COVID-19 in congenital heart disease (COaCHeD) study.

Background: COVID-19 has caused significant worldwide morbidity and mortality. Congenital heart disease (CHD) is likely to increase vulnerability and understanding the predictors of adverse outcomes is key to optimising care.

Objective: Ascertain the impact of COVID-19 on people with CHD and define risk factors for adverse outcomes.

Consensus guidelines on the management of musculoskeletal infection affecting children in the UK.

Aims: The aim of this study was to determine the consensus best practice approach for the investigation and management of children (aged 0 to 15 years) in the UK with musculoskeletal infection (including septic arthritis, osteomyelitis, pyomyositis, tenosynovitis, fasciitis, and discitis). This consensus can then be used to ensure consistent, safe care for children in UK hospitals and those elsewhere with similar healthcare systems.

Methods: A Delphi approach was used to determine consensus in three core aspects of care: 1) assessment, investigation, and diagnosis; 2) treatment; and 3) service, pathways, and networks.

Hypogonadism in the Prader-Willi syndrome from birth to adulthood: a 28-year experience in a single centre.

Background: Hypogonadism is a key feature of Prader-Willi syndrome (PWS) but clear strategies for hormone replacement are lacking.

Objective: To evaluate gonadal status and outcome in patients attending a Scottish PWS clinic from 1991-2019.

Methods: In 93 (35F:56M) patients, median follow-up 11.

Workplace-based assessment: how to use case-based discussion as a formative assessment.

Workplace-based assessments are increasingly used as a way of gaining insight into clinician performance in real-life situations. Although some can be used to inform a summative (pass/fail) assessment, many have a much greater role in the formative assessment of trainees, and can be used as tools for teaching and training and in identifying the development needs of trainees. There is considerable variation between different European countries in the use of formative, workplace-based assessment, such as a structured case-based discussion (CbD), during training.

Paediatric burns in LMICs: An evaluation of the barriers and facilitators faced by staff involved in burns education training programmes in Blantyre, Malawi.

Background: A burn prevention and education programme - the Reduction of Burn and Scald Mortality and Morbidity in Children in Malawi project - was implemented from January 2010-2013 in Queen Elizabeth Central Hospital, Malawi. This study aimed to investigate the barriers and facilitators of implementing education-training programmes.

Methods: Semi-structured interviews with 14 Scottish and Malawian staff delivering and receiving teaching at training education programmes were conducted.

Challenges of Assessing Maltreated Children Coming into Foster Care.

Children who have experienced early adversity have been known to be at risk of developing cognitive, attachment, and mental health problems; therefore, it is crucial that children entering foster care can be properly assessed as early as possible. There are known difficulties in assessing children in foster care, for example, in finding a reliable informant. An ongoing randomised controlled trial in Glasgow, Scotland, recruiting infants entering foster care, provides a unique opportunity to explore some of the issues which need to be considered when assessing these children.

Two-Strip Technique to Repair Common Atrioventricular Valve Regurgitation in Single-Ventricle Palliation.

A common atrioventricular valve (CAVV) presents a challenge in single-ventricle palliation (SVP) because of its propensity to leak, with CAVV regurgitation being an important risk factor for repeated operation and mortality. We propose a new technique of CAVV repair in the context of SVP. The method uses the techniques of complete repair of antrioventricular septal defect while maintaining free communication at atrial and ventricular levels necessary for single-ventricle physiology.

Changing indications for paediatric tracheostomy and the role of a multidisciplinary tracheostomy clinic.

Objective: This paper presents our experience of managing children with a tracheostomy in a multidisciplinary team clinic consisting of an ENT consultant, paediatric respiratory consultant, a nurse specialist, and speech and language therapist.

Method: A retrospective case note review was conducted of all children seen in the multidisciplinary team tracheostomy clinic (at a tertiary paediatric hospital) between February 2009 and September 2014.

Results: Ninety-seven patients were examined.

The pitfalls associated with urinary steroid metabolite ratios in children undergoing investigations for suspected disorders of steroid synthesis.

Background: Urinary steroid metabolite ratios may improve the diagnostic yield of potential disorders of steroid hormone synthesis.

Objectives: To investigate the range of ratios and their predictive value in children with suspected disorders of steroid synthesis.

Design And Methods: Twelve ratios were calculated on steroid metabolite data analysed by gas chromatography-mass spectrometry in urine samples collected between 2008-2010 from 93 children.

Associations between social isolation, pro-social behaviour and emotional development in preschool aged children: a population based survey of kindergarten staff.

Background: The impact of peer relationships has been extensively reported during adolescence, when peer influence is generally considered to be at its greatest. Research on social isolation during childhood has found associations with school achievement, future relationships and adult mental health. Much of the evidence is derived from either parent or child-rated assessment of peer relationships, each of which have their limitations.

