Transitioning children using home invasive mechanical ventilation from hospital to home: Discharge criteria, disparities, and ethical considerations.

Children using home invasive mechanical ventilation (HIMV), a valuable therapeutic option for chronic respiratory failure, constitute a growing population. Transitioning children using HIMV from hospital to home care is a complex process that requires a multidisciplinary approach involving healthcare professionals, caregivers, and community resources. Medical stability, caregiver competence, and home environment suitability are essential factors in determining discharge readiness.

Monogenic disorders as mimics of juvenile idiopathic arthritis.

Background: Juvenile idiopathic arthritis is the most common chronic rheumatic disease of childhood. The term JIA encompasses a heterogenous group of diseases. The variability in phenotype of patients affected by the disease means it is not uncommon for mimics of JIA to be misdiagnosed.

Pediatric Antifungal Prescribing Patterns Identify Significant Opportunities to Rationalize Antifungal Use in Children.

Objective: The need for pediatric antifungal stewardship programs has been driven by an increasing consumption of antifungals for prophylactic and empirical use. Drivers and rational of antifungal prescribing need to be identified to optimize prescription behaviors.

Methods: A prospective modified weekly Point Prevalence Survey capturing antifungal prescriptions for children (> 90 days to < 18 years of age) in 12 centers in England during 26 consecutive weeks was performed.

Hemofiltration in the Management of Severe Pediatric Burns: Experience in a UK Burns Centre and Systematic Review.

Pediatric burns are life-threatening injuries due to the acute injury and secondary complications. In acute-phase burns, hypovolemia and vasoconstriction cause renal impairment. Sepsis and multiorgan failure compound the problem resulting in morbidity and mortality.

Genomic Classification and Clinical Outcome in Rhabdomyosarcoma: A Report From an International Consortium.

Purpose: Rhabdomyosarcoma is the most common soft tissue sarcoma of childhood. Despite aggressive therapy, the 5-year survival rate for patients with metastatic or recurrent disease remains poor, and beyond fusion status, no genomic markers are available for risk stratification. We present an international consortium study designed to determine the incidence of driver mutations and their association with clinical outcome.

Acute Cardiovascular Manifestations in 286 Children With Multisystem Inflammatory Syndrome Associated With COVID-19 Infection in Europe.

Background: The aim of the study was to document cardiovascular clinical findings, cardiac imaging, and laboratory markers in children presenting with the novel multisystem inflammatory syndrome associated with coronavirus disease 2019 (COVID-19) infection.

Methods: This real-time internet-based survey has been endorsed by the Association for European Paediatric and Congenital Cardiologists Working Groups for Cardiac Imaging and Cardiovascular Intensive Care. Children 0 to 18 years of age admitted to a hospital between February 1 and June 6, 2020, with a diagnosis of an inflammatory syndrome and acute cardiovascular complications were included.

Scandcleft randomized trials of primary surgery for unilateral cleft lip and palate: dental anomalies in 8-year olds.

Background: Children born with unilateral cleft lip and palate (UCLP) are reported to display several dental anomalies including agenesis, supernumeraries, as well as variations in dental size, shape, and path of eruption. The extensive sample of individuals with UCLP included in the Scandcleft randomized control trials offers the opportunity to study more rare conditions, which is seldom possible with limited samples.

Objectives: The aim was to study dental anomalies at 8 years of age in children born with UCLP included in the Scandcleft randomized control trials.

Neonatal Antifungal Consumption Is Dominated by Prophylactic Use; Outcomes From The Pediatric Antifungal Stewardship: Optimizing Antifungal Prescription Study.

Background: Diagnostic challenges combined with the vulnerability of neonates to develop invasive candidiasis (IC) may lead to antifungal administration in the absence of IC. A modified point-prevalence study was performed to obtain an improved insight and understanding of antifungal prescribing in this specific patient population.

Methods: Neonates and infants ≤90 days of age receiving systemic antifungals from 12 centers in England were included.

The potential for pre-, pro- and synbiotics in the management of infants at risk of cow's milk allergy or with cow's milk allergy: An exploration of the rationale, available evidence and remaining questions.

Cow's milk allergy is one of the most commonly reported childhood food allergies, with increasing incidence, persistence and severity in many countries across the world. The World Allergy Organization Special Committee on Food Allergy has identified cow's milk allergy as an area in need of a rationale-based approach in order to make progress against what it considered an onerous problem, with worldwide public health impact. There is growing interest in the potential role of the gut microbiota in the early programming and development of immune responses and allergy.

Genetic testing in steroid-resistant nephrotic syndrome: why, who, when and how?

Steroid-resistant nephrotic syndrome (SRNS) is a common cause of chronic kidney disease in childhood and has a significant risk of rapid progression to end-stage renal disease. The identification of over 50 monogenic causes of SRNS has revealed dysfunction in podocyte-associated proteins in the pathogenesis of proteinuria, highlighting their essential role in glomerular function. Recent technological advances in high-throughput sequencing have enabled indication-driven genetic panel testing for patients with SRNS.

Highlights from the 30th North American Cystic Fibrosis Conference, Orlando 2016.

This is a selection of papers presented at the 30th North American Cystic Fibrosis Conference held in Orlando in October 2016. The papers discussed are thought to be of particular interest to CF caregivers in the UK. We highlight the major themes covered in the conference including novel therapies, recently published and proposed guidelines and insights from registry studies.

