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This editorial perspective summarises the key findings of the independent review of gender identity services for children and young people commissioned by the National Health Service (NHS) in England (the Cass Review). Although the evidence underpinning endocrine interventions in this group of young people remains weak, there is much that mental health practitioners can do to improve their well‐being and support their families. The controversies surrounding this group of young people have disempowered local professionals, who have lost confidence in their ability to provide care for them. The reality is that CAMHS professionals already have all the necessary transferrable skills and with a small amount of top up training, they can make a profound difference to the lives of this vulnerable group, which has been marginalised in our healthcare system. [ABSTRACT FROM AUTHOR]

Summary: This editorial describes the Cass Review findings and the extraordinary challenge we all face in managing uncertainty amid a toxic and highly polarised debate. Children and young people will only get the best care if patients and professionals join forces to seek answers collaboratively and respectfully. [ABSTRACT FROM AUTHOR]

This study aimed to (1) explore views of known experts leading on transition, (2) gather insights on the essential features of transitional care models and (3) highlight opportunities for and barriers to change: to inform core elements of benchmarks for transitional care. We held three workshops (n = 20) and used a telephone interview (n = 1) with health and social care professionals with expertise working with young people with a range of health conditions and disabilities. The workshops included individual brainstorming and group discussion. Data were analysed using qualitative content analysis. The general consensus from stakeholders' discussions about transition was that 'things have become stuck'. Themes included: professionals' attitudes towards and knowledge about young people and transition, organizational barriers and 'lack of joined-up thinking' between services. Our work offers further insight into experts' perceptions of transition services within the United Kingdom. It is clear that there is still much to be done to improve transition, to better meet the needs of young people and parents and begin to offer equitable access to transitional care programmes. The benchmarks offer a starting point for professionals seeking to improve transition through enabling the identification of gaps in services and providing a platform to share successful practice initiatives. [ABSTRACT FROM AUTHOR]

The article focuses on findings of a study related to childhood survival in Great Britain, as of October 2010. It states that the Millennium Development Goal of United Nations have significantly helped improve childhood survival in the country by reducing mortality among children under the age of five, and cites the trends in child mortality in Great Britain and Europe. It mentions possible reasons for higher child and adolescent mortality like poverty and socioeconomic inequalities.

Purpose: The purpose of the study was to determine the proportion of pediatric deaths investigated by HM Coronial autopsy which were potentially preventable deaths due to treatable natural disease, and what implications such findings may have for health policies to reduce their occurrence. Methods: A retrospective study of 1779 autopsies of individuals between 7 days and 14 years of age requested by HM Coroner, taking place in one specialist pediatric autopsy center, was undertaken. Cases were included if they involved a definite natural disease process in which appropriate recognition and treatment was likely to have affected their outcome. Strict criteria were used and cases were excluded where the individual had any longstanding condition which might have predisposed them to, or altered the recognition of, acute illness, or its response to therapy. Results: Almost 8 % (134/1779) of the study group were potentially preventable deaths as a result of natural disease, the majority occurring in children younger than 2 years of age. Most individuals reported between 1 and 7 days of symptoms before their death, and the majority had sought medical advice during this period, including from general practitioners within working hours, and hospital emergency departments. Of those who had sought medical attention, around one-third had done so more than once (28 %, 15/53). Sepsis and pneumonia accounted for the majority of deaths (46 and 34 % respectively), with all infections (sepsis, pneumonia and meningitis) accounting for 110/134 (82 %). Conclusion: Around 10 % of pediatric deaths referred to HM Coroner are potentially preventable, being the result of treatable natural acute illnesses. In many cases medical advice had been sought during the final illness. The results highlight how a review of autopsy data can identify significant findings with the potential to reduce mortality, and the importance of centralized investigation and reporting of pediatric deaths. [ABSTRACT FROM AUTHOR]

Introduction: Despite clinical advances in neonatal and paediatric palliative care, there is limited educational provision to underpin practice. To develop appropriate educational content, the needs of staff working in this area must be identified. Aim: To explore the educational needs of staff working with families with palliative or end-of-life care requirements. Methods: A training needs analysis (TNA) explored the perceived knowledge, confidence, and support of neonatal and paediatric health professionals in a tertiary children's hospital in London. Results: An online Likert scale TNA was completed by 111 participants. The results indicated that the staff did not feel educationally prepared in their working areas, despite having regular contact with families with palliative or end-of-life care issues. Discussion: Understanding the educational needs of staff can underpin the development of appropriate educational interventions. These modules require evaluation to determine their impact on health professionals' knowledge and confidence. [ABSTRACT FROM AUTHOR]

Introduction: Despite clinical advances in neonatal and paediatric palliative care, there is limited educational provision to underpin practice. To develop appropriate educational content, the needs of staff working in this area must be identified. Aim: To explore the educational needs of staff working with families with palliative or end-of-life care requirements. Methods: A training needs analysis (TNA) explored the perceived knowledge, confidence, and support of neonatal and paediatric health professionals in a tertiary children's hospital in London. Results: An online Likert scale TNA was completed by 111 participants. The results indicated that the staff did not feel educationally prepared in their working areas, despite having regular contact with families with palliative or end-of-life care issues. Discussion: Understanding the educational needs of staff can underpin the development of appropriate educational interventions. These modules require evaluation to determine their impact on health professionals' knowledge and confidence. [ABSTRACT FROM AUTHOR]

The article focuses on poor medical care services of children in Great Britain and also offers suggestions for their improvement. It informs that child health services in the country are inferior in comparison to other European countries and that there is no comprehensive evaluation of the quality of healthcare services for children. As per Great Britain's Commission for Healthcare Audit and Inspection, a public body sponsored by the country's Department of Health, 46 percent of acute trusts of the nation are weak in providing paediatric outpatient care. It suggests that gap between primary and secondary care should be filled by giving high quality emergency care for minor diseases. It also recommends for mandatory training of general practitioners in paediatrics.

The editor discusses results of an international literature review by the National Patient Safety Agency, which outlines the factors for reducing paediatric medication error through the use of quality improvement networks. The review explores the use of a collaborative approach to the development of interventions intending to reduce the impacts of medicine error in child medications. The editor also highlights the developing pragmatic approach on electronic prescribing (e-prescribing) medicine.

Background As clinical leadership becomes an expectation in medical professionals of the future, careful consideration needs to be given to the planning, design, structure and evaluation of leadership development programmes. Aim To take a structured approach to the designing and planning of a work-based leadership development programme for paediatric doctors in training. Methods A total of 30 trainees within the London Specialty School of Paediatrics were given the opportunity to develop their leadership skills by working in small groups alongside the training programme directors in a number of areas of the work of the School. The design of the leadership programme was built around learning from three key sources, namely literature review, focus groups involving the participants, and small-groupworkshops, all of which contributed to the thinking behind the ethos of the programme. Learning outcomes were derived from the Medical Leadership Competency Framework. A comprehensive evaluation of the programme, using a combination of peer assessment and self-assessment tools, was planned and piloted prior to the start of the programme. Results The literature review found very few leadership development programmes that had been fully evaluated. Where planning had been described, the benefit of aligning programmes to existing leadership curricula was highlighted. The majority of programmes took learners out of their working environment to learn. Although the focus groups provided useful information about the leadership perspectives of trainees, above all it was the process of participation that engendered confidence and a feeling of ownership in the planning and structure of the programme. This concept of trainee participation was taken further with the small-group workshops which used peer learning to produce practical ideas for the benefit of the group. Conclusions Leadership development programmes, as with all educational interventions, need to be properly planned and evaluated. Programmes need to be aligned to leadership curricula, and should incorporate the views of participants. There are also benefits to considering an approach to learning that is embedded in the clinical workplace. [ABSTRACT FROM AUTHOR]

