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Key Points Provides an overview of coeliac disease in children and discusses the various oro-dental manifestations of coeliac disease. Emphasises the importance of general dental practitioners in early recognition and long-term monitoring of children with coeliac disease. Highlights a few scenarios for general dental practitioners where suspicion regarding coeliac disease needs to be raised. Coeliac disease (CD) is an immune-mediated systemic disorder caused by ingestion of gluten found in wheat, rye and barley. It affects around 1% of children, but 90% of cases are considered to remain undiagnosed. CD classically presents with gastrointestinal manifestations including diarrhoea, bloating, weight loss and abdominal pain, but extra-intestinal features (including oral and dental manifestations) are increasingly being reported. Dental and oral manifestations such as dental enamel defects, delayed eruption of teeth, recurrent aphthous ulcers are well-recognised manifestations of CD. In patients with yet undiagnosed CD, these can sometimes be the only presenting features. Dentists have regular contact with well children, and therefore the visit to the dentist is an opportunity to suspect CD. When CD is suspected, Dental practitioners can liaise with the general medical practitioner to organise screening for coeliac disease. Positive serology will prompt onward referral to a paediatric gastroenterologist to confirm the diagnosis. The recent European Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) guidelines revised in 2012 have streamlined the diagnostic pathway for faster diagnosis of CD. Management involves strict adherence to a gluten free diet, which should lead to resolution of symptoms, recovery of intestinal mucosa and prevention of long-term complications associated with it. This article aims to describe CD, inform of recent changes to the diagnostic pathway and highlight the dental manifestations of the condition to equip dental practitioners to aid early diagnosis and initiation of treatment for children with CD.

The joint European Society of Gastrointestinal Endoscopy and European Society of Paediatric Gastroenterology Hepatology and Nutrition guidelines on therapeutic indications of endoscopy recommend the use of esophagogastroduodenoscopy in the dilatation of oesophagus and upper gastrointestinal strictures and the use of ileocolonoscopy for dilatation of ileocolonic and colonic stenosis.1We conducted a retrospective observational study of gastrointestinal dilatations performed by Paediatric Gastroenterologists over a 10 yr period (1 January 2012 to 31 December 2022) in our centre. Prospective records of procedures were used to identify the patients and the electronic health records were reviewed.163 endoscopic dilatations were performed in 52 patients during this period. The procedures were either performed or supervised by Gastroenterologist with an interest in therapeutic endoscopy. The age group of the patients ranged between 3 months to 17 yrs with a mean of 12.3 years. Anatomical sites dilated were as follows: 92 oesophageal, 7 pyloric/antral, 16 duodenal, 9 ileal, 2 colonic, 1 ileostomy, 3 rectal stumps and 32 rectal.Hagar dilators with or without balloon dilatation were used in 15 of 32 rectal strictures. Through the scope balloon dilatation was used for all other strictures 130 of 163. In 40 procedures there were additional interventions performed alongside dilatation which included 2 endoknife cut, 1 argon laser and hemostatic clip application, 26 injection of Triamcinolone acetonide, 9 Mitomycin c spray and 4 injection of botulinum toxin.Underlying aetiologies were recorded as follows: 34 congenital stricture/web, 46 Crohn’s disease, 11 anastomotic stricture, 1 radiotherapy-induced stricture, 41 strictures secondary to gastroesophageal reflux disease, and unknown aetiology in 7. Inflamed mucosa at the stricture site was noted in 63 procedures. 158 of these procedures were performed by the Gastroenterologist independently and 5 procedures were done jointly with a Paediatric Surgeon.Children were discharged the same day for 124 procedures. The rest were done during an inpatient stay. Four procedures were associated with serious complications. Two had perforation requiring surgery and intensive care admission; both children were 2 yrs or younger. One patient became unwell with the intraabdominal collection but no perforation. One patient required 24 hrs of observations for persistent rectal bleeding but did not require further intervention. One patient was readmitted within 8 days of discharge.In conclusion, endoscopic dilatation when performed by a gastroenterologist with therapeutic interest appears to be a safe alternative to surgical management of strictures. The risk of complications is small and the majority can be performed as a day case. Procedures in children under 2 yrs of age carry the most risk of bowel perforation. Tringali A, Thomson M, et al. Pediatric gastrointestinal endoscopy: european society of gastro- intestinal endoscopy (ESGE) and European society for paediatric gastroenterology hepatology and nutrition (ESPGHAN) guideline executive summary. Endoscopy 2017;49:83–91.

