Validating a risk assessment tool in United Kingdom and Irish paediatric cardiac catheterisation practice.

Background: No established risk prediction tool exists in United Kingdom and Irish Paediatric Cardiology practice for patients undergoing cardiac catheterisation. The Catheterisation RISk score for Paediatrics is used primarily in North American practice to assess risk prior to cardiac catheterisation. Validating the utility and transferability of such a tool in practice provides the opportunity to employ an already established risk assessment tool in everyday practice.

Consultant staffing in UK congenital cardiac services: a 10-year survey of leavers and joiners.

Objectives: To report the numbers of consultant congenital cardiac surgeons and cardiologists who have joined and left UK practice over the last 10 years and explore the reasons for leaving.

Methods: Retrospective observational questionnaire study completed between 11 June 2019 and 1 July 2020 by UK level 1 congenital cardiac centres of 10-year consultant staff movement and reasons suggested for leaving UK practice.

Results: At survey completion there were 218 (202 whole time equivalent (WTE)) consultant cardiologists and surgeons working within level 1 centres made up of 39 (38 WTE) surgeons, 137 (128.

Application of the Clavien-Dindo classification to a pediatric surgical network.

Introduction: A comprehensive validated system to evaluate surgical complications is required in our specialty to facilitate comparison and audit. The Clavien-Dindo (CD) classification of post-surgical complications was originally described in an adult general surgical setting in 1992 and has become widely used. We aimed to apply this to a pediatric surgical setting.

The immunogenicity of ReFacto AF (moroctocog alfa AF-CC) in previously untreated patients with haemophilia A in the United Kingdom.

Introduction: Factor VIII inhibitor development is currently the most serious complication of the treatment of haemophilia A. Differences in manufacturing and the molecular structure of brands of recombinant factor VIII have led to speculation that concentrates may differ in immunogenicity. This has led to a regulatory focus on the immunogenicity of factor VIII concentrates both before and after licensure.

Exome sequencing in an admixed isolated population indicates NFXL1 variants confer a risk for specific language impairment.

Children affected by Specific Language Impairment (SLI) fail to acquire age appropriate language skills despite adequate intelligence and opportunity. SLI is highly heritable, but the understanding of underlying genetic mechanisms has proved challenging. In this study, we use molecular genetic techniques to investigate an admixed isolated founder population from the Robinson Crusoe Island (Chile), who are affected by a high incidence of SLI, increasing the power to discover contributory genetic factors.

Changes in Dickkopf-1 (DKK1) and Sclerostin following a Loading Dose of Vitamin D 2 (300,000 IU).

Background. Vitamin D is important for bone health, although high loading doses have been associated with an increase in fracture risk. The mechanisms remain uncertain.

Development, evaluation and implementation of video-EEG telemetry at home.

Purpose: To describe the development and implementation of video EEG telemetry (VT) in the patient's home (home video telemetry, HVT) in a single centre.

Methods: HVT met the UK Medical Research Council definition of a complex intervention, and we used its guidance to evaluate the process of piloting, evaluating, developing and implementing this new clinical service. The first phase was a feasibility study, comparing inpatient VT (IVT) with HVT in a test-retest design (n=5), to assess data quality and yield of clinically relevant events.

Effect of a 300 000-IU loading dose of ergocalciferol (Vitamin D2) on circulating 1,25(OH)2-vitamin D and fibroblast growth factor-23 (FGF-23) in vitamin D insufficiency.

Context: Several trials have reported an increased risk of fractures and falls after intermittent high-dose vitamin D. Treatment with loading doses of vitamin D may increase 1,25(OH)(2) vitamin D catabolism through changes in calcium/phosphate homeostasis and fibroblast growth factor-23 (FGF-23).

Objective: The aim was to determine the effects of high-dose vitamin D on circulating concentrations of 1,25(OH)(2) vitamin D and FGF-23 in patients with osteoporosis and vitamin D insufficiency.

Constipation and incontinence in childhood: two sides of the same coin?

Perspective on the paper by Loening‐Baucke ()