A Scandcleft randomised trials of primary surgery for unilateral cleft lip and palate: 1. Planning and management.

Background And Aims: Longstanding uncertainty surrounds the selection of surgical protocols for the closure of unilateral cleft lip and palate, and randomised trials have only rarely been performed. This paper is an introduction to three randomised trials of primary surgery for children born with complete unilateral cleft lip and palate (UCLP). It presents the protocol developed for the trials in CONSORT format, and describes the management structure that was developed to achieve the long-term engagement and commitment required to complete the project.

Scandcleft randomised trials of primary surgery for unilateral cleft lip and palate: 7. Occlusion in 5 year-olds according to the Huddart and Bodenham index.

Background And Aim: Good dentofacial development and good occlusion are main goals in the treatment of UCLP. The aim was to evaluate dental occlusion at age 5 years with the Huddart and Bodenham index after four different protocols of primary surgery for UCLP.

Design: Three parallel randomised controlled trials were undertaken as an international multicentre study by 10 cleft teams in five countries: Denmark, Finland, Sweden, Norway, and the UK.

Scandcleft randomised trials of primary surgery for unilateral cleft lip and palate: 5. Speech outcomes in 5-year-olds - consonant proficiency and errors.

Background And Aim: Normal articulation before school start is a main objective in cleft palate treatment. The aim was to investigate if differences exist in consonant proficiency at age 5 years between children with unilateral cleft lip and palate (UCLP) randomised to different surgical protocols for primary palatal repair. A secondary aim was to estimate burden of care in terms of received additional secondary surgeries and speech therapy.

Scandcleft randomised trials of primary surgery for unilateral cleft lip and palate: 8. Assessing naso-labial appearance in 5-year-olds - a preliminary study.

Background And Aim: Facial appearance is one of the most relevant measures of success in cleft lip and palate treatment. The aim was to assess nasolabial appearance at 5 years of age in all children in the project. In this part of the project the local protocol for lip closure continued to be used because the primary lip and nose operations were not part of the randomisation.

Use of the Brainlab Disposable Stylet for endoscope and peel-away navigation.

Background: Neuronavigation, the ability to perform real-time intra-operative guidance during cranial and/or spinal surgery, has increased both accuracy and safety in neurosurgery [2]. Cranial navigation of existing surgical instruments using Brainlab requires the use of an instrument adapter and clamp, which in our experience renders an endoscope 'top-heavy', difficult to manipulate, and the process of registration of the adapter quite time-consuming.

Methods: A Brainlab Disposable Stylet was used to navigate fenestration of an entrapped temporal horn in a pediatric case.

Treatment prescribing patterns in patients with juvenile idiopathic arthritis (JIA): Analysis from the UK Childhood Arthritis Prospective Study (CAPS).

Objective: Initial treatment of juvenile idiopathic arthritis (JIA) is largely based on the extent of joint involvement, disease severity and ILAR category. The licensing of biologic therapies for JIA has expanded treatment options. The aims of the study are (1) to describe treatment prescribing patterns in JIA over the first 3 years following first presentation to paediatric rheumatology and (2) to determine whether patterns of treatment have changed as biologics have become more widely available.

Association of Dietary Calcium Intake and Body Fat with Hypertension in Indian Adolescents.

To explore association of dietary calcium intake and body composition with blood-pressure, 417 apparently healthy adolescents (218 boys) were studied for anthropometry, blood pressure, body composition and nutrient intakes using standard protocols. Blood pressure correlated negatively with dietary calcium (r= -0.120, P<0.

Short bowel syndrome in children: surgical and medical perspectives.

The main cause of intestinal failure in children is due to short bowel syndrome (SBS) resulting from congenital or acquired intestinal lesions. From the first lengthening procedure introduced by Bianchi, the last three decades have seen lengthening procedures established as fundamental components of multidisciplinary intestinal rehabilitation programs. Debate on indications and timing of the procedures is still open leaving SBS surgical treatment a great challenge.

Granular hemostat deposits mimicking disseminated malignancy.

Hemostatic matrices are a part of the surgeon's armamentarium against profuse intraoperative bleeding. Granular deposits may form after the use of a liquid hemostat which can be mistaken for tumor recurrence or metastatic disease in the setting of neoplasia. We present two cases that highlight the importance of full knowledge of product usage during previous operations and the need for histological examination of these lesions.

The anaesthetic management of children with anterior mediastinal masses.

Children with anterior mediastinal masses may experience serious complications during general anaesthesia. We retrospectively surveyed the records of children with an anterior mediastinal mass who had been admitted to our hospital over a 7 year period. The presence of pre-operative symptoms or signs, findings of any special investigations performed and the anaesthetic outcome were noted.

Carnitine transporter defect: diagnosis in asymptomatic adult women following analysis of acylcarnitines in their newborn infants.

Carnitine transporter defect (CTD) is an autosomal recessive disorder characterized by episodes of non-ketotic hypoglycaemia, hyperammonaemia and liver disease, or by the development of cardiomyopathy, both of which occur in infancy and childhood. Blood carnitine concentrations are extremely low. The diagnosis can be confirmed by finding abnormal fat oxidation and carnitine uptake in skin fibroblasts.