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Inflammatory bowel disease (IBD) should be considered in any child with a persistently altered bowel habit. Growth failure may be a consequence and there may also be extra-intestinal manifestations. Oesophago-gastroduodenoscopy and colonoscopy and conventional histopathology are the diagnostic tools of choice in IBD. The identification and management of children with IBD in resource-poor settings is difficult and there are few data on its prevalence in low- and middle-income countries. The main challenges are a lack of resources and infrastructure including trained personnel in settings where there are other priorities for maintaining the health and wellbeing of children. The identification and management of children with inflammatory bowel conditions often depends on the enthusiasm, skill and commitment of a few dedicated individuals. Abbreviations: ADA: Adalimumab; CD: Crohn disease; ECCO: European Crohn's and Colitis Organisation; EEN: exclusive enteral nutrition; ESPGHAN: European Society for Paediatric Gastroenterology Hepatology and Nutrition; FMT: faecal microbiota transplantation; GDP: gross domestic product; HIC: high-income countries; IBD: inflammatory bowel disease; IBDU: inflammatory bowel disease unclassified; IC: ileocolonoscopy; IFX: infliximab; IPAA: ileal pouch anal anastomosis; LMIC: low- and middle-income countries; MH: mucosal healing; OGD: oesophago-gastroduodenoscopy; PCDAI: Paediatric Crohn's Disease Activity Index; PIBD: paediatric inflammatory bowel disease; PUCAI: Paediatric Ulcerative Colitis Activity Index; UC: ulcerative colitis; UGIT: upper gastrointestinal tract; VEO-IBD: very early-onset IBD; WLE: white light endoscopy; 5-ASA: 5 aminosalicylic acid; 6-MP: 6-mercaptopurine.

Background: this study compared the efficacy of an elemental formula (EF) to a polymeric formula (PF) in inducing remission for pediatric Crohn's disease (CD). Methods: newly diagnosed CD children were randomized to EF or PF for 6 weeks. Change in the Pediatric Crohn's Disease Activity Index (PCDAI), fecal calprotectin, and plasma fatty acids were measured at 0 and 6 weeks. Patients were followed up for 2 years. Time and treatment choice for first relapse were documented. Results: thirty-four children completed the study; EF: 15 (7 M, 8 F), PF: 19 (13 M, 6 F). The mean age was (years) EF: 12.6, PF: 11.7. Ninety-three percent of children (14/15) achieved remission in the EF group and 79% (15/19) in the PF group. One-third of patients maintained remission for 2 years. Mean time to relapse (days); EF: 183 (63-286), PF: 162 (53-301). Most children who relapsed used feed as a treatment for that relapse (EF: 9/10 and PF: 8/13). With PF, an increase of eicosapentanoic acid (EPA) and alpha linolenic acid was found with a reciprocal decrease in arachidonic acid (AA). With EF, AA and EPA levels were reduced with a significant decrease in docosahexaenoic acid. Fecal calprotectin measurements decreased significantly but did not normalize at the end of week 6. Conclusions: there was no significant difference between EF and PF in inducing remission. One-third of children maintained remission. Changes in plasma polyunsaturated fatty acid status were subtle and may be relevant; however, further evaluation is recommended

To describe the indications and practice of percutaneous endoscopic gastrostomy (PEG) device insertion for nutritional support and to ascertain procedure-related complications and outcome.: Observational study with prospectively collected data on children who underwent PEG-related procedures (PEG insertion, removal, or change to low profile button devices [LPBD]) for a 5-year period (2002-2006) at our centre.: Six hundred one PEG-related procedures were performed during the study period (384 insertions, 165 conversions to LPBD, 49 permanent PEG removals, and 3 PEG reinsertions). The main indications for PEG insertion were feeding difficulties associated with neurodisability (160/384; 41.6%) and congenital heart disease (CHD 115/384; 30%). Children with CHD required feeding nutritional support at an earlier age than children with neurodisability regardless of underlying cardiac condition. The age of PEG insertion was significantly different between the 2 main groups (neurodisability 3.56 years vs CHD 0.39 years; P0.001 [t test]). Fifty-nine patients were discharged on the same day as PEG insertion without complications (day case gastrostomy). The median time between PEG insertion to LPBD conversion was 0.83 year (0.12-3.86). Twenty-four of the 49 children having permanent PEG removal had CHD.: Neurodisabilty and CHD were the main indications for PEG insertion, those with CHD requiring feeding support at an earlier age. Although CHD was an indication for PEG insertion in 30% of instances, a greater percentage of PEG removals (50%) were seen in this group indicating resolution of feeding difficulties.