We present a case history where a major surgery was prevented by successful therapeutic endoscopic interventions in an child who presented with gastric outlet obstruction.A 15yr old boy presented to Hospital A with a two-week history of acute onset progressive epigastric pain, nausea, vomiting and 15% weight loss. He was previously well and trained at a high level in martial arts but denied any significant injuries.Abstract OC73 Figure 1CT scan abdomen[Figure omitted. See PDF]His blood tests showed liver enzymes raised >3 times the upper limit of the normal, normal synthetic function of the liver and mildly raised amylase. His ultrasound scan of the abdomen showed dilated common bile duct and extrinsic compression of the pancreatic head by jejunum which was confirmed by MRCP. The CT scan of the abdomen suggested invagination of gastric pylorus and antrum into jejunum and external compression of CBD at the ampulla. He was transferred to surgical team in our centre and following a joint review was planned for Gastroscopy followed by Laparotomy with Paediatric Gastroenterologist.Abstract OC73 Figure 2Gastroscopy showed a giant cauliflowler pedunculated polyp atleast 120mm in diameter. This was retrieved into the stomach with alligator forceps. Peicemeal hot snare polypectomy with electrocautery performed and retrieved twelve 20–25mm pieces. Haemostais was achieved and additional hemospray used. Histology showed mucosa with interdigitating smooth muscle bundles characteristic of Peutz-Jegher’s polyp. He was admitted in 6 weeks and remaining 8omm poplypectomy performed with endoloop and hot snare polypectomy successfully.He had no mucocutaneous pigmentation and Video capsule endoscopy did not show any other small bowel polyps. This was a case of sporadic hamartomatous polyp.We report a successful therapeutic endoscopic treatment of a giant hamartomous polyp presenting with gastric outlet and biliary obstruction.

Background Inflammatory bowel disease (IBD) is understood to result from the interaction of genetic, immunological and environmental factors. There has been a marked increase in the incidence of IBD over the last 25 years, suggesting environmental factors are important. A previous study found a higher incidence of Coeliac disease in the least deprived socioeconomic groups.1 The objective of this study was to investigate the relationship between IBD and socioeconomic position. Methodology Bristol Children’s Hospital is the single regional centre where all children with suspected IBD from the South-west of England are referred. Data was collected prospectively on all children diagnosed between May 2004–March 2013. Socioeconomic status was determined by quintile rank of Index of multiple deprivation score (IMD-10 score) based on postcode at diagnosis. This has been shown to provide a nationally consistent measure of how deprived an area is. Population data was obtained from the 2011 Census. Data was analysed using Pearson Chi Squared test. Children with a postcode outside of the City of Bristol were excluded from the analysis. Results 384 children aged 0–17 years were diagnosed with IBD over the study period of which 50 had a postcode of residence within the City of Bristol. The incidence of IBS was higher in the three lower socio-economic classes compared to the two highest socio-economic classes (see Figure 1). However, the difference in incidence between the socio-economic classes was not statistically significant. Abstract G81(P) Figure 1 Conclusion Our data suggests a higher incidence of diagnosed IBD in children from lower socioeconomic classes which may favour an environmental aetiology. However this did not reach statistical significance, possibly due to small numbers. A larger study is warranted. Reference Whitburn and Sandhu. Coeliac disease and relationship to socio-economic status. Arch Dis Child 2013;98:A86

