婴儿配方粉成分的全球标准:欧洲儿科胃肠病、肝病和营养学会牵头的国际专家组建议

联合国粮食及农业组织(FAO)和WHO的食品法典委员会提出的食品标准、指南和其他相关文件旨在保护消费者健康以及全球的公平贸易。全世界的大多数人口居住在160多个食品法典成员国的国度里。法典中关于婴儿配方粉的标准是在1981年被采纳的,其制定依据是20世纪70年代的研究资料,目前正在对这一标准进行修订。作为修订过程的一部分,营养和特殊膳食用食品法典委员会(Codex Committee on Nutrition and Foods for Special DietaryUses,CCNFSDU)要求欧洲儿科胃肠病、肝病和营养学会(European Society of Paediatric Gastroenterology,Hep…     

2017年北美及欧洲儿科胃肠肝脏病和营养学会婴儿胆汁淤积性黄疸评估指南的胆道闭锁评估解读

胆汁淤积(cholestasis)按病因可分为胆管性(梗阻、肝外胆管扩张或肝内胆管过小)和肝细胞性(膜转运体缺陷,遗传或代谢性障碍)[1].胆汁淤积性黄疸在足月产婴儿发病率约为1/2 500,临床上常与新生儿内科性黄疸相混淆[2].婴儿胆汁淤积性黄疸常见的病因包括胆道闭锁(25％～40％)、遗传性疾病(25％)、胆总管结石或胆总管囊肿导致的肝外梗阻、代谢障碍(酪氨酸血症Ⅰ型、半乳糖血症、先天性胆汁酸代谢障碍)、全垂体功能减退、Alagille综合征、感染及肠外营养相关的肝脏疾病[3].     

风湿热的一级预防及急性链球菌咽炎的诊治——美国心脏学会儿童心血管疾病委员会风湿热、心内膜炎和川崎病组及美国儿科学会2009年指南节选

急性风湿热一级预防的主要方法是合理诊断并应用足量抗生素治疗A组乙型溶血性链球菌咽炎.A组乙型溶血性链球菌咽炎依靠临床表现和实验室检查结果综合诊断,其金标准是咽培养.治疗上首选青霉素,对青霉素过敏者,可口服头孢菌素、庆大霉素或大环内酯类/氮杂内酯类药物.     

Role of wireless capsule endoscopy in reclassifying inflammatory bowel disease in children

ObjectiveTo evaluate the role of wireless capsule endoscopy in identifying small bowel lesions in pediatric patients with newly diagnosed colonic inflammatory bowel disease (IBD) type unclassified (IBDU), and to assess whether capsule endoscopy findings result in altered patient management.MethodsTen pediatric patients recently diagnosed with IBDU through standard investigations were recruited from the pediatric gastroenterology clinic at McMaster Children's Hospital to undergo capsule endoscopy using the Pillcam SB^TM (Given Imaging) capsule. Findings consistent with a diagnosis of Crohn's disease required the identification of at least three ulcerations.ResultsThree out of ten patients had newly identified findings on capsule endoscopy that met criteria for Crohn's disease. Three more patients had findings suspicious for Crohn's disease, but failed to meet the diagnostic criteria. Three additional patients had findings most consistent with ulcerative colitis, and one had possible gastritis with a normal intestine. Findings from capsule endoscopy allowed for changes in the medical management of three patients. In all ten cases, capsule endoscopy allowed for a better characterization of the type and extent of disease. No adverse outcomes occurred in the present cohort.ConclusionThis prospective study reveals that wireless capsule endoscopy is feasible, valuable, and non-invasive, offering the ability to potentially better characterize newly diagnosed pediatric IBDU cases by identifying lesions in the small bowel and reclassifying these as Crohn's disease.     

