Perspectives of paediatric and adult gastroenterologists on transfer and transition care of adolescents with inflammatory bowel disease.

BACKGROUND: Programmes specific to inflammatory bowel disease (IBD) that facilitate transition from paediatric to adult care are currently lacking. AIM: We aimed to explore the perceived needs of adolescents with IBD among paediatric and adult gastroenterologists and to identify barriers to effective transition. METHODS: A web-based survey of paediatric and adult gastroenterologists in Australia and New Zealand employed both ranked items (Likert scale; from 1 not important to 5 very important) and forced choice items regarding the importance of various factors in facilitating effective transition of adolescents from paediatric to adult care. RESULTS: Response rate among 178 clinicians was 41%. Only 23% of respondents felt that adolescents with IBD were adequately prepared for transition to adult care. Psychological maturity (Mean = 4.3, standard deviation (SD) = 0.70) and readiness as assessed by adult caregiver (Mean = 4, SD = 0.72) were prioritised as the most important factors in determining timing of transfer. Self-efficacy and readiness as assessed by adult caregiver were considered the two most important factors to determine timing of transition by both groups of gastroenterologists. Poor medical and surgical handover (Mean = 4.10, SD = 0.8) and patients' lack of responsibility for their own care (Mean= 4.10, SD = 0.82) were perceived as major barriers to successful transition by both paediatric and adult gastroenterologists. CONCLUSIONS: Deficiencies exist in current transition care of adolescents with IBD in Australia and New Zealand. Standardising transition care practices with strategies aimed at optimising communication, patient education, self-efficacy and adherence may improve outcomes.

Pathological fractures in paediatric patients with inflammatory bowel disease.

UNLABELLED: Paediatric inflammatory bowel disease (IBD), especially Crohn's disease (CD), is commonly associated with poor skeletal health, related to the direct effects of chronic inflammation, prolonged use of glucocorticoid (GC), poor nutrition, delayed puberty and low muscle mass. Low bone mineral density is commonly reported, although the prevalence of long bone fractures may not be increased in these patients. Emerging evidence however suggests that there may be an increased risk of vertebral fractures (VFs) in this group. VFs presenting at diagnosis of paediatric CD, prior to any GC exposure, have been reported, highlighting the deleterious effect of inflammation on skeletal health. This paper reviews the published literature on pathophysiology of skeletal morbidity and fractures in paediatric IBD, illustrated with a new case report of multiple VFs in a prepubertal girl with CD, soon after diagnosis, who received minimal amounts of oral GC. Optimising control of disease, addressing vitamin D deficiency, encouraging physical activity and ensuring normal growth and pubertal progression are paramount to management of bone health in these patients. Despite the lack of evidence, there may be a place for bisphosphonate treatment, especially in the presence of symptomatic pathological fractures, but this requires close monitoring by clinicians with expertise in paediatric bone health. CONCLUSION: Chronic inflammation mediated by pro-inflammatory cytokines may have adverse effects on skeletal health in paediatric patients with IBD. The risk of vertebral fractures may be increased, even without exposure to glucocorticoid. Clinical monitoring of these patients requires careful attention to the various factors that impact on bone health.

Enteral nutrition in Crohn's disease: an underused therapy.

This paper reviews the literature on the history, efficacy, and putative mechanism of action of enteral nutrition for inflammatory bowel disease in both paediatric and adult patients. It also analyses the reasoning behind the low popularity of exclusive enteral nutrition in clinical practice despite the benefits and safety profile.

Novel Burkholderiales 23S rRNA genes identified in ileal biopsy samples from children: preliminary evidence that a subtype is associated with perianal Crohn's disease.

A novel family of Burkholderiales bacteria was identified in ileal biopsy specimens from children presenting with symptoms of inflammatory bowel disease. A molecular subtyping approach based on sequencing of a variable region of the bacteria's 23S rRNA genes identified three variants. Pilot analysis identified one variant to be significantly associated with perianal Crohn's disease.

Hypnotherapy for treatment of irritable bowel syndrome.

BACKGROUND: Irritable bowel syndrome (IBS) is a common functional gastrointestinal disorder of unknown aetiology. Current pharmacological treatments have limited value. Hypnotherapy has been reported to have beneficial effects for IBS symptoms. OBJECTIVES: To evaluate the efficacy of hypnotherapy for the treatment of irritable bowel syndrome. SEARCH STRATEGY: Published and unpublished randomised clinical trials and quasi-randomised clinical trials were identified through structured searches of MEDLINE (1966 to March 2006), EMBASE (1980 to March 2006), PsycINFO (1806 to March 2006), CINAHL (Cumulative Index to Nursing and Allied Health Literature, 1982 to March 2006), AMED (Allied and Complementary Medicine Database, 1985 to March 2006) and The Cochrane Central Register of Controlled trials. Conference proceedings from Digestive Disease Week (1980 to 2005) were also searched. SELECTION CRITERIA: Eligible studies included all randomised and quasi-randomised clinical studies comparing hypnotherapy for the treatment of irritable bowel syndrome with no treatment or another therapeutic intervention. DATA COLLECTION AND ANALYSIS: All studies were evaluated for eligibility for inclusion. Included studies were assessed for quality and data were extracted independently by four authors. The primary outcome measure of interest was the overall bowel symptom severity score which combines abdominal pain, diarrhoea or constipation and bloating. Secondary outcomes included abdominal pain, diarrhoea, constipation, bloating, quality of life, patient's overall assessment of well-being, psychological measures as per validated questionnaires, and adverse events. MAIN RESULTS: Four studies including a total of 147 patients met the inclusion criteria. Only one study compared hypnotherapy to an alternative therapy (psychotherapy and placebo pill), two studies compared hypnotherapy with waiting-list controls and the final study compared hypnotherapy to usual medical management. Data were not pooled for meta-analysis due to differences in outcome measures and study design. The therapeutic effect of hypnotherapy was found to be superior to that of a waiting list control or usual medical management, for abdominal pain and composite primary IBS symptoms, in the short term in patients who fail standard medical therapy. Harmful side-effects were not reported in any of the trials. However, the results of these studies should be interpreted with caution due to poor methodological quality and small size. AUTHORS' CONCLUSIONS: The quality of the included trials was inadequate to allow any conclusion about the efficacy of hypnotherapy for irritable bowel syndrome. More research with high quality trials is needed.

Bowel imbrication in the management of anorectal anomalies.

Four patients who had imbrication of their proximal rectum and distal sigmoid colon as part of the management of constipation following an anorectoplasty for an anorectal anomaly. Three children with an anorectal anomaly presented with constipation and marked dilation of the rectosigmoid portion of the large bowel; each had longitudinal imbrication of the dilated segment, via a left iliac fossa incision. The forth was born with a cloacal anomaly with associated colonic atresia. The small bowel was used to construct the anorectum following a redo anorectoplasty. Subsequently, the small bowel became ectatic, resulting in the patient developing persistent watery diarrhoea and severe perianal excoriation, which was managed with a 30 cm longitudinal imbrication of the distal bowel during an extensive laparotomy. All 4 have patients now have near normal bowel motions with minimal medication, after only a short hospital stay. Residual problematic dilatation of the rectosigmoid colon in patients with a high anorectal anomaly, in the presence of constipation, can be successfully managed by imbrication of the dilated segment, if carefully selected.

Effect of gluten-free diet and adherence on growth and diabetic control in diabetics with coeliac disease.

AIMS: To study the effect of gluten-free diet on growth and diabetic control of children with type 1 diabetes mellitus and coeliac disease. METHODS: Twenty one children (mean age 7.5 years, range 1.6-12.9) with type 1 diabetes, primarily initially identified on the basis of symptoms and consecutively diagnosed with coeliac disease by biopsy over a 10 year period, were matched by sex, age at onset, and duration of diabetes with two diabetic controls without coeliac disease. Weight, height, haemoglobin A1c, and insulin requirements were measured before and for 12 months after the diagnosis and treatment of coeliac disease. Dietary awareness and adherence were assessed by structured questionnaire. RESULTS: A gluten-free diet resulted in a significant increase in weight-for-age z scores at 12 months after diagnosis (mean increase in z score 0.33) and in BMI (mean increase in z score 0.32). Increases in height did not achieve statistical significance. Controls showed no significant changes in weight, height, or BMI over the same period. Insulin dosage at diagnosis was less in coeliacs than in controls (mean difference 0.16 units/kg/day), but was similar to controls once a gluten-free diet had been established. Questionnaires were obtained in 20 patients. There appeared to be a relation between dietary awareness/adherence and growth parameters, but the small number of patients with "poor/fair" dietary adherence prevented meaningful analysis of this group. CONCLUSION: Identification and dietary treatment of coeliac disease in children with diabetes improved growth and influenced diabetic control. Evaluation of the outcome of treatment of coeliac disease in diabetics should include assessments of gluten intake.