IntroductionWhile the usefulness of percutaneous endoscopic gastrostomy is clearly established in the nutritional support of children with neurodisability, the role in substituting for prolonged nasogastric feeding in children with congenitally malformed hearts is a relatively recent development. There are no previously published experiences of the perceptions of parents or those providing care following the insertion of percutaneous endoscopic gastrostomy in such children.MethodsDescriptive qualitative survey of parental perceptions using a semi-structured questionnaire.ResultsWe obtained completed 27 point semi-structured questionnaires from 38 providers of care for children with congenitally malformed hearts. Time taken to feed their children reduced significantly after the percutaneous endoscopic gastrostomy, from 30 to 60 minutes previously to 15 minutes subsequently. The frequency of feeding also reduced significantly, from 6 times a day to 4 to 5 times a day. Those providing care perceived significant reductions in pre-procedural symptoms, the ease of administering medications, and noted an enhanced level of happiness in their children. Of those providing care, 97% were highly satisfied with the procedure, with 15 parents (40%) wishing that the operation was done earlier, while the remainder considered it had been done at the appropriate time.ConclusionsThose caring for children with congenitally malformed hearts perceive significant improvements in the symptoms, wellbeing, and ease of administering medication for their children after percutaneous endoscopic gastrostomy. Of the group, 97% regarded the procedure as the appropriate means of assisting nutritional support.

Adequate nutrition is crucial to the management of children and infants with cardiac disease. Difficulties with feeding are extremely common, and maintaining an adequate caloric intake, in order to achieve sustained growth, is often not possible without nutritional support. We retrospectively reviewed our experience between 1995 and 1999 in treating 37 children with cardiac disease who underwent percutaneous endoscopic construction of a gastrostomy to augment nutritional needs. We stratified the patients into those with cyanotic heart disease, when saturations of oxygen were less than 95%; those with non-cyanotic heart disease with saturations greater than 95%, and those with minor cardiac disease associated with a systemic disorder. Each group was compared to control children matched for age, sex, and diagnosis. We evaluated the variation in standard deviation score for body weight over a median period of follow-up of 295 days. Improvements in the standard deviation score for body weight occurred in each of the groups, whereas children in the control groups demonstrated a decrease in standard deviation score for body weight. The median change of the score for body weight was significantly higher in patients managed with gastrostomy compared to controls. We conclude that supplementation using a gastrostomy tube allows the safe delivery of the caloric intake needed to support malnourished children with cardiac disease.

The management of children with intestinal failure is a rewarding but resource intensive process. There is however variability in practice and outcome for patients, despite the basic principles of care and measures of success being well defined. The importance of multidisciplinary working is paramount and there is an urgent need to obtain collaboration between paediatric surgical and medical gastroenterological colleagues and an obligation of commissioners to see that there is recognition and implementation of ideal practice as an essential element in improving the outlook for children with intestinal failure in the United Kingdom.

Background and aim The effect of vitamin D supplementation on immune disorders has been a topical research focus. The aim of this systematic review was to examine the current evidence of the effect of vitamin D supplementation as a therapy for colitis. Methods The following databases were searched: MEDLINE, Pubmed, Scopus, Web of Knowledge, Cinicaltrials.gov and the Cochrane Central Register of Controlled Trials using the terms ‘inflammatory bowel disease’ ‘Crohn's disease’ ‘ulcerative colitis’ ‘colitis’ [and] ‘vitamin D’. Both human and animal studies published in English language were examined. The reference lists of included studies and review articles were manually searched for any relevant studies. Results Four studies were included in this systematic review. All reported an improvement in disease activity with vitamin D supplementation. The only high quality human study reported a non-significant reduction of relapse rate for Crohn's disease. No major adverse effects of vitamin D supplementation were reported. Conclusions Although there is some evidence that supplemental vitamin D, as an adjunctive treatment, may help in controlling colitis, this evidence is not enough to justify using vitamin D in treating inflammatory bowel disease (IBD). Large high quality placebo-controlled randomised controlled trials are needed to explore a possible benefit of using vitamin D in treating IBD.

