For more than 10 years, children at our national center for pediatric liver transplantation (LT) have been treated with Molecular Adsorbent Recirculating System (MARS) liver dialysis as a bridging therapy to high-urgency LT. Treatment was reserved for 20 patients with the highest degrees of hepatic encephalopathy (HE; median grade 5 3.5). Death from neurological sequelae was considered imminent for these patients, and this was further reflected in significantly higher international normalized ratios and ammonia levels and worse prognostic liver indices (Model for End-Stage Liver Disease/Pediatric End-Stage Liver Disease scores and liver injury units) in comparison with 32 wait-listed patients who did not receive MARS dialysis. MARS therapy was generally well tolerated, with a reduction in thrombocytes and hemorrhaging as the most common side effects. HE improvement was documented in 30% of the treated patients, but progression to grade IV encephalopathy occurred in 45% of the patients despite the treatment. Serum ammonia, bilirubin, bile acid, and creatinine levels significantly decreased during treatment. Eighty percent of MARS-treated patients survived to undergo LT, and their survival was equivalent to that of non-MARStreated patients with severe liver failure (69%, P 5 0.52). The heterogeneity between MARS-treated patients and non-MARStreated patients in our cohort precluded a statistical evaluation of a benefit from MARS for patient survival. Our data demonstrate the safety of MARS even in the most severely ill patients awaiting LT, but strategies that promote the more rapid and widespread availability of high-quality donor organs remain of critical importance for improving patient survival in cases of severe acute liver failure. Liver Transpl 21:369-380, 2015. V C 2015 AASLD.

A 9-year-old boy with the classical type of Ehlers-Danlos syndrome (EDS) developed a symptomatic aneurysm of the superior mesenteric artery. His EDS diagnosis had been confirmed biochemically and genetically. Vascular complications are known to be associated with the vascular type of EDS, but this is the first report of a child with classical EDS who developed a major vascular complication. Clinicians should be aware that severe vascular complications albeit rare, can also occur in classical EDS.

BackgroundPaediatric Early Warning Scores (PEWS) are increasingly being used for early identification and management of clinical deterioration in paediatric patients. A PEWS system includes scores, cut-off points and appropriate early intervention. In 2011, The Dutch Ministry of Health advised hospitals to implement a PEWS system in order to improve patient safety in paediatric wards. The objective of this study was to examine the results of implementation of PEWS systems and to gain insight into the attitudes of professionals towards using a PEWS system in Dutch non-university hospitals.MethodsQuantitative data were gathered at start, midway and at the end of the implementation period through retrospective patient record review (n = 554). Semi-structured interviews with professionals (n = 8) were used to gain insight in the implementation process and experiences. The interviews were transcribed and analysed using an inductive approach.ResultsLooking at PEWS systems of the five participating hospitals, different parameters and policies were found. While all hospitals included heart rate and respiratory rate, other variables differed among hospitals. At baseline, none of the hospitals used a PEWS system. After 1 year, PEWS were recorded in 69.2% of the patient records and elevated PEWS resulted in appropriate action in 49.1%. Three themes emerged from the interviews: 1) while the importance of using a PEWS system was acknowledged, professionals voiced some doubts about the effectiveness and validity of their PEWS system 2) registering PEWS required little extra effort and was facilitated by PEWS being integrated into the electronic patient record 3) Without a national PEWS system or guidelines, hospitals found it difficult to identify a suitable PEWS system for their setting. Existing systems were not always considered applicable in a non-university setting.ConclusionsAfter 1 year, hospitals showed improvements in the use of their PEWS system, although some were decidedly more successful than others. Doubts among staff about validity, effectiveness and communication with other hospitals during transfer to higher level care hospital might hinder sustainable implementation. For these purposes the development of a national PEWS system is recommended, consisting of a “core set” of PEWS, cut-off points and associated early intervention.

In a double-blind crossover study the remineralizing effects of the combinations: placebo toothpaste, placebo gel; amine fluoride toothpaste, placebo gel; amine fluoride toothpaste, 0.25% F^–– amine fluoride gel and amine fluoride toothpaste, 0.4% F^–– amine fluoride gel were investigated in vivo. Artificially demineralized flattened enamel slabs were placed in the buccal flanges of partial dentures. It was found during the 3-week experimental periods that there was a significantly higher increase in remineralization in the 2 fluoride gel groups. This occurred in both the surface layer and subsurface lesion and resulted in a significant decrease in lesion depth and increase in hardness. It is suggested that the weekly use of these gels should be considered in patients with early enamel lesions if they are formed during, or fail to remineralize under, a normal preventive regime.