Children with Hirschsprung's Disease Report Dietary Effects on Gastrointestinal Complaints More Frequently than Controls.

Hirschspung's disease (HD) is a congenital gastrointestinal (GI) disorder frequently accompanied by GI complaints. Despite the lack of evidence regarding whether diet affects GI symptoms, advice on dietary changes is common. The aim was to investigate self-reported dietary effects on GI symptoms, comparing children with HD with healthy children. This was an observational, cross-sectional, self-reported case-control study using the validated Diet and Bowel Function questionnaire. All children with HD aged 1-18 years were surgically treated during 2003-2021 at a national HD center, and their parents were invited to participate. Healthy children served as controls. The data were presented as median (range) and n (%). 71/85 children with HD (6 years (1-17); 76% boys) and 265/300 controls (9 years (1-18); 52% boys) participated. Dietary effects on GI symptoms were reported more frequently by children with HD than controls (55/71 [77%] vs. 137/265 [52%], p ≤ 0.001), as were dietary adjustments to improve GI symptoms (49/71 [69%] vs. 84/265 [32%], p ≤ 0.001), and social limitations due to dietary adjustments (20/48 [42%] vs. 22/121 [18%], p = 0.002). Of 90 food items, children with HD reported that more of the items induced GI symptoms compared to controls (7 (0-66) vs. 2 (0-34), p = 0.001). Diet-induced GI symptoms and dietary adjustments' impact on daily life are reported more frequently by children with HD than controls. Moreover, the number and types of food items causing GI symptoms differ. The results indicate the need for disease-specific dietary advice to improve support for families of children with HD.

Self-Reported Effects of Diet on Gastrointestinal Symptoms in Healthy Children.

OBJECTIVES: Evidence on dietary effects on gastrointestinal (GI) symptoms in healthy children is lacking. Despite this, dietary advice is still common practice in the treatment of children's GI symptoms. The aim was to investigate self-reported dietary effects on GI symptoms in healthy children. METHODS: In this observational cross-sectional study on children, a validated self-reporting questionnaire including 90 specified food items was used. Healthy children aged 1-18 years old and their parents were invited to participate. Descriptive data were presented as median (range) and n (%). RESULTS: In total, 265 of 300 children (9 years [1-18]; 52% boys) answered the questionnaire. Overall, 21 of 265 (8%) reported that diet induced GI symptoms regularly. In total, 2 (0-34) food items were reported per child as inducing GI symptoms. The most frequently reported items were beans (24%), plums (21%), and cream (14%). More children with GI symptoms (constipation, abdominal pain, troublesome gases) than with No/Seldom GI symptoms reported that diet could potentially induce GI symptoms (17/77 [22%] vs 4/188 [2%], P ≤ 0.001). Furthermore, they adjusted their diet to regulate GI symptoms (16/77 [21%] vs 8/188 [4%], P ≤ 0.001). CONCLUSIONS: Few healthy children reported that diet induced GI symptoms, and only a minority of food items were reported to induce GI symptoms. Children who had already experienced GI symptoms reported that diet impacted on GI symptoms to a greater, but still very limited, extent. Results can be used to determine accurate expectations and goals of dietary treatment of GI symptoms in children.

Diet and bowel function in children with Hirschsprung's disease: development and content validation of a patient-reported questionnaire.

BACKGROUND: Although dietary adjustments are recommended frequently for bowel symptoms, evidence of diet's impact on bowel function is lacking. The aim was to develop a patient-reported outcome instrument, for children with and without Hirschsprung's disease (HD), to explore experiences of dietary effects on bowel function. METHODS: Children with and without HD and their parents participated. Questionnaire items regarding the impact of diet on bowel function originated from focus group discussions. Specific food items, reported in the literature or in focus groups to cause bowel functional effects, were listed requesting each item's effect size and effect type. Content validity was tested within two separate semistructured interviews. A pilot test was performed. Assessing comprehension, relevance and wording clarity structurally, revisions were made accordingly. Children's bowel function was assessed through the validated Rintala Bowel Function Score. RESULTS: A total of 13 children with and without HD, median age 7 (range 2-15) years, and 18 parents participated in the validation. Each question's relevance had been ranked highly early in the validation process but most questions needed refining for improving clarity and comprehension. Wordings regarding bowel symptoms and emotions connected to food in particular were perceived to be sensitive and complex. Specifically wording regarding some bowel symptoms (gases, pain) and parental stress emotions (guilt, ambivalence) were, consistent with participants' opinions, subjected to multiple step revisions. Following the validation process, which included two semistructure interviews with different participants and then a pilot test with a third cohort, a full track overview of changes and rewording made in all steps of the validation process was presented. The final questionnaire then comprised 13 questions assessing foods' significance for bowel function, emotions, social impact and 90 specific food items' possible effects and effect sizes on bowel function. CONCLUSIONS: The Diet and Bowel Function questionnaire, enabling answering by children, was developed and the content validated qualitatively. This report presents insights into the whole validation process, declaring reasons for the selected question- and answering options, and their wordings. The Diet and Bowel Function questionnaire can be used as a survey questionnaire to enhance understanding of dietary effects on bowel function in children, and its results can be supportive in improving dietary-treatment programs.

Diet plays a central role in parental self-treatment of children with Hirschsprung's disease-a qