Gastrointestinal and nutritional care in pediatric neuromuscular disorders

Neuromuscular diseases(NMDs)affect the development and growth of the neuromuscular system in children.The pathology can occur anywhere along the neuromuscular pathway,from the brain to the nerves to the muscle fibers.These diseases have a profound impact on the quality of life not only of children but also of their families.The predominant manifestation in NMDs is hypotonia,which leads to muscle weakness and fatigue,reduced mobility,and decreased physical performance.However,multiple organ systems can be affected,with resulting orthopedic,cardiac,infectious,respiratory,and nutritional problems.Children with NMD present an increased risk for several dietary and feeding difficulties because of their neuromuscular diagnosis,presentation,and severity.These problems include chronic gastrointestinal issues(constipation,dysphagia,gastroesophageal reflux,and diarrhea),dysphagia,malnutrition,and body composition alterations.As a result,compared to the overall pediatric population,infants and children with NMD are more likely to be malnourished,ranging from failure to thrive to overweight or obesity.Disease-specific guidelines vary in level of detail and recommendations for dietary management.Overall,nutritional data available are sparse,with the exception of Duchenne muscular dystrophy,spinal muscular atrophy,and congenital muscular dystrophy.The purpose of this review is to describe the spectrum of nutritional challenges in children with NMD and to summarize the main dietary and gastrointestinal recommendations for each neuromuscular disorder to provide guidance for daily clinical practice.

Pediatric gastrointestinal bleeding: Perspectives from the Italian Society of Pediatric Gastroenterology

There are many causes of gastrointestinal bleeding(GIB) in children, and this condition is not rare, having a reported incidence of 6.4%. Causes vary with age, but show considerable overlap; moreover, while many of the causes in the pediatric population are similar to those in adults, some lesions are unique to children. The diagnostic approach for pediatric GIB includes definition of the etiology, localization of the bleeding site and determination of the severity of bleeding; timely and accurate diagnosis is necessary to reduce morbidity and mortality. To assist medical care providers in the evaluation and management of children with GIB, the "Gastro-Ped Bleed Team" of the Italian Society of Pediatric Gastroenterology, Hepatology and Nutrition(SIGENP) carried out a systematic search on MEDLINE via Pub Med(http://www.ncbi.nlm.nih.gov/pubmed/) to identify all articles published in English from January 1990 to 2016; the following key words were used to conduct the electronic search: "upper GIB" and "pediatric" [all fields]; "lower GIB" and "pediatric" [all fields]; "obscure GIB" and "pediatric" [all fields]; "GIB" and "endoscopy" [all fields]; "GIB" and "therapy" [all fields]. The identified publications included articles describing randomized controlled trials, reviews, case reports, cohort studies, casecontrol studies and observational studies. References from the pertinent articles were also reviewed. This paper expresses a position statement of SIGENP that can have an immediate impact on clinical practice and for which sufficient evidence is not available in literature. The experts participating in this effort were selected according to their expertise and professional qualifications.

Italian survey on non-steroidal anti-inflammatory drugsand gastrointestinal bleeding in children

AIM: To investigate gastrointestinal complications associated with non-steroidal anti-inflammatory drug(NSAIDs) use in children.METHODS: A retrospective, multicenter study was conducted between January 2005 and January 2013, with the participation of 8 Italian pediatric gastroenterology centers. We collected all the cases of patients who refer to emergency room for suspected gastrointestinal bleeding following NSAIDs consumption, and underwent endoscopic evaluation. Previous medical history, associated risk factors, symptoms and signs at presentation, diagnostic procedures, severity of bleeding and management of gastrointestinal bleeding were collected. In addition, data regarding type of drug used, indication, dose, duration of treatment and prescriber(physician or selfmedication) were examined. RESULTS: Fifty-one patients, including 34 males, were enrolled(median age: 7.8 years). Ibuprofen was the most used NSAID [35/51 patients(68.6%)]. Pain was the most frequent indication for NSAIDs use [29/51 patients(56.9%)]. Seven patients had positive family history of Helicobacter pylori(H. pylori) infection or peptic ulcer, and 12 had associated comorbidities. Twenty-four(47%) out of 51 patients used medication inappropriately. Hematemesis was the most frequent symptom(33.3%). Upper gastrointestinal endoscopy revealed gastric lesions in 32/51(62%) patients, duodenal lesions in 17(33%) and esophageal lesions in 8(15%). In 10/51(19.6%) patients, a diagnosis of H. pylori gastritis was made. Forty-eight(94%) patients underwent medical therapy, with spontaneous bleeding resolution, while in 3/51(6%) patients, an endoscopic hemostasis was needed.CONCLUSION: The data collected in this study confirms that adverse events with the involvement of the gastrointestinal tract secondary to NSAID use are also common in