Recurrent respiratory problems are common in children with severe neurodisability, and respiratory deterioration is a leading cause of premature death in this group. Although the etiology is multifactorial, recurrent pulmonary aspiration is thought to play a significant role. Gastroesophageal reflux is known to be common, as is oral-motor discoordination. Differentiating direct aspiration of food and saliva and gastric reflux aspiration is difficult and presents a challenge in managing patients and assessing their suitability for surgical antireflux procedures. This is particularly the case when children present with predominantly respiratory symptoms, where there may be direct aspiration, reflux aspiration, neither, or both. A clinical biomarker to identify and quantify reflux aspiration would therefore be useful in surgical assessment and may also be applicable as an outcome measure for clinical trials of antireflux surgery. In this review, we discuss the evidence base behind existing and potentially novel biomarkers of aspiration in bronchoalveolar lavage fluid. We highlight the limitations of the lipid-laden macrophage index, particularly with regard to its specificity and interrater/intrarater reliability. We discuss the laboratory methods available to measure promising new biomarkers (pepsin and bile acids) and highlight their potential advantages and disadvantages. Finally, to understand how aspiration causes clinical signs and symptoms in our patients, we need to study the effect of aspirated substances on the lung, and here we review the available in vivo and in vitro literature.

The relationship between percutaneous endoscopic gastrostomy (PEG) insertion and gastro-oesophageal reflux disease (GERD) is widely disputed in the current literature. The aim of this systematic review is to examine the available evidence documenting the association between PEG and GERD.The following databases were searched: MEDLINE (1950 to week 2, January 2011), PubMed, ISI Web of Knowledge (1898 to week 2, January, 2011), EMBASE (1980 to week 2, January 2011) and The Cochrane Central Register of Controlled Trials (CENTRAL) using the terms "gastroesophageal reflux", "gastroesophageal disease", "GERD", "GERD", "GER", "GER" and "percutaneous endoscopic gastrostomy", "PEG", "gastrostomy". In addition, the reference lists of all included studies were reviewed for relevant citations. Studies examining children pre and post insertion of PEG for GERD and written in English language were included. Data extraction was performed by two authors, and the methodology and statistical analysis of each study were assessed.Eight studies were included in this systematic review. Two reported increased incidence of GERD after PEG. However, neither was of high methodological quality. The remaining six reported no change or decreased GERD. Nonetheless, few demonstrated rigorous methodology.The current evidence examining the effect of PEG insertion on GERD has been inconsistent and is not of high quality and therefore is unconvincing, preventing a definitive conclusion. Overall, the available literature on this topic does not demonstrate a causal effect of PEG insertion on GERD.

We describe in this report what we believe to be the first report of a rare presentation of a very rare tumor, especially in this age group. We highlight the importance of early consideration of malignancy as a cause of chylous ascites in infancy and we discuss different causes of chylous ascites.

The identification and cloning of the cystic fibrosis gene has broadened the scientific research base, with particular opportunities for biochemical and physiologic investigation of the basic defect. Gene therapy is an exciting prospect, and clinical research of the gastrointestinal manifestations of cystic fibrosis provide further understanding. Imaging, surgical, and therapeutic advances continue to benefit the child with pancreatic disease.

BackgroundChildren with severe neurodisability (ND) commonly suffer from chronic respiratory symptoms that impact greatly on quality of life, and lead to recurrent hospital admissions. This morbidity (and its causes) is poorly described, despite being well recognised by paediatricians. In this study, we characterised respiratory symptoms in children with ND at times of stability and deterioration. We also assessed the relationship between respiratory symptoms, lower airway inflammatory markers and levels of infection/colonisation.MethodsND children were recruited upon admission for elective surgery (Elective-ND [n = 16]), or acutely upon admission to Intensive Care (PICU-ND [n = 19]), and compared to healthy control children [n = 12]. Parents completed a validated respiratory symptom questionnaire in which symptoms associated with activity were removed (total maximal score of 108). Bronchoalveolar lavage (BAL) was collected, and BAL neutrophil counts, IL-8 and TGFβ-1 levels measured. BAL microbial analysis was performed using a 16S/18S rRNA gene based assay and Pseudomonas aeruginosa PCR.ResultsAll ND children had high levels of respiratory symptoms (median [IQR] symptom score PICU-ND, 55[38-64]; Elective-ND, 26[7-45]; Control, 4[0-7]: p20 invariably had BAL neutrophilia. Elective patients with 16S/18S microbial rDNA positive BAL had higher neutrophil counts (positive, 33[18-70]%; negative, 8[4-38]%: pConclusionsChildren with severe ND often have high levels of chronic respiratory symptoms, which may relate to lower airway inflammation. Bacterial airway colonisation, particularly with oral commensals, may play a role in both symptom generation and inflammation.