We aimed to improve the understanding of genotype–phenotype correlations in Rett syndrome (RS) by adopting a novel approach to categorising phenotypic dimensions – separating typicality of presentation, outcome severity and age of onset – and by classifying MECP2 mutations strictly by predicted functional attributes. MECP2 mutation screening results were available on 190 patients with a clinical diagnosis of RS (140 cases with classic RS, 50 with atypical RS). 135 cases had identified mutations. Of the 140 patients, 116 with classic RS (82.9%) had an identified mutation compared with 19 of 50 patients (38%) with an atypical presentation. Cases with early onset of regression and seizures, and those with clinical features that might indicate alternative aetiologies, were less likely to have mutations. Individuals with late truncating mutations had a less typical presentation than cases with missense and early truncating mutations, presumably reflecting greater residual function of MECP2 protein. Individuals with early truncating mutations had a more severe outcome than cases with missense and late truncating mutations. These findings held when restricting the analysis to cases over 15 years of age and classic cases only. Previous findings of variation in severity among the common mutations were confirmed. The approach to phenotypic and genotypic classification adopted here allowed us to identify genotype–phenotype associations in RS that may aid our understanding of pathogenesis and also contribute to clinical knowledge on the impact of different types of mutations.European Journal of Human Genetics (2005) 13, 1121–1130. doi:10.1038/sj.ejhg.5201471; published online 3 August 2005 [ABSTRACT FROM AUTHOR]

Presents a diagnosis of autism. Fact that parents want autism to be diagnosed as early as possible; Identification of autism; Increase in the number of children diagnosed with autism; Possible outcomes for measuring the success of therapeutic teaching intervention. INSET: A parent's perspective.

It has long been recognized that there is phenotypic overlap between Rett syndrome (RS) and autism. Advances in our clinical and genetic understanding of RS over the past decade have made clear that the cause and course of RS and autism are distinct (except perhaps in a few cases). Despite this, further delineation of the phenotypic overlap between RS and autism is warranted to enhance clinical decision-making and to further understanding of neuropathological development in both disorders. The present study measured autistic symptoms using the Autism Behavior Checklist (ABC) in a sample of girls with RS and a comparison group of girls with severe and profound mental retardation (SMR). Controlling for developmental level and motor ability, girls with RS scored more highly than those with SMR on the Sensory and Relating subscales. In contrast, there were no group differences on the Body and Object use, Language and Social and Self-help subscales. Further work on the characterisation of the behavioral phenotype of genetic disorders such as RS and autism may aid in identifying the neuropathogenic processes that lead from gene-to-brain-to-behavior. [ABSTRACT FROM AUTHOR]

Abstract Problem: Compliance with UK regulations on junior doctors' working hours cannot be achieved by manipulating rotas that maintain existing tiers of cover and work practices. More radical solutions are needed. Design: Audit of change. Setting: Paediatric night rota in large children's hospital. Key measures for improvement: Compliance with regulations on working hours assessed by diary cards; workload assessed by staff attendance on wards; patient safety assessed through critical incident reports. Strategies for change: Development of new staff roles, followed by change from a partial shift rota comprising 11 doctors and one senior nurse, to a full shift night team comprising three middle grade doctors and two senior nurses. Effects of change: Compliance with regulations on working hours increased from 33% to 77%. Workload changed little and was well within the capacity of the new night team. The effect on patient care and on medical staff requires further evaluation. Lessons learnt: Reduction of junior doctors' working hours requires changes to roles, processes, and practices throughout the organisation. [ABSTRACT FROM AUTHOR]