Background In 5–15% of children diagnosed with inflammatory bowel disease (IBD), the histological picture at diagnosis doesn’t fit in with either ulcerative colitis (UC) or Crohn's disease (CD) and is classified as unclassified-IBD (IBDU) or indeterminate colitis.1 The aim of this prospective study is to determine whether IBDU evolves into UC or CD. Methods Prospective data has been collected on all the newly diagnosed children with IBD at the only regional paediatric gastroenterology centre covering southwest of England. All patients suspected of IBD had upper and lower gastrointestinal endoscopy and MRE scan or barium meal as recommended by BSPGHAN.2 Patients diagnosed with IBDU during 2004–2011 were included in the study and followed up for a minimum of 2 years (range 2–9 years). The patient notes were reviewed in 2013 and any changes in diagnosis recorded. Results 333 children were diagnosed with IBD between 2004–2011: 193 (58%) had CD, 115 (34.5%) UC and 25 (7.5%) IBDU. Age (mean) at diagnosis: 10.2 years (IBDU), 11.5 years (CD) and 11.6 years (UC). 7/26 (27%) IBDU had pan-colitis and 19/26 (63%) had patchy or left-sided colitis on lower gastrointestinal endoscopy. After 2 to 9 years, IBDU evolved into CD in 5 patients (22.8%), UC in 3 (13.6%) and remained IBDU in 14 patients (63.6%). Latest data was unavailable for 3 (11.6%) because of transfer to distant adult services. ANCA was positive in 3 out of 4 patients whose diagnosis was revised as CD. Conclusion This large prospective study has documented that over 2–9 years, 22.8% IBDU evolved into CD, 13.6% into UC and 63.6% remained IBDU. IBDU patients tended to be younger at diagnosis. Positive ANCA was not an useful predictive marker. This has implications for management of IBDU patients especially where surgical treatment is considered. References De Bie CL, et al. (2012). J Pediatr Gastroenterol Nutr. 54(3):374-80 Sandhu BK, et al. (2010). J Pediatr Gastroenterol Nutr. 50:S1-S13

AimTo investigate the availability of IBD related laboratory investigations in NHS laboratories in England and to discern whether there are regional variations.Subjects and MethodsA structured telephone survey was conducted in July 2016 by a single interviewer by contacting the clinical Laboratories in Acute NHS trusts across England with paediatric services. The available online handbooks for each lab were also accessed and where appropriate scientists were unavailable, the survey questions were sent by email. The data was collected on a database and analysed using Microsoft excel. No ethical approval was required for this study.ResultsResponse was obtained from 136 out of 139 laboratories (97.8%). 1) Inflammatory markers (other than CRP): ESR is widely available at 98%, followed by Plasma viscosity (PV) at 71% and Orosomucoid (ORM) at 48%. Regional variations are significant with East of England and London having least access to PV and ORM2) Faecal calprotectin was available in 89% of labs although only 51% offer in house testing. 84% allow any clinician to request the test whereas the rest allow only a few clinician groups to request3) ANCA can be tested in 94% of labs but ASCA is available only in 29%4) TPMT activity was available in 96% of labs with only 29% testing this on site5) 6-Thioguanine metabolites was offered only by 58% of labs with 89% outsourcing it. This was most widely accessible in the south east.6) Infliximab serology is offered in only 61% of labs with only 14% able to test this on site. This is least accessible in the East Midlands. Summary and conclusionThere is extensive regional heterogeneity in the availability of laboratory investigations for PIBD in England with low levels of on site testing. More research is needed to confirm the utility of the laboratory investigations in PIBD and establish their use. National guidelines should include standards for the investigations required and provide information on cost effectiveness to allow Paediatric units in all regions to access the investigations promptly to maintain equal standards of health care for PIBD accross England.