Usefulness of wireless capsule endoscopy for detecting inflammatory bowel disease in children presenting with arthropathy

Inflammatory bowel disease (IBD) is a cause of chronic intestinal inflammation in children. In a subset of patients affected by IBD, arthropathy may be the leading presenting sign. In the past years, remarkable advances in gastrointestinal endoscopy techniques have been achieved; recently, the development of capsule endoscopy (CE) provided a non-invasive method for the complete endoscopic evaluation, including small bowel assessment. We report three children suffering from IBD but presenting with articular complaints in whom CE was a useful tool for detecting gut inflammation. Patients were investigated with the wireless CE: PillCam™ SB2 (Given Imaging, Yoqneam, Israel) capsule, the second-generation capsule, was used in our paediatric patients. Three patients were initially evaluated for arthropathy. Enteropathic arthritis was suspected for gastrointestinal symptoms and/or persistence of inflammatory markers elevation. In one of these children, conventional endoscopy was refused by parents, while in the other two children, CE was proposed as first-line diagnostic tool. In all patients, CE revealed to be safe and provided information that led to diagnosis. Paediatric rheumatologists should consider CE as a valid, non-invasive tool, eventually first level diagnostic approach in order to evaluate the presence of IBD in children presenting with chronic articular complaints.     

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??Inflammatory bowel disease??IBD????including ulcerative colitis??UC?? and Crohn’s disease??CD????is a chronic relapsing inflammatory disorder of the gastrointestinal tract. Probiotics could be of benefit since involvement of the microbiota in the pathogenesis of IBD is evident. Although some promising studies are underway??the evidence for probiotics is still insufficient to recommend its use for IBD in daily clinical practice. Available data showed that the primary outcomes were correlated with probiotic strains and dosages. In general??probiotics have proven valuable in the management of ulcerative colitis. However??the probiotics seem to confer little benefit in Crohn’s disease.     

Carditis complicating inflammatory bowel disease in children

A 10-year-old child with ulcerative colitis is described who during the quiescent phase of his disease suffered from pericarditis with pleuropericardial effusion. The literature on cardiac involvement in inflammatory bowel disease is reviewed with emphasis on the paediatric age group.     

Diagnostic role of upper gastrointestinal endoscopy in pediatric inflammatory bowel disease

BACKGROUND: Discrimination between ulcerative colitis (UC) and Crohn disease (CD) may be difficult on ileo-colonoscopy alone because of a lack of definitive lesions. Retrospective studies show upper gastrointestinal endoscopy may be helpful in confirming diagnosis in such cases. AIMS: To prospectively determine importance of upper gastrointestinal endoscopy in diagnosis of inflammatory bowel disease (IBD) and assess factors predictive of upper gastrointestinal involvement in IBD. METHODS: All pediatric patients were enrolled prospectively and consecutively over a 2-year period and investigated with an ileo-colonoscopy and barium meal follow-through. Children with procto-sigmoiditis, later confirmed histologically to be typical of UC, were excluded from the study. The remainder underwent upper gastrointestinal endoscopy. The protocol and methodology were determined a priori. RESULTS: 65 children suspected of IBD underwent colonoscopy. Of the total, 11 had recto-sigmoiditis with typical macroscopic appearances of UC; once this was confirmed on histology these patients were excluded from the study. Of the 54 children (males, 31; median age, 11.1 years) remaining, 23 were initially diagnosed with CD on ileo-colonoscopy and 18 (33%) were diagnosed with UC. The diagnosis remained ambiguous in 13 (six colonic, four ileo-colonic, three normal colon) on clinical, radiologic and histologic grounds. Upper GI endoscopy helped to confirm CD in a further 11 (20.4%). Two patients were diagnosed with indeterminate colitis.Upper gastrointestinal inflammation was seen in 29 of 54 (22 CD; 7 UC ). Epigastric and abdominal pain, nausea and vomiting, weight loss and pan-ileocolitis were predictive of upper gastrointestinal involvement (P < 0.05). However, 9 children with upper gastrointestinal involvement were asymptomatic at presentation (31%). Overall upper gastrointestinal tract inflammation was most common in the stomach (67%), followed by the esophagus (54%) and duodenum (22%). CONCLUSIONS: Upper gastrointestinal tract endoscopy should be part of the first-line investigation in all new cases suspected of IBD. Absence of specific upper gastrointestinal symptoms do not preclude presence of upper gastrointestinal inflammation.     