Systematic data from a multidisciplinary clinical assessment of a large series of females with Rett syndrome (RS; n=87) is presented. Participants' ages ranged from 2 years 1 month to 44 years 10 months. Areas assessed included oromotor skills, feeding problems, growth, breathing abnormalities, mobility, postural abnormalities and joint deformities, epilepsy, hand use and stereotypies, self-care, and cognitive and communication skills. Many previously reported trends in the presentation of RS over time were confirmed, notably the increasingly poor growth and near pervasiveness of fixed joint deformities and scoliosis in adulthood. In contrast, there was a slight trend towards improved autonomic function in adulthood, whereas feeding difficulties increased into middle childhood and then reached a plateau. Improvements in mobility into adolescence were followed by a decline in those skills in adulthood. Levels of dependency were high, confirming findings from previous studies. Despite the presence of repetitive hand movements, a range of hand-use skills was seen in individuals of all ages. Cognitive and communication skills were limited, but there was little evidence of deterioration of these abilities with age. These findings confirm that RS is not a degenerative condition and indicate that intervention and support to maintain and increase motor skills, daily living skills, and cognitive and communicative functioning are appropriate targets for individuals with RS. [ABSTRACT FROM AUTHOR]

Background: Although physical features, including loss of hand skills, deceleration of head growth, spasticity and scoliosis, are cardinal features of Rett syndrome (RS), a number of behavioural features are also associated with the disorder, including hand stereotypies, hyperventilation and breath holding. No study has tested the specificity of these behavioural features to individuals with RS, compared to individuals with severe to profound mental retardation (SMR). Method: A novel checklist of characteristic RS behavioural and emotional features, the Rett Syndrome Behaviour Questionnaire (RSBQ), was developed to test the type and specificity of behavioural features of RS against those found in girls with SMR. Results: After controlling for the effects of RS-related physical disabilities, the RSBQ discriminated between the groups. Some aspects of the behaviours found to be specific to RS are included in the necessary or supportive RS diagnostic criteria, notably hand behaviours and breathing problems. Additional behavioural features were also more frequently reported in the RS than the SMR group, including mood fluctuations and signs of fear/anxiety, inconsolable crying and screaming at night, and repetitive mouth and tongue movements and grimacing. Conclusions: Full validation of the scale requires confirmation of its discriminatory power and reliability with independent samples of individuals with RS and SMR. Further delineation of the specific profile of behaviours seen in RS may help in identification of the function of the MECP2 gene and in improved differential diagnosis and management of individuals with RS. [ABSTRACT FROM AUTHOR]

Prompt and accurate diagnosis of Munchausen's syndrome by proxy is essential in order to minimise the risk of iatrogenic and parentally-induced harm to the child. However, inaccurate diagnosis of MSBP may have devastating consequences for the child, family and professionals involved. 'Psychiatric' Munchausen's syndrome by proxy, which involves the presentation of signs and symptoms suggestive of a psychiatric disorder in the child, is even more difficult to diagnose and requires an unbiased and full assessment by the appropriate multidisciplinary team. [ABSTRACT FROM AUTHOR]

Prompt and accurate diagnosis of Munchausen's syndrome by proxy is essential in order to minimise the risk of iatrogenic and parentally-induced harm to the child. However, inaccurate diagnosis of MSBP may have devastating consequences for the child, family and professionals involved. ‘Psychiatric’ Munchausen's syndrome by proxy, which involves the presentation of signs and symptoms suggestive of a psychiatric disorder in the child, is even more difficult to diagnose and requires an unbiased and full assessment by the appropriate multidisciplinary team. [ABSTRACT FROM AUTHOR]