Aligned with recommendations from the 2021 paediatric gastroenterology, hepatology and nutrition national census,1 our tertiary paediatric gastroenterology department implemented an out-of-hours (OOH) service in January 2022. This included provision for 24/7 specialist telephone advice, consultant-led weekend ward rounds and OOH emergency endoscopy.We sought to evaluate the OOH service one year post-introduction. We collected admissions data and endoscopy logs from a 5-year period (1 January 2017- 31 December 2021) representing our service pre-OOH service and compared this with the one-year period (1 January 2022–31 December 2022) representing our service after introduction of the OOH service. We also sent an online survey to general paediatricians, subspecialty paediatricians and paediatric surgeons in our tertiary centre and our ten regional network hospitals for feedback on the OOH service.From 2017–2021, there was an average of 124 admissions/year, compared with 170 in 2022. Those patients admitted electively for procedures and infusions were excluded. Median length of hospital stay for both epochs was 7 days. There was a significant increase in weekend discharges (12.9% vs 20%, p<0.05). There was no significant difference in weekend admissions (14.3% vs 15.9%, p=0.6).In 2022 alone, 25 patients underwent endoscopy out-of-hours (figure 1). 17/25 (68%) occurred during the weekend, the remainder during weekday evenings. The most common indication for endoscopy was inflammatory bowel disease assessment (n=14, 56%). In comparison, 20 patients underwent endoscopy out-of-hours in the five years preceding this.We received 37 survey responses, of which 24 (65%) came from general paediatricians, 11 (30%) from subspecialty paediatricians and 2 (5%) from paediatric surgeons. 10 respondents (27%) contacted the OOH service for specialist advice in 2022. All found it easier/much easier to access timely specialist advice, compared to before. In terms of qualitative feedback, respondents were generally complimentary of the OOH service, and found its availability reassuring. In terms of constructive feedback, one respondent recommended better advertisement of the service; another respondent noted an increased challenge with coordinating the timing of different weekend ward rounds with the available trainees.The introduction of our OOH service has improved patient care. Increased weekend discharges reflect the impact of senior decision-making during the weekend. There is greater access to OOH endoscopy, reducing treatment delays. Professional feedback is also highly positive. There has been an increased OOH workload, and therefore an OOH service needs to be carefully job-planned. We hope our experience will be helpful to other specialist centres planning to introduce an OOH service.Abstract OC97 Figure 1OOH endoscopy procedures per year[Figure omitted. See PDF] BSPGHAN and RCPCH Paediatric Gastroenterology, Hepatology and Nutrition UK National Census 2021 – report. Available at https://www.rcpch.ac.uk/resources/pghan-national-census-2021

IntroductionThe ESPGHAN 2020 guidelines have streamlined the diagnostic pathways for paediatric coeliac disease [CD] and have recommended that all children with TGA-IgA ≥10xULN can be safely diagnosed via the no-biopsy pathway [NBP].1 During the COVID-19 pandemic there has been greater emphasis on the use of the NBP for lower TGA-IgA titres than that recommended by the ESPGHAN for diagnosing CD across the world including the UK.2 This practice has been supported by papers published from tertiary gastroenterology centres in Italy, India and England where a cut-off value of TGA ≥5xULN have been deemed to be safe for NBP.3–5 An English study published in 2017 have highlighted the variation in the TGA-IgA titres used across the centres in the UK including southwest England where the titre varied from 4 IU/ml to 30 IU/ml.6AimsTo establish whether the lower TGA-IgA ≥5xULN can be safely used for diagnosing CD via NBP across various TGA-IgA titres used in the non-specialist regional centres in the Southwest England [SWE].MethodsAnonymous data between 2013 and 2020 were obtained from 5 regional centres across the SWE for the patients with TGA-IgA between ≥5xULN and <10xULN at the time of referral and as to whether they were finally diagnosed with CD following upper GI endoscopy and histology. Data was also collected on the age of the patients at first TGA-IgA titres, sex, symptoms and other high risk factors e.g. Type-1 diabetes mellitus, first degree relatives.ResultsFive centres participated in the study. A total of 8 TGA-IgA titres have been used with values varying between 4 IU/ml to 30 CU/ml. 67 patients were identified and 48 underwent UGIE and biopsies, 45/48 (93.75%) got confirmation for CD on histology. The 3 who did not have confirmatory histology had repeat TGA-IgA <5xULN at the time of endoscopy. Out of rest 19, 13 were diagnosed via NBP and rest 6 did not undergo a formal diagnostic process to confirm or rule out CD. There were 44 females and 23 males; mean age was 10.1 years [range 2 – 17 years]. Out of 67 children, 5 had family history, 4 had type 1 diabetes, 1 each had auto-immune condition and Down’s syndrome, rest 8 had no documented indication for undergoing a screening for CD. Figure 1 shows the breakdown for the 5 centres and their conversion for diagnosed CD.ConclusionsThis 5 centre study shows 93.75% correlation of TGA-IgA 5–10xULN at referral to final diagnosis of CD despite 8 variations in assay methods. This appears to improve to 100% when repeated at time of endoscopy. 19.4% of this group were given a diagnosis of CD via NBP based on clinician’s decision. There is need for similar collaborative studies through the BSPGHAN PeGHAN network to firmly establish similar findings for various TGA-IgA titres used across the UK.Abstract OC18 Figure 1References Husby, et al. J Pediatr Gastroenterol Nutr. 2020;70(1):141–156. Ruan, et al. Gastroenterology. 2020;159(4):1547–1550. Bhattacharya, et al. Indian J Gastroenterol. 2014;33(4):350–4. Trovato, et al. Eur J Gastroenterol Hepatol. 2020;32(12):1523–1526. Paul, et al. Arch Dis Child. 2022;107(8):747–751. Paul, et al. Arch Dis Child. 2017;102(10):942–946.