Hyperhomocystinemia in children with inflammatory bowel disease

OBJECTIVES: Thromboembolism is a significant cause of morbidity and mortality in patients with inflammatory bowel disease (IBD). Plasma total homocysteine (tHcy) is a risk factor for vascular disease and has been implicated as a mediator of thromboembolic events in adults with IBD. The authors studied the link between tHcy and IBD in children, in whom associations may be clearer, and investigated associations with plasma von Willebrand factor antigen, a marker of vascular damage. METHODS: This cross-sectional study included 43 patients with IBD (27 Crohn disease, 9 ulcerative colitis, and 7 indeterminate colitis) and 46 control subjects from a pediatric gastroenterology clinic. Plasma tHcy, plasma 5-methyl tetrahydrofolate, red cell folate, plasma vitamin B12, plasma von Willebrand factor antigen, and methylene tetrahydrofolate reductase (MTHFR) genotype (for the C677T mutation) were measured. RESULTS: Plasma tHcy concentrations were higher in children with IBD than in control subjects, when corrected for age (P < 0.05), and plasma tHcy was negatively correlated with plasma 5 methyl tetrahydrofolate (P < 0.0005). Plasma 5 methyl tetrahydrofolate and age were the main predictors of plasma tHcy. Neither MTHFR genotype nor von Willebrand factor showed any association with any other measure, and there were no differences between children with IBD and control subjects. CONCLUSIONS: Elevated plasma tHcy is a consequence of IBD in children, probably mediated by poor folate status associated with diet or the pathophysiology of the disease.     

Amyloidosis in children with inflammatory bowel disease

Reactive systemic or secondary amyloidosis occurs in 1-29% of adults with Crohn's disease, but only sporadic cases of amyloidosis have been recognized in children with inflammatory bowel disease. We therefore have studied operative specimens (ileal, ileocolonic, and colonic) from 46 children (30 with Crohn's disease and 16 with ulcerative colitis) to determine the frequency of amyloid deposits. Sections of bowel, skin, and lymph nodes (n = 940) were stained by Congo red and examined by light microscopy and by polarized light. Amyloid deposits were found in only one of 46 subjects, an 18-year-old girl who had had Crohn's disease for 6 years. Intestinal amyloid deposits, present 16 months before the clinical diagnosis of amyloidosis, were patchy and seen predominantly in the intestinal mucosa. We conclude that amyloidosis is rare in children requiring surgery for Crohn's disease and ulcerative colitis. Examination of Congo red-stained sections can detect even subclinical amyloidosis. The amyloid deposits in our patient, which were both patchy and consistently mucosal, suggest that multiple endoscopic biopsy samples, not necessarily containing submucosa, are sufficient for diagnosis.     

Myocarditis in children with inflammatory bowel disease

Inflammatory bowel disease (IBD) in children and adults is often associated with extracolonic manifestations in other organs, such as the joints, skin, and liver. Cardiac manifestations are considered to be very rare; in a recent survey of the literature we found only 23 cases, most of them in adults. Several extensive reviews and standard textbooks of IBD do not even mention perimyocarditis as a complication. During the past 10 years we have treated 106 patients with IBD in the Department of Pediatrics at St. G?ran's Children's Hospital. Of these patients, two adolescent boys developed myocarditis as an extracolonic manifestation. In reporting these two cases, we wish to point out that cardiac involvement occurs in children and we also wish to show that the prognosis is usually good. Another interesting feature was that both of these boys developed myocarditis at a time when one of them had only minor gastrointestinal symptoms and the other had not yet been diagnosed as having IBD.