Intestinal failure in children and young people with neurodisabling conditions.

Gastrointestinal dysmotility is common in children and young people with neurodisabling conditions. In this article we seek to highlight the increasing difficulties faced by paediatricians in managing intestinal failure in this patient group. It is becoming clear that, as the median age for survival increases, intestinal failure is a significant problem, and can in some cases become life-limiting. The ethical issues around starting children with life-limiting conditions on parenteral nutrition (PN) are extremely complicated, not least because we are ignorant of the mechanism of intestinal failure in these children, and indeed, which of these children might be able to return to enteral feeding after a period of PN. Our article highlights these issues, drawing on our experience of a particularly difficult case, which we hope will stimulate further discussion among paediatricians providing care for children with neurodisabling conditions.

The epidemiology of coeliac disease in South Wales: a 28-year perspective.

OBJECTIVES: The diagnosis of coeliac disease (CD) has increased in frequency, particularly since the accuracy of serological antibody testing has improved. Previous studies from South Wales have shown an increase in the frequency of diagnosis from 1983 to 2004 with a decrease in specific gastro-intestinal symptoms, as well as an increasing age at diagnosis. AIMS/METHODS: We aimed to determine whether the frequency of diagnosis, the age at presentation and the clinical presentation of CD have changed between 2005 and 2011 compared with previously published data from 1983 to 2004. We reviewed all patients with CD presenting to the South Wales' Regional Centre between 2005 and 2011 and compared the age and documented mode of presentation with previous data from the same area. RESULTS: 163 cases of CD were diagnosed between 2005 and 2011 (23 cases/year) with the median age at diagnosis increasing to 14 years (range 0.8-16 years) compared with 50 cases (8/year) between 1999 and 2004 (median age at diagnosis 8 years), 25 cases (2.5/year) between 1990 and 1998 and 11 cases (1.5/year) between 1983 and 1989. 41% presented with specific gastro-intestinal symptoms, 23% with non-gastro-intestinal features and 36% were asymptomatic and diagnosed after serological screening of high-risk groups. Compared with the most recent previous study from the same population, the percentage of patients presenting with gastro-intestinal symptoms remain similar (42% vs 41%) but patients diagnosed after targeted screening had increased from 26% to 36%. CONCLUSIONS: The frequency of diagnosis of CD in this defined population has continued to rise, with an increase in the median age at diagnosis, and over 50% of patients exhibited few or no symptoms.

Management of gastroenteritis over 10 years: changing culture and maintaining the change.

OBJECTIVE: To examine the impact of an implementation program on adherence to a guideline for the management of acute gastroenteritis. DESIGN: Using four retrospective audits over a 10-year period, the authors examined the change in practice and maintenance of that change following a targeted implementation program for the clinical guideline. SETTING: Tertiary children's hospital in South Wales. PARTICIPANTS: 447 children aged less than 5 years, admitted to hospital with acute gastroenteritis, comprising four cross-sectional samples (106 in 1999, 153 in 2002, 99 in 2004, 89 in 2009). MAIN OUTCOME MEASURES: Age of child, hydration status, method of rehydration and duration of admission, for each audit, with an implementation strategy delivered after the second audit. RESULTS: In 1999 and 2002, intravenous rehydration was used in 20% and 15% of cases, respectively. After the implementation program in 2004, compared to 1999, there was a significant decrease in the intravenous rehydration rate to 4% in 2004 (p<0.001); in 2009 the intravenous rehydration rate was maintained at a low level of 6% (p<0.001). CONCLUSION: It was only after the implementation program that a change in practice was achieved. Once change had been accepted, it was maintained even in the absence of targeted training. Audit does not improve clinical practice unless, in addition, there is a clear, succinct guideline with an implementation programme in place.

Incidence and presentation of reported coeliac disease in Cardiff and the Vale of Glamorgan: the next 10 years.

OBJECTIVE: To determine whether there is a continued increase in the incidence of coeliac disease (CD) in the population of Cardiff and the Vale of Glamorgan between 1996 and 2005 compared with previous data for 1981-1995, and to describe the presenting features during this time. DESIGN: Retrospective case-finding study using pathology, dietetic and clinical records held in hospitals and general practice within Cardiff and the Vale of Glamorgan. All local consultants including those at private hospitals were contacted. Incidence rates were calculated using the Welsh Assembly Government's mid-year estimates. RESULTS: In total, 347 newly diagnosed cases of CD (42 children, 305 adults) were detected. Compared with previous published data, incidence rates in adults per 100 000 have increased from 3.08 at the end of 1995 to 11.13 in 2005. In children, the disease incidence has trebled to 6.89 per 100 000. There have been some changes in presenting symptoms, with a marked preponderance of abdominal pain and bloating in women (P<0.05). There has been a 14-fold increase in the numbers of patients undergoing coeliac serology testing from 1996 to 2005, associated with an increased absolute number of new cases. However, the proportion of new cases diagnosed compared with numbers of serological tests performed decreased from 5.8 to 1.1%. CONCLUSION: The incidence of CD in children and adults has markedly increased. One of the most striking features of our data in adult CD is the increasing frequency of abdominal pain and bloating in the female cohort. Incorporation of antibody testing into clinical guidelines is likely to result in a wider spectrum of individuals with nonspecific gastrointestinal symptoms being investigated and diagnosed with CD in the future.

The provision of paediatric gastrointestinal endoscopy services in the United Kingdom.

OBJECTIVE: There are no specific data available regarding paediatric endoscopy provision in the UK and anecdotal experiences suggest that such provision varies widely between the units. The aim of our study was to identify the current provision of paediatric endoscopy services in the UK, the number of endoscopies performed in each unit, the number of operators performing these endoscopies and whether endoscopies were performed under sedation or general anaesthesia. METHODS AND RESULTS: An email questionnaire was sent to all 31 units in the UK performing paediatric endoscopies and responses were received from 25 centres (81%). The median number of total endoscopies (upper and lower) per unit each year was 332 (range 64-2040). The median number of gastrosopy per consultant in each centre was 101 (range 20-288) and median number of colonoscopies performed per consultant per year was 49 (range 10-215). 18 of the 25 centres performed all endoscopies under general anaesthesia with 7 centres using sedation as well as general anaesthesia. Percutaneous endoscopic gastrostomy insertion (PEG) was performed in 24 out of 25 centres with the service undertaken by paediatric surgeons in 11 centres. 11 centres provided formal out of hours endoscopy services. CONCLUSION: There is wide variation in paediatric endoscopy provision and the British Society of Paediatric Gastroenterology, Hepatology and Nutrition (BSPGHAN) endoscopy working group is collaborating with the Joint Advisory Group (JAG) to provide specific standards for paediatric endoscopy services in the UK.