Objectives: We observed a cohort of children presenting with rectal bleeding that were identified as having Enterobius vermicularis at colonoscopy and questioned the reliability of conventional diagnostic methods of identifying E. vermicularis. Patients and Methods: The study was retrospective in nature and subjects were investigated by colonoscopy between May 1997 and December 1999. Patients were identified as having E. vermicularis infestation by direct visualisation of the adult worms at colonoscopy. Patients were treated with mebendazole and a record of their clinical response documented. Results: A total of 180 colonoscopic examinations were performed during the study period. E. vermicularis was identified macroscopically in 31 cases (17.2%). The symptom profile of patients with E. vermicularis were abdominal pain, 19 of 26 (73%); rectal bleeding, 16 of 26 (62%); chronic diarrhoea, 13 of 26 (50%) and weight loss, 11 of 26 (42%). Ova cysts and parasites were identified in none of the saline swabs analysed in 20 patients. Sellotape testing was performed in only 4 patients and was negative in all. Of the 26 children, 21 (81 %) demonstrated histopathological features of nonspecific colitis. There was clinical resolution of symptoms in 19 of 23 patients. Conclusions: We suggest that in patients with symptoms suggestive of inflammatory bowel disease, E. vermicularis infestation must be excluded as a common cause of nonspecific colitis. We also suggest that diagnostic tests such as saline swabs and Sellotape testing may be lacking in sensitivity.

Tufting enteropathy (intestinal epithelial dysplasia), a rare congenital enteropathy, presents in the first few months of life with chronic watery diarrhoea and impaired growth. The molecular basis for this condition is not known. We report our experience with a case of tufting enteropathy that developed an unusual skeletal dysplasia with an abnormal blood picture. After extensive investigations including repeated gastrointestinal endoscopies and biopsies, the diagnosis of tufting enteropathy was made. During the third year of her life, the patient’s height was static. A full skeletal survey was performed and demonstrated features of generalised skeletal dysplasia, some of them consistent with those of parastremmatic dwarfism. At the age of five years, she developed Coomb’s positive haemolytic anaemia and thrombocytopenia with a negative auto-antibody screen including anti-enterocyte antibodies. There might be generalised matrix (including cartilage matrix protein), basement membrane abnormalities or both. A secondary protein leak might occur in the intestine with autosensitisation and development of autoimmune phenomena. More molecular research is needed to identify a possible link.

