Management of Ganglioneuroma and Ganglioneuroblastoma Intermixed: A United Kingdom Children's Cancer and Leukaemia Group (UK CCLG) Nationwide Study Report.

Background: Ganglioneuroblastoma intermixed (GNBi) and ganglioneuroma (GN) represent benign variants of peripheral neuroblastic tumours. While historically surgical resection was recommended, watchful active observation has become the accepted management for a subset of patients.

Objectives: To review clinical features, biology, natural history and management of a retrospective UK CCLG study cohort of GN and GNBi, and compare outcomes of patients treated with surgical resection or watchful active observation.

Exploring the complexity of cystic fibrosis (CF) and psychosocial wellbeing in the 2020s: Current and future challenges.

Cystic fibrosis (CF) is traditionally associated with considerable and progressive multisystem pathology, onerous treatment burden, complex psychosocial challenges, and reduced life-expectancy [1-9].This decade has seen transformative change in management for many, but not all, people with CF. The most notable change comes from Cystic Fibrosis Transmembrane Receptor (CFTR) modulators, which bring significant benefits for people who are eligible for, and able to access, them [10].

Successful treatment approaches for tumoral calcinosis in children and young people: A condition of diverse pathogenesis.

Background: Ectopic calcification is inappropriate biomineralization of soft tissues occurring due to genetic or acquired causes of hyperphosphataemia and rarely in normophosphataemic individuals. Tumoral Calcinosis (TC) is a rare metabolic bone disorder commonly presenting in childhood and adolescence with periarticular extra-capsular calcinosis. Three subtypes of TC have been recognised: primary hyperphosphataemic familial TC (HFTC), primary normophosphataemic familial TC and secondary TC most commonly seen in chronic renal failure.

Evaluating end of treatment care of young people with cancer.

Background: Existing literature implies there may be gaps in post-treatment support for young people with cancer. This service evaluation explored the needs and experiences of young people when ending cancer treatment in a UK children's hospital to inform service provisions.

Methods: Semi-structured interviews were conducted with nine young people, aged 13-18 years, who had finished active cancer treatment and were receiving follow-up care.

Quality of Life in Children and Young People With Congenital Adrenal Hyperplasia-UK Nationwide Multicenter Assessment.

Context: Quality of life (QoL) has been inconsistently reported in children and young people (CYP) with congenital adrenal hyperplasia (CAH).

Objective: Assess QoL in CYP with CAH in the UK alongside biometric and androgen profiles.

Design: To define the evidence base for health care delivery, we conducted a cross-sectional study in CYP with CAH in the UK.

Rare variants in the MECP2 gene in girls with central precocious puberty: a translational cohort study.

Background: Identification of genetic causes of central precocious puberty have revealed epigenetic mechanisms as regulators of human pubertal timing. MECP2, an X-linked gene, encodes a chromatin-associated protein with a role in gene transcription. MECP2 loss-of-function mutations usually cause Rett syndrome, a severe neurodevelopmental disorder.

Experiences of Parenting a Child Receiving Dexamethasone During Maintenance Chemotherapy for Acute Lymphoblastic Leukemia.

The purpose of this research was to understand the experience of parenting a child receiving dexamethasone during maintenance chemotherapy for acute lymphoblastic leukemia (ALL). Previous research has shown that dexamethasone's high level of toxicity causes many physical, behavioral, and emotional side effects, which reduce the quality of life during ALL treatment. Less is known about the experience of parenting a child receiving dexamethasone and the impact on the parent-child relationship.

Examining the Psychosocial Well-Being of Children and Adolescents With Coeliac Disease: A Systematic Review.

This review aimed to synthesize the available literature regarding the psychosocial well-being of children and adolescents with coeliac disease (CD). Research on psychosocial well-being outcomes in children and adolescents with CD under the age of 18 were identified through a systematic search in the PsychInfo, Medline, Embase, and Cumulative Index to Nursing and Allied Health Literature (CINAHL) databases in July 2021. Outcomes, including health-related quality of life (HRQOL), psychological adjustment, mental health, and social functioning were examined.

10 Year Follow-Up of Randomized Trial of Laparoscopic Nissen Versus Thal Fundoplication in Children.

The aim of this study was to compare the long-term outcomes of laparoscopic complete (Nissen) fundoplication (LNF) with laparoscopic partial (Thal) fundoplication (LTF) in children. This is the only prospective, randomized study to follow patients up for more than 10 years. Interim results published in 2011 at median 2.

Suprarenal Masses in Very Young Infants: Is It Safe to Watch and Wait? Report of a SIOPEN Observational Study Results.

To assess whether expectant observation of infants ≤ 90 days old with small suprarenal masses (sSRMs) could avoid unnecessary surgery without impacting outcome. Infants ≤ 90 days with a ≤ 5 cm mass, without midline extension or lymph node or distant spread were registered (ClinicalTrials.org:NCT01728155).

Health status of children and young persons with congenital adrenal hyperplasia in the UK (CAH-UK): a cross-sectional multi-centre study.

Objective: There is limited knowledge on the onset of comorbidities in congenital adrenal hyperplasia (CAH) during childhood. We aimed to establish the health status of children with CAH in the UK.

Design And Methods: This cross-sectional multicentre study involved 14 tertiary endocrine UK units, recruiting 101 patients aged 8-18 years with classic 21-hydroxylase deficiency and 83 controls.

"I sort of never felt like I should be worried about it or that I could be worried about it'" an interpretative phenomenological analysis of perceived barriers to disclosure by young people with coeliac disease.

Objectives: There has been little research in the United Kingdom regarding young people's experiences of disclosure of psychological difficulties relating to coeliac disease (CD) to others, particularly healthcare professionals. This study sought to address this systematically with a focus on the lived experiences of young people with CD. This study aimed to gain insight into how paediatric gastroenterology services could improve the patient experience for those with CD and support the identification of patients who may benefit from further psychological support.

The experiences of adolescents diagnosed with functional gastrointestinal disorders: An interpretative phenomenological analysis.

The aim of this study was to provide a qualitative perspective of adolescents' experiences of functional gastrointestinal disorders. In-depth semi structured interviews were conducted with eleven adolescents aged 11-16. The transcripts were analysed using Interpretative Phenomenological Analysis.

Exploring factors which contribute to the resilience of nurses working in the neonatal care unit: A grounded theory study.

Objectives: This study aimed to produce a theoretical model to understand factors which affect the resilience of neonatal nurses. Nurses experience high levels of burnout due to the occupational stress they are exposed to. Burnout negatively affects both staff and patient outcomes, and in this population would likely affect the establishment of crucial early caregiver-infant relationships in the neonatal unit.

A Feeling of Otherness: A Qualitative Research Synthesis Exploring the Lived Experiences of Stigma in Individuals with Inflammatory Bowel Disease.

Inflammatory bowel disease (IBD) consists of Crohn's disease and ulcerative colitis, chronic conditions involving inflammation and ulceration of the gastrointestinal tract. Individuals with IBD may be susceptible to experiencing health-related stigma: experienced, perceived, or internalised social exclusion, rejection, blame, or devaluation resulting from negative social judgements based on the disease. This qualitative research synthesis draws together findings from 38 studies describing lived experiences to develop a unified interpretative account of the experience of stigma in IBD.

Neurological manifestations of SARS-CoV-2 infection in hospitalised children and adolescents in the UK: a prospective national cohort study.

Background: The spectrum of neurological and psychiatric complications associated with paediatric SARS-CoV-2 infection is poorly understood. We aimed to analyse the range and prevalence of these complications in hospitalised children and adolescents.

Methods: We did a prospective national cohort study in the UK using an online network of secure rapid-response notification portals established by the CoroNerve study group.