Building physical activity and sedentary behavior support into care for youth with type 1 diabetes: patient, parent and diabetes professional perceptions.

Objective: To explore stakeholder's perceptions of physical activity (PA) and sedentary behaviour support in youth with type 1 diabetes (T1D), to aid intervention development.

Methods: Primary data were collected between February and September 2012. Patients (N = 16), parents (N = 16), and professionals (N = 9) were recruited from a diabetes clinic for a qualitative study.

Complications of ventilation tube insertion in children with and without cleft palate: a nested case-control comparison.

Importance: Optimizing hearing in patients with cleft lip and/or palate (CLP) by early recognition and management of otitis media with effusion is essential for speech development. Some evidence has suggested higher complication rates from ventilation tube (VT) insertion in patients with CLP and has led to a trend not to treat these patients surgically. However, studies have failed to match comparison groups for age and sex.

Germline FH mutations presenting with pheochromocytoma.

Context: At least a third of the patients with pheochromocytoma (PCC) or paraganglioma (PGL) harbor an underlying germline mutation in a known PCC/PGL gene. Mutations in genes (SDHB, SDHD, SDHC, and SDHA) encoding a component of the tricarboxylic acid cycle, succinate dehydrogenase (SDH), are a major cause of inherited PCC and PGL. SDHB mutations are also, albeit less frequently, associated with inherited renal cell carcinoma.

Myoclonic seizures in a preterm baby: is this a presentation of venlafaxine withdrawal?

Venlafaxine, a serotonin and norepinephrine reuptake inhibitor, is increasingly used in pregnant women with pre-existing depression who require continued treatment. However, its in uteroeffects on the developing fetus are not clear. Herein, we report the unusual presentation of venlafaxine withdrawal in a female preterm baby of 29 weeks gestation, who presented with myoclonic seizures on her second day of life.

Repair of early left main stem stenosis after switch operation using an autologous innominate vein patch.

We describe the repair of the postoperative left main stem stenosis of a 24-day old male patient who initially underwent arterial switch operation for dextro-transposition of the great arteries. The repair was facilitated by using an autologous left innominate vein patch, while there was a shortage of suitable repair material in this neonate. Postoperative echocardiogram prior to discharge confirmed a satisfactory flow through the left main stem and improved left ventricle function.

A multidisciplinary approach to understanding skeletal dysplasias.

The skeletal dysplasias are a heterogeneous group of conditions of abnormal cartilage and bone development, resulting in a wide range of phenotypes of variable severity from perinatal lethality to mild short stature. Elucidation of the molecular mechanisms underlying these disorders is allowing us to understand more about the etiology of these conditions and classify them based upon the underlying gene defect. This article will discuss the development of bone and cartilage in relation to these conditions, present a clinical approach to their diagnosis and management, and consider new avenues of therapy.

Intraoperative dexamethasone and the risk of secondary posttonsillectomy hemorrhage.

Objective: to determine whether intraoperative dexamethasone is a risk factor for secondary posttonsillectomy bleeding.

Design: retrospective chart review.

Setting: tertiary care referral centres in Scotland.

Review of laparoscopic training in pediatric surgery in the United Kingdom.

Aim: To review the exposure pediatric surgery trainees have to laparoscopic surgery in the United Kingdom (UK).

Methods: A confidential postal questionnaire was sent to all trainees working at registrar level in centers responsible for pediatric surgical training in the UK. Questions assessed the number of consultants with an interest in laparoscopic surgery, types of cases performed laparoscopically, and trainees' role in laparoscopic appendicectomy (LA).

Body composition and nutritional intake in children with chronic kidney disease.

The aim of this study was to assess body composition in children with chronic renal failure (CRF) and post renal transplantation (Tx), and to compare it to body mass index (BMI) and nutritional intake. Dietary assessment using 3-day diaries, total and regional body composition assessment by dual x-ray energy absorptiometry of 50 CRF children (29M, 21F), median age 8.9 yrs and 50 Tx children (32M, 18F), median age 12.

A review of the complications and results of implantation of the AMS artificial urinary sphincter.

The history and evolution of devices designed to achieve urinary continence are reviewed. Passive and active (including volume and pressure regulated) devices are described in detail. Meta-analysis of the published studies revealed that continence improved in 88% of patients and total continence was achieved in 73%.

Sudden death 10 years after patch aortoplasty for coarctation.

A 25-year-old man died 10 years after a Dacron patch was used to repair a coarctation of the aorta. Death was due to rupture of an unrecognized aneurysm at the site of the patch. After the initial operation at the age of 15 years, there had been no signs of residual or recurrent obstruction.