Pleural effusions commonly complicate pancreatitis and are usually reactive. Massive pleural effusions are a rare but recognised complication of pancreatitis, the management of which may require surgical intervention. Bishop et al. [1] reported massive pleural effusion secondary to pancreaticopleural fistula in a 4-year-old girl who, following failure of medical management, underwent surgery. Namazi and Mowla [2] also reported a patient with massive pleural effusion secondary to pancreatitis, who underwent drainage of a pancreatic cyst following failure of conservative management. Medical management of massive pleural effusion secondary to pancreatitis is reported to be successful in approximately 40% of adult cases [3]. We report successful resolution following medical management in a 12-year-old girl who presented with a massive right pleural effusion secondary to chronic pancreatitis. A 12-yearold girl presented with poor weight gain, and abdominal and retrosternal chest pain. She underwent a barium meal and an upper and lower gastrointestinal endoscopy. Apart from Enterobius vermicularis infestation resulting in a mild nonspecific proctitis, these investigations were normal. Empirical treatment with Gaviscon and mebendazole resulted in weight gain and symptom resolution. Six months later, she presented with a 2-month history of progressive shortness of breath and poor exercise tolerance. She was noted to be pyrexial and complained of right upper quadrant abdominal and chest pain. Clinical examination revealed no signs of respiratory distress, normal pulse oximetry and respiratory rate. However, there were absent breath sounds over the right lung and a chest X-ray film demonstrated a right pleural effusion (Fig. 1). An ultrasound scan and a chest CT scan confirmed a hyper-echoic fluid collection, total collapse of the right lung with no evidence of septation, mediastinal mass or lymphadenopathy. A Mantoux test was negative. Analysis of pleural fluid obtained after drainage revealed a white cell count of 1.05·10/l, raised amylase of 24777 U/l with a protein content of 32 g/l. Simultaneous serum analysis revealed a serum albumin of 31 g/l and an amylase of 304 U/l. The patient was treated conservatively with chest tube drainage, intravenous antibiotics, and intravenous octreotide (1 lg/kg/h), omeprazole and parentral nutrition. Within 2 weeks her pleural effusion resolved, her plasma amylase normalised and enteral nutrition was reintroduced. A magnetic resonance cholangiopancreatography delineated the pancreas and confirmed a dilated tortuous and beaded pancreatic duct with no evidence of pancreatopleural fistula or pseudocyst (Fig. 2). Further investigation confirmed that the patient and her father both carry the N291 mutation in the PRSS1 hereditary pancreatitis gene explaining her findings of chronic pancreatitis. The patient has remained symptom free with normal plasma amylase levels for 12 months following presentation, and her father has never had symptoms suggestive of pancreatitis. Pleural effusions commonly complicate acute pancreatitis and are usually small, left-sided and reactive. They arise from inflammation of the diaphragm and pleura, the amylase concentration of which may be moderately elevated. Pleural effusions arising from chronic pancreatitis are rare in children and result from either posterior disruption of the pancreatic duct into the retroperitoneal space leading to the formation of a fistulous tract between the pancreas and the pleural cavity through the aortic or oesophageal hiatus, or direct extension of a pancreatic pseudocyst [3]. These effusions tend to be large and recurrent with a dramatically elevated amylase content. M. Lawson (&) AE A. M. Dalzell Department of Paediatric Gastroenterology, Royal Liverpool Children’s Hospital NHS Trust Alder Hey, Eaton Road, L12 2AP Liverpool, UK E-mail: maureenlawson@doctors.org.uk Tel.: +44-151-2525373 Fax: +44-151-2525928

In a hospital-based study from England, antiHelicobacter pylori ( H.p ylori) IgG antibodies were found in 13.6 % of children presenting with recurrent abdominal pain; however, on endoscopy, only 64 % of them were found to have H. pylori gastritis [2]. We present an interesting association of H. pylori gastritis and autoimmune hepatitis in a 13-year-old patient. A 13-year-old girl was referred to our Centre with an acute onset of jaundice. There was no history of blood transfusion or recent exposure to drugs or toxins. Apart from jaundice and mild hepatomegaly, clinical examination was unremarkable. Her blood investigations showed a conjugated hyperbilirubinaemia of 71 lmol/l with ALT and AST levels of 572 IU/l and 909 IU/l respectively, a gamma-glutamyl transpeptidase activity of 52 IU/l and a slightly deranged clotting profile (INR 1.64). She had negative hepatitis A, B, C and Epstein Barr virus screens and normal serum coeruloplasmin and alpha-1-antitrypsin levels and phenotype. An autoantibody screen was positive for antinuclear and smooth muscle antibodies (a titre of 1:640 for both) and the antidouble stranded DNA antibody level was 10 IU/ml (normal value

We present a case of nonbullous impetigo neonatorum associated with late onset group B streptococcal meningitis in a 12-day-old infant. Both skin lesions and meningitis resolved with antibiotic therapy. This is the first reported case of meningitis during the course of this skin disease.