Chest physiotherapy, gastro-oesophageal reflux, and arousal in infants with cystic fibrosis.

BACKGROUND: Postural drainage chest physiotherapy in infants with cystic fibrosis (CF) exacerbates gastro-oesophageal reflux (GOR) and may contribute to a more rapid deterioration in lung function. AIMS: To compare standard postural drainage chest physiotherapy (SPT) and a modified physiotherapy regimen (MPT) without head-down tilt, with regard to GOR, arousal state, and cardiorespiratory function. METHODS: Twenty infants with CF underwent 30 hour oesophageal pH monitoring, during which four chest physiotherapy sessions were administered (day 1: MPT-SPT; day 2: SPT-MPT). Arousal state, heart rate, and oxygen saturation were documented for each of the physiotherapy positions (supine, prone, right lateral, and left lateral with (SPT) or without (MPT) 30 degrees head-down tilt). RESULTS: Significantly more reflux episodes occurred during SPT than during MPT, but there were no significant differences in median episode duration or fractional reflux time. During SPT, left lateral positioning was associated with fewer reflux episodes compared to other positions. During supine and prone positioning, more reflux episodes occurred during SPT than during MPT. Infants were significantly more likely to be awake or cry during SPT. There was a significant association between crying and reflux episodes for SPT. Non-nutritive sucking was associated with a significant reduction in reflux episodes during SPT. Oxygen saturation during SPT was significantly lower during crying and other waking, and non-nutritive sucking during SPT was associated with a significant increase in oxygen saturation. CONCLUSIONS: SPT is associated with GOR, distressed behaviour, and lower oxygen saturation.

Gastrointestinal transit and anorectal manometry in children with colonic substance P deficiency.

BACKGROUND AND AIMS: Severe intractable constipation in children may be associated with a reduction of substance P (SP)- containing fibers in colonic circular muscle. The aim of this study was to characterize gastrointestinal transit (GIT), anorectal manometry (ARM) and electromyographic (EMG) changes in these children. METHODS: Seromuscular laparoscopic biopsies of the colon were obtained from 35 children with severe constipation. Immunofluorescent staining for SP and vasoactive intestinal peptide (VIP) were then performed on these specimens. The cohort of patients studied included a SP-deficient group (SPD, n = 25) who had reduced numbers of SP-immunoreactive nerve fibers. The other group consisted of patients with normal staining for both SP and VIP (SPN, n = 10). Gastrointestinal transit studies (gastric emptying, orocecal and colonic transit) suitable for analysis were available for 17 patients (SPD, n = 9 and SPN, n = 8). The colon was divided into segments and radioactivity counts in each segment were expressed as a percentage of the total colonic count at each time point (6, 24, 32 and 48 h). The geometric center (GC), ARM, EMG, clinical and demographic data characteristics of both groups of patients were compared. RESULTS: There were no differences in demographic data, gastric emptying, orocecal transit or geometric center of transit in the colon between the two patient groups. The ARM and EMG studies suggested that the SPN group have a higher mean threshold volume of balloon distension required to initiate a rectoanal inhibitory reflex, and a higher incidence of anismus; however, this did not reach statistical significance. CONCLUSIONS: These data suggest a trend that the SPN patients have a greater problem with obstructive defecation and abnormal rectal sensation than those with SPD. We were unable to confirm any defect in colonic transit in the SPD patients compared with the SPN group.

Botulinum toxin for achalasia in children.

BACKGROUND: Injection of botulinum toxin (BTx) into the lower esophageal sphincter (LES) of adult patients with achalasia results in the effective relief of symptoms. The aim of the present study was to examine the effectiveness of BTx in pediatric patients suffering from achalasia. METHODS: Seven patients suffering from achalasia with or without prior treatment were treated with intrasphincteric injection of BTx. The median duration of follow up was 15 months. RESULTS: All seven patients improved. The median interval before recurrence of symptoms was 4 months (range 1-14 months). There was an inverse relationship between the pretreatment LES pressure and the duration of response (r=-0.6). The mean pretreatment LES pressure in the subgroup with a response greater than 6 months was 38+/-10 mmHg compared with 61+/-12 mmHg in the other four patients (P= 0.05). All seven patients required retreatment. CONCLUSION: Botulinum toxin is effective in relieving symptoms in pediatric patients suffering from achalasia, producing a sustained response beyond 6 months in 43% of patients.

Effects of the laryngeal mask airway on the lower oesophageal barrier pressure in children.

The effects of laryngeal mask airway (LMA) insertion and cuff inflation on lower oesophageal sphincter, gastric and barrier pressure, and the relationship of the LMA cuff pressure and volume on the change in the barrier pressure were studied in 20 children. Subjects were aged one to five years, undergoing eye examination under general anaesthesia. There was no significant change in barrier pressure after insertion and inflation of the LMA compared with baseline measures. The cuff pressure and volume were not related to the change in barrier pressure. Two patients had marked decreases (10 to 15 mmHg) in barrier pressure after the LMA insertion. These decreases in barrier pressure would be expected to increase the risk of gastro-oesophageal reflux. We conclude that, although LMA use had little effect on barrier pressure in most children, occasional children will have potentially clinically significant decreases in barrier pressure with use of the LMA.

School absenteeism after upper gastrointestinal endoscopy in children.

BACKGROUND AND AIMS: To examine the functional impact of upper gastrointestinal endoscopy as a day procedure, particularly in relation to subsequent school attendance. METHODS: Symptoms and morbidities were prospectively recorded from school-aged children during observation in hospital and for 3 days at home after endoscopy by using a structured questionnaire. Reasons for school absence were identified. RESULTS: Sixty children (31 boys, 29 girls) were enrolled in the study (mean age 10.6 +/- 2.8 years, range 6.1-16.2 years). Following the procedure, symptoms were reported at home in 68.3% (same day), 56.7% (day 1) and 20% (day 2).The commonest symptoms were sore throat, tiredness and dizziness. Twenty-nine children (48.3%) did not attend school on the day following the procedure but most (26 of 29) had returned to school by the second day. The main reason for their absence was residual physical discomfort related to the procedure (55.2%). CONCLUSIONS: Persisting physical discomfort and school absenteeism are common following upper gastrointestinal endoscopy in children.

Role of food protein intolerance in infants with persistent distress attributed to reflux esophagitis.

BACKGROUND: Distressed behavior is common in infants and is often attributed to gastroesophageal reflux (GER) or food protein intolerance. OBJECTIVE: To examine the effect of a hypoallergenic amino acid-based infant formula (AAF) on distressed behavior and GER symptoms in infants who failed to respond to extensively hydrolyzed formula and antireflux medications. STUDY DESIGN: Nineteen distressed infants (9 boys and 10 girls; median age, 5.0 months) with presumed GER underwent gastroscopy (n = 17) and esophageal 24-hour pH monitoring (n = 14). Double-blind placebo-controlled (DBPC) formula challenges of AAF versus previously besttolerated formula were conducted. RESULTS: Nine infants had histologic evidence of esophagitis, and 9 had inflammatory changes in the stomach and/or duodenum. Symptoms remitted in all infants within 2 weeks of the start of feeding with AAF. On DBPC challenge after a median period of 3 months of receiving AAF, 12 infants were intolerant to active formula (distress score, 287 vs 580 min/wk,P =. 01; symptom score, 23.1 vs 36.1, P =.03). Seven infants did not relapse and were considered tolerant (distress score, 470 vs 581, P =.77; symptom score, 29.5 vs 20.2; P =.89). CONCLUSION: Treatment with AAF may reduce distressed behavior and symptoms of GER in infants with food protein intolerance.

Effect of butyrate on paracellular permeability in rat distal colonic mucosa ex vivo.