BackgroundESPGHAN 2012 guidelines on coeliac disease (CD) recommend that symptomatic children with anti-tissue-transglutaminase (anti-tTG) >10 x upper-limit-of-normal (ULN), positive EMA and HLA-DQ2/8, can be diagnosed without biopsy. Serological diagnosis is not appropriate for certain groups of patients.AimsGain understanding of awareness and use of ESPGHAN guidelines for diagnosing paediatric CD among general paediatriciansMethodsTelephone/email survey was conducted among general paediatric consultants (n»140) across Southwest England. Survey included 8-questions incorporating 3 main themes: when nonbiopsy diagnoses can be made, HLADQ2/8 genotyping should be requested and whether asymptomatic children from high-risk groups with anti-tTG >10xULN can be diagnosed serologically.Results85/140 (61%) responses obtained. 83% of paediatricians were unable to state all conditions required for non-biopsy diagnosis. None could describe all situations where HLA-DQ2/8 genotyping should be requested. 33% of paediatricians responded that asymptomatic children with anti-tTG >10xULN can be diagnosed with CD without a biopsy while 24% said they were unsure or would seek advice.Summary and ConclusionsSurvey highlighted need for greater in-depth awareness of non-biopsy pathway and situations where HLA-DQ2/8 genotyping is indicated. There is possible misinterpretation regarding the ESPGHAN guidelines as 1/3rd of paediatricians considered non-biopsy pathway is applicable for asymptomatic children. Adequate knowledge of the ESPGHAN guidelines for CD is required among paediatricians. A userfriendly Apps is planned to improve the diagnostic process.

BackgroundIn neonatal units, increased output in nappies is recorded as urine output unless semi-formed or formed stools are noted. Watery stools are likely be recorded as increased urine output prompting renal line investigations delaying the diagnosis of congenital diarrhoeas.AimWe present a case of congenital watery diarrhoea to highlight the diagnostic and treatment approaches in a neonate.Subjects and MethodsReviewed the presentation and progress of a neonate admitted in NICU with abdominal distension and significant metabolic and electrolyte disturbance.ResultsThe neonate had diagnostic laparotomy for intestinal obstruction, he was suspected to have possible necrotising enterocolitis. By day 9 of life, he developed severe hyponatraemia, hypochloraemia and metabolic alkalosis and was identified to have high urine output. Had extensive renal investigations for polyuria which was unremarkable. With faltering of growth and need for TPN, gastrointestinal loss was suspected and profuse watery diarrhoea became apparent. Hypochloraemic, Hyponatraemic metabolic alkalosis with high stool chloride confirmed the diagnosis of congenital chloride diarrhoea. Treatment will involve (i) life-long salt substitution; (ii) management of acute dehydration and hypokalaemia during gastroenteritis or other infections; and (iii) recognition and treatment of other manifestations of the disease, such as intestinal inflammation, renal impairment and male sub fertility.Summary and conclusionCongenital chloride diarrhoea is a rare autosomal recessive disease characterised by life-long watery diarrhoea of prenatal onset with high faecal chloride concentration. The diagnosis may easily be missed unless there is a high index of suspicion in a neonate with increased stool or presumed urine output.