Background: Cow's milk-induced intestinal bleeding is a well-recognized cause of rectal bleeding in infancy. The authors report on 5 older children who presented with either visible rectal bleeding or profound anemia associated with occult intestinal bleeding secondary to cow's milk enteropathy. Methods: Five children (3 boys and 2 girls) aged between 20 months and 9 years were referred for further investigation of gastrointestinal bleeding. Two had been investigated previously on multiple occasions, and both had undergone laparotomies with negative results. Further investigations showed evidence of allergic colitis in 3 detected only on proximal colonic biopsy findings. Results: In all cases, bleeding resolved completely after instituting a cow's milk-free diet. Two of the patients subsequently have undergone a cow's milk challenge leading to prompt recurrence of symptoms, which again resolved after simple dietary manipulation. Conclusions: Cow's milk enteropathy may cause overt rectal bleeding or profound anemia from occult intestinal bleeding even in older children. Histological abnormalities in such cases may be confined to the proximal colon. After appropriate investigation, a trial of cow's milk exclusion should always be considered before laparotomy for obscure, chronic gastrointestinal bleeding in children.

Omeprazole; the first proton pump inhibitor (PPI) showing an effective acid inhibitory ability, provides the satisfactory therapy either in gastro-esophageal reflux symptom relief or in healing of erosive esophagitis. It's also effective in peptic ulcer disease. Up to date, omeprazole efficacy and safety are well established in many trials. Omeprazole-related hepatotoxicity is not very well recognized especially in pediatric population. We report a child who developed hepatitis following omeprazole intake. We believe that this is the first case report of omeprazole-induced hepatitis in pediatric population.

A 4 year old girl was referred to the paediatric department with a six month history of abdominal distension, irritable mood, reduced appetite, and weight loss. She had recently had loose stools four to five times a day without any associated blood or mucus and “crampy” type abdominal pains after eating foods such as bread and oats. This led her mother to suspect a “food allergy,” and avoidance of these foods improved her symptoms. She had no other medical problems. Her birth and developmental histories were normal, and there was no family history of medical problems. On examination she had a distended abdomen, which was soft on palpation and tympanic to percussion. There was no organomegaly, ascites, or lymphadenopathy. She had no peripheral signs of gastrointestinal disease, and all other examinations were unremarkable. Her height and weight were plotted on the 25th centile, and there were no previous measurements with which to compare. Blood investigations showed haemoglobin at 78 g/L (reference range 120-160), total white blood cell count 9.7×109/L (4-11), neutrophils 4.6×109/L (2-7.5), and platelets 211×109/L (150-500). Her blood film was normal, and C reactive protein concentration was 5 mg/L (

David Howard Casson (“Dave”) was born in Didsbury in Manchester, son of a general practitioner. He attended Manchester Grammar School and St Edmund Hall, Oxford, before postgraduate studies at St Bartholomew’s Hospital, London. Dave travelled widely from London to Bournemouth, Bath, and more, including Pakistan and Australia, and was appointed consultant paediatric gastroenterologist at Alder Hey in 2000. It was in Dave’s nature to always …

We investigated the nutritional requirements of four adolescent females with anorexia nervosa, and describe a management approach based on nutritional principles. The median age of presentation was 14.15 years (range 14-16.1 years). The median weight loss in the preceding six months was 27% (range 21-33%). At presentation the median energy intake was 2710 Kj per day (range 2083-4166 Kj) and represented a median of 26% of that expected. Energy requirements to reverse the state of severe malnutrition were 14634 kj per day. In addition to voluntary intake, energy was delivered via nasogastric tube using energy dense defined formulae in order to ensure a minimum intake of 150-200% RDA, until 50% of lost weight had been regained. The median time to regain 50% of lost weight was 44 days. Continued weight gain occurred during 1 year follow up. Energy deficits in patients with anorexia nervosa are considerable and can be safely and rapidly restored by an enteral feeding programme which is well tolerated by the patient.

Publisher Summary All countries that would consider entering into a multilateral arrangement for the storage of spent fuel would weigh the relative economic costs of various spent fuel storage programs. Therefore, the economics of any arrangement should be acceptable to the countries that might participate. This chapter discusses the economic elements governing the acceptability of a spent fuel storage regime to the participating countries. The economic model of the spent fuel arrangement presented in this chapter shows that countries could be encouraged to form international regional arrangements for interim storage; these regional arrangements can be justified on economic grounds, even over great distances, and are likely to have broader political appeal than bilateral arrangements with nuclear supplier states. Before enumerating the various costs of pool storage, interim storage costs should be viewed in the perspective of total fuel cycle costs and the general costs of electricity production. Interim storage and transportation costs amount to only about 3% of fuel cycle costs, and the fuel cycle costs in turn constitute only 15% of the total generating costs.