Developmental setback in children initially thought to be of normal cognitive potential is a serious complication of severe visual impairment; the prevalence, diagnostic specificity, clinical presentation, and factors that contribute to its genesis require systematic investigation. The findings are reported of a retrospective case review over a 15 year period of children attending the developmental vision clinic at the Wolfson Centre of the Institute of Child Health. One hundred and two children met the inclusion criteria of a period of normal development confirmed at initial assessment when aged less than 16 months, absence of additional disabilities, and follow up to at least 2.5 years of age. Developmental setback in their second or third year occurred in 10 (31%) of 32 children who were totally blind throughout (minimal perception of light or less), one (4%) of 25 who, though blind at first assessment, showed visual improvement, and none of 49 children with better vision throughout (awareness for near, large objects). This represents a significantly greater risk for totally blind children than for the other groups. The course and characteristics of the affected children varied, but all had visual diagnoses involving the nervous elements of the visual system, and 60% had major social adversity factors. The role of primary maldevelopment of the central nervous system, the degree of visual impairment, the developmental and emotional climate, and the stage of attentional and behavioural development in the causation of adverse developmental outcome are discussed. [ABSTRACT FROM AUTHOR]

Parental and professional responses to questionnaires evaluating a paediatric disability service are reported and the viability of auditing structural, process, and outcome aspects of clinical practice are discussed. Expectations of waiting time to first appointment (met for only 52% of consumers) illustrate structural issues. Process issues are reflected in consumer reactions to outreach work (for example, 94% of parents and 84% of professionals found this supportive). Outcome measures such as consumer satisfaction with the service (76% of consumers reported being 'very satisfied' and 20% 'fairly satisfied') suggest that service aims are being met. Good concurrence of service aims with consumer needs is indicated by parental reasons for referral (for example, 75% for diagnostic help, 73% for a better understanding of the disorder, 88% for practical help), referrers' reasons (for example, 55% for a second diagnostic opinion, 45% due to lack of local expertise), and reports from most other professionals involved with the case that a similar service was not provided locally. [ABSTRACT FROM AUTHOR]

Many children with sensory impairment are part of a growing cohort of children with medical complexity (CMC), characterized by Cohen et al.[3] as 'those with significant and/or multiple chronic health conditions, functional limitations, and substantial healthcare use'. My question is, therefore: to what extent is this "our problem" as paediatric neurologists and neurodisability consultants? However, I believe that paediatric neurologists and neurodisability consultants still retain something very important: an understanding of the fundamentals of disability and a commitment to maximizing the potential of all children, regardless of the severity of their condition. [Extracted from the article]

This commentary is on the original article by Joachim et al. on pages 201–206 of this issue. [ABSTRACT FROM AUTHOR]

The article focuses on the decision-making power and ethics in clinicians or the hospital staff when dealing disagreement between the patients and the clinicians. It talks about such disagreement growing due to the mass demonstrations and the social media. It tells about the importance of social change in the medical care field which is influenced by death and loss in the society.

The author reflects on the aspects of child protection as a part of paediatric practice in Great Britain by referring to a research by A.M. Kemp and colleagues concerning child abuse. Topics discussed include role of paediatricians in providing right diagnosis of physical abuse, need for training of social workers and teachers to improve early detection of physical abuse, and guidelines of the General Medical Council regarding the duty of doctors to report child abuse and neglect.

In this article the author discuses the increasing availability of a wide range of high-quality electronic-learning (e-learning) resources. He opines that the approach of computers alongwith fast Internet connections is increasing the development of e-learning methods of teaching. He suggests that the institutions should provide their educational materials in an accessible format for free use by others throughout the world. He has also given his views on the emertging Web 2.0 applications.

An introduction is presented in which the author discusses various reports within the issue on topics including increased survival rates for infants born between 22 and 25 weeks gestation, advances in pediatric surgical techniques, and developments in pediatric palliative care.

The article discusses the delay in the implementation of rules on cigarette packaging law in Great Britain. Topics covered include the need for the government to table the regulations in order to complete the parliamentary scrutiny processes in time and the need for health professionals to know if the prime minister will stand up to the tobacco industry and make commitments to introduce standardised packaging.

In this article, the author offers information on a survey by the Royal College of Paediatrics and Child Health (RCPCH) for Urgent and Emergency Care (U&EC) setting. He states that in this survey, children delivered their view on what aspects of U&EC setting are most important. The survey also included cognitive testing of the draft survey and it survey was counseled in several U&EC organizations in England.