ethnically diverse metropolitan setting with the predicted weights using the Paediatric Advanced Weight Prediction in the Emergency Room (PAWPER) tape, Broselow tape, Mercy system and calculated weights using the updated Advanced Paediatric Life Support (APLS), Luscombe and Owens and Best Guess formulae., Methods: A prospective, cross-sectional, observational, blinded, convenience study conducted at the Children's Hospital at Westmead Paediatric Emergency Department in Sydney, Australia. Comparisons were made using Bland-Altman plots, mean difference, limits of agreement and estimated weight within 10% and 20% of actual weight., Results: 199 patients were enrolled in the study with a mean actual weight of 27.2 kg (SD 17.2). Length-based tools, with or without body habitus adjustment, performed better than age-based formulae. When measuring estimated weight within 10% of actual weight, PAWPER performed best with 73%, followed by Mercy (69%), PAWPER with no adjustment (62%), Broselow (60%), Best Guess (47%), Luscombe and Owens (41%) and revised APLS (40%). Mean difference was similar across all methods ranging from 0.4 kg (0.0, 0.9) for Mercy to -2.2 kg (-3.5, -0.9) for revised APLS. Limits of agreement were narrower for the length-based tools (-5.9, 6.8 Mercy; -8.3, 5.6 Broselow; -9.0, 7.1 PAWPER adjusted; -12.1, 9.2 PAWPER unadjusted) than the age-based formulae (-18.6, 17.4 Best Guess; -19.4, 15.1 revised APLS, -21.8, 17.7 Luscombe and Owens)., Conclusion: In an ethnically diverse population, length-based methods with or without body habitus modification are superior to age-based methods for predicting actual body weight. Body habitus modifications increase the accuracy and precision slightly., Competing Interests: Conflicts of Interest: None declared., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.)

Aim: To measure the long-term improvement in the documented provision of an asthma action plan (AAP) to children with asthma and wheeze discharged from the Emergency Department following the introduction of the electronic AAP (eAAP) and to determine the need for an electronic pre-school wheeze action plan in our population., Methods: A retrospective case note review, from July 2014 to June 2015, of all patients over 12 months old discharged from the Emergency Department or Emergency Medical Unit, with a discharge diagnosis of either asthma or wheeze. The primary outcome was the documentation of an AAP, either recorded electronically as an eAAP or a report of an AAP as part of the patient medical record., Results: Two thousand three hundred and forty-two patients were included in the study, 926 with asthma and 1416 with wheeze. The median age was 3.3 years (interquartile range (IQR) 3.5, range 1-15.9 years). The median age of the children with asthma was 5.3 years (IQR 4.6) and of the children with wheeze was 2.5 years (IQR 2.0).Overall, 1683 (71.9%) children had a documented AAP, with a significant difference between those with a discharge diagnosis of asthma (85.9%) compared with wheeze (62.9%), P < 0.001. These results justified the design of the electronic pre-school wheeze action plan., Conclusions: The integration of an eAAP into the Emergency Department has resulted in a sustained improvement in the documented provision of an AAP to children with a discharge diagnosis of asthma. Children with a discharge diagnosis of wheeze are significantly less likely to receive an action plan., (© 2016 The Authors. Journal of Paediatrics and Child Health published by John Wiley & Sons Australia, Ltd on behalf of Paediatrics and Child Health Division (The Royal Australasian College of Physicians).)

Autosomal recessive polycystic kidney disease (ARPKD/PKHD1) is an important cause of renal-related and liver-related morbidity and mortality in childhood. Recently mutations in the PKHD1 gene on chromosome 6p21.1-p12 have been identified as the molecular cause of ARPKD. The longest continuous open reading frame (ORF) is encoded by a 67-exon transcript and predicted to yield a 4074-amino acid protein ("polyductin") of thus far unknown function. By now, a total of 29 different PKHD1 mutations have been described. This study reports mutation screening in 90 ARPKD patients and identifies mutations in 110 alleles making up a detection rate of 61%. Thirty-four of the detected mutations have not been reported previously. Two underlying mutations in 40 patients and one mutation in 30 cases are disclosed, and no mutation was detected on the remaining chromosomes. Mutations were found to be scattered throughout the gene without evidence of clustering at specific sites. About 45% of the changes were predicted to truncate the protein. All missense mutations were nonconservative, with the affected amino acid residues found to be conserved in the murine polyductin orthologue. One recurrent missense mutation (T36M) likely represents a mutational hotspot and occurs in a variety of populations. Two founder mutations (R496X and V3471G) make up about 60% of PKHD1 mutations in the Finnish population. Preliminary genotype-phenotype correlations could be established for the type of mutation rather than for the site of the individual mutation. All patients carrying two truncating mutations displayed a severe phenotype with perinatal or neonatal demise. PKHD1 mutation analysis is a powerful tool to establish the molecular cause of ARPKD in a given family. Direct identification of mutations allows an unequivocal diagnosis and accurate genetic counseling even in families displaying diagnostic challenges.