BACKGROUND AND AIMS: The effects of butyrate on colonic epithelial barrier function are poorly understood. The aim of this study was to examine the short-term effects of butyrate on paracellular permeability of rat distal colonic epithelium. METHODS: Mucosa mounted in Ussing chambers was treated with butyrate (1-10 mmol/L) for 4 h. Transepithelial conductance, [51Cr]-EDTA flux, mucosal brush border hydrolase activity and epithelial kinetics, using proliferating cell nuclear antigen (PCNA) staining, were measured. RESULTS: On exposure to butyrate (10 mmol/L, but not 1 or 5 mmol/L), transepithelial conductance was 65 +/- 2% higher (mean +/- SEM; n = 8, P < 0.05, paired t-test) and the rate coefficient for [51Cr]-EDTA flux was 65 +/- 25% higher (P = 0.03) than those of control tissue. Histologically, the epithelium exhibited no signs of injury, but butyrate-treated tissue exhibited interstitial oedema consistent with water uptake in association with butyrate absorption. Butyrate caused a reduction in crypt column height to 30.6 +/- 1.6 cells from 33.4 +/- 1.8 cells in controls (n = 10, P < 0.03), but the number of cells per crypt column staining with PCNA was unchanged. Butyrate significantly reduced the mucosal activities of alkaline phosphatase by 40 +/- 16%, maltase by 54 +/- 12% and dipeptidyl peptidase IV by 41 +/- 14%. CONCLUSIONS: Acute exposure to butyrate increased paracellular permeability in rat distal colon. The mechanism involved may relate to the loss of differentiated surface epithelial cells, or as a physiological response to Na+-coupled butyrate uptake.

Modulation of distal colonic epithelial barrier function by dietary fibre in normal rats.

BACKGROUND: Dietary fibre influences the turnover and differentiation of the colonic epithelium, but its effects on barrier function are unknown. AIMS: To determine whether altering the type and amount of fibre in the diet affects paracellular permeability of intestinal epithelium, and to identify the mechanisms of action. METHODS: Rats were fed isoenergetic low fibre diets with or without supplements of wheat bran (10%) or methylcellulose (10%), for four weeks. Paracellular permeability was determined by measurement of conductance and 51Cr-EDTA flux across tissue mounted in Ussing chambers. Faecal short chain fatty acid (SCFA) concentrations were assessed by gas chromatography, epithelial kinetics stathmokinetically, and mucosal brush border hydrolase activities spectrophotometrically. RESULTS: Body weight was similar across the dietary groups. Conductance and 51Cr-EDTA flux were approximately 25% higher in animals fed no fibre, compared with those fed wheat bran or methylcellulose in the distal colon, but not in the caecum or jejunum. Histologically, there was no evidence of epithelial injury or erosion associated with any diet. The fibres exerted different spectra of effects on luminal SCFA concentrations and pH, and on mucosal indexes, but both bulked the faeces, were trophic to the epithelium, and stimulated expression of a marker of epithelial differentiation. CONCLUSIONS: Both a fermentable and a non-fermentable fibre reduce paracellular permeability specifically in the distal colon, possibly by promoting epithelial cell differentiation. The mechanisms by which the two fibres exert their effects are likely to be different.

Physiological and psychosocial contributors to malnutrition in children with cystic fibrosis: review.

Malnutrition was once thought to be an inevitable consequence of cystic fibrosis (CF). It is now considered preventable but still contributes considerable morbidity in children. Malnutrition is linked to poorer pulmonary function, reduced survival and quality of life. As the anticipated lifespan of children with CF continues to lengthen, the prevention of malnutrition attains greater importance. This review explores the complex organic and psychosocial factors implicated in the aetiology of malnutrition associated with CF.

Clinical significance of anismus in encopresis.

Treatments designed to relieve paradoxical contraction of the anal sphincters during defecation (anismus) have had limited success in children with encopresis. This has raised doubts as to the clinical relevance of this diagnosis in childhood as anorectal dysfunction. Our aim was to determine whether, in patients who had treatment-resistant encopresis, the presence of electromyographic anismus was associated with increased faecal retention. Sixty-eight children with soiling (mean age 8.7+/-2.06 years) were assessed by clinical examination, abdominal radiography and then with anorectal manometry. Patients with electromyographic anismus (n=32; 47%) had significantly increased radiographic rectal faecal retention and were significantly less likely to be able to defecate water-filled balloons. There were no significant differences in response to prior therapy, history of primary encopresis, behavioural adjustment or in sociodemographic data. Our results suggest that electromyographic anismus is associated with obstructed defecation and faecal retention.