Current issues in pediatric inflammatory bowel diseaseassociated arthropathies

Joint involvement is the most common extraintestinal manifestation in children with inflammatory bowel disease(IBD)and may involve 16%-33%of patients at diagnosis or during follow-up.It is possible to distinguish asymmetrical,transitory and migrating arthritis(pauciarticular and polyarticular)and spondyloarthropathy(SpA).Clinical manifestations can be variable,and peripheral arthritis often occurs before gastrointestinal symptoms develop.The inflammatory intestinal pattern is variable,ranging from sub-clinical inflammation conditions,classified as indeterminate colitis and nodular lymphoid hyperplasia of the ileum,to Crohn’s disease or ulcerative colitis.Unlike the axial form,there is an association between gut inflammation and evolution of recurrent peripheral articular disease that coincides with a flare-up of intestinal disease.This finding seems to confirm a key role of intestinal inflammation in the pathogenesis of SpA.An association between genetic background and human leukocyte antigen-B27 status is less common in pediatric than n adult populations.Seronegative sacroiliitis and SpA are the most frequent forms of arthropathy in children with IBD.In pediatric patients,a correct therapeutic approach relies on the use of nonsteroidal antiinflammatory drugs,local steroid injections,physiotherapy and anti-tumor necrosis factor therapy(infliximab).Early diagnosis of these manifestations reduces the risk of progression and complications,and as well as increasing the efficacy of the therapy.

Clinical significance of NOD2/CARD15 and Toll-like receptor 4 gene single nucleotide polymorphisms in inflammatory bowel disease

AIM: To evaluate the role of genetic factors in the pathogenesis of Crohn's disease (CD) and ulcerative colitis (UC), we investigated the single nucleotide poly- morphisms (SNPs) of NOD2/CARD15 (R702W, G908R and L1007fi nsC), and Toll-like receptor 4 (TLR4) genes (D299G and T399I) in a selected inflammatory bowel disease (IBD) population coming from Southern Italy. METHODS: Allele and genotype frequencies of NOD2/ CARD15 (R702W, G908R and L1007finsC) and TLR4 (D299G and T399I) SNPs were examined in 133 CD pa-tients, in 45 UC patients, and in 103 healthy controls. A genotype-phenotype correlation was performed. RESULTS: NOD2/CARD15 R702W mutation was sig-nificantly more frequent in CD (9.8%) than in controls (2.4%, P = 0.001) and in UC (2.3%, P = 0.03). No sig-nificant difference was found between UC patients and control group (P > 0.05). In CD and UC patients, no signifi cant association with G908R variant was found. L1007f insC SNP showed an association with CD (9.8%) compared with controls (2.9%, P = 0.002) and UC patients (2.3%, P = 0.01). Moreover, in CD patients, G908R and L1007finsC mutations were significantly associated with different phenotypes compared to CD wild-type patients. No association of IBD with the TLR4 SNPs was found in either cohort (allele frequencies: D299G-controls 3.9%, CD 3.7%, UC 3.4%, P > 0.05; T399I-controls 2.9%, CD 3.0%, UC 3.4%, P > 0.05). CONCLUSION: These findings confirm that, in our IBD patients selected from Southern Italy, the NOD2/ CARD15, but not TLR4 SNPs, are associated with in-creased risk of CD.

Partially hydrolyzed guar gum in pediatric functional abdominal pain

AIM:To assess the effects of partially hydrolyzed guar gum(PHGG) diet supplement in pediatric chronic abdominal pain(CAP) and irritable bowel syndrome(IBS).METHODS:A randomized,double-blind pilot study was performed in sixty children(8-16 years) with functional bowel disorders,such as CAP or IBS,diagnosed according to Rome Ⅲ criteria.All patients underwent ultrasound,blood and stool examinations to rule out any organic disease.Patients were allocated to receive PHGG at dosage of 5 g/d(n = 30) or placebo(fruitjuice n = 30) for 4 wk.The evaluation of the efficacy of fiber supplement included IBS symptom severity score(Birmingham IBS Questionnaire),severity of abdominal pain(Wong-Baker Face Pain Rating Score) and bowel habit(Bristol Stool Scale).Symptom scores were completed at 2,4,and 8 wk.The change from baseline in the symptom severity scale at the end of treatment and at 4 wk follow-up after treatment was the primary endpoint.The secondary endpoint was to evaluate compliance to supplementation with the PHGG in the pediatric population.Differences within groups during the treatment period and follow-up were evaluated by the Wilcoxon signed-rank test.RESULTS:The results of the study were assessed considering some variables,such as frequency and intensity of symptoms with modifications of the bowel habit.Both groups were balanced for baseline characteristics and all patients completed the study.Group A(PHGG group) presented a higher level of efficacy compared to group B(control group),(43% vs 5%,P = 0.025) in reducing clinical symptoms with modification of Birmingham IBS score(median 0 ± 1 vs 4 ± 1,P = 0.025),in intensity of CAP assessed with the Wong-Baker Face Pain Rating Score and in normalization of bowel habit evaluated with the Bristol Stool Scale(40% vs 13.3%,P = 0.025).In IBS subgroups,statistical analysis shown a tendency toward normalization of bowel movements,but there was no difference in the prevalence of improvement in two bowel habit subsets.PHGG was therefore better tolerated without any adverse effects.CONCLUSION:Although the cause of pediatric functional gastrointestinal disorders is not known,the results show that complementary therapy with PHGG may have beneficial effects on symptom control.

Review on sedation for gastrointestinal tract endoscopy in children by non-anesthesiologists

AIM: To present evidence and formulate recommendations for sedation in pediatric gastrointestinal(GI) endoscopy by non-anesthesiologists.METHODS: The databases MEDLINE, Cochrane and EMBASE were searched for the following keywords "endoscopy, GI", "endoscopy, digestive system" AND "sedation", "conscious sedation", "moderate sedation", "deep sedation" and "hypnotics and sedatives" for publications in English restricted to the pediatric age. We searched additional information published between January 2011 and January 2014. Searches for(upper) GI endoscopy sedation in pediatrics and sedation guidelines by non-anesthesiologists for the adult population were performed. RESULTS: From the available studies three sedation protocols are highlighted. Propofol, which seems to offer the best balance between efficacy and safety is rarely used by non-anesthesiologists mainly because of legal restrictions. Ketamine and a combination of a benzodiazepine and an opioid are more frequently used. Data regarding other sedatives, anesthetics and adjuvant medications used for pediatric GI endoscopy are also presented.CONCLUSION: General anesthesia by a multidisciplinary team led by an anesthesiologist is preferred. The creation of sedation teams led by non-anesthesiologists and a careful selection of anesthetic drugs may offer an alternative, but should be in line with national legislation and institutional regulations.

Pancreatic involvement in pediatric inflammatory bowel diseases

Background:Inflammatory bowel diseases(IBDs)are a group of chronic diseases affecting the gastrointestinal tract,with a disabling course.The incidence of IBDs is increasing in different geographical areas,indicating its emergence as a global disease,especially in children.Many patients with IBDs develop extraintestinal manifestations(EIMs)during follow-up,as IBDs have a potential risk of systemic involvement..Data sources:A systematic review of the literature was made to analyze latest studies on pancreatic involvement in children with IBD including our experience in assessing possible implications and its future application.Results:The involvement of the hepatobiliary system is considered a rare EIM of children with IBD,with an incidence much higher than that in the general population.Isolated pancreatic hyperenzymemia,which occurs in the absence of typical symptoms and/or characteristic imaging findings,may be found in many patients with IBD.The frequent causes of pancreatitis are drugs,bilio-pancreatic disorders,immunologic disturbances and pancreatic auto-antibodies,although in some cases idiopathic forms have been described.Conclusions:It is important to establish a correct diagnostic approach based on etiology and to assess the most appropriate therapeutic strategy,thus avoiding complications and improving the quality of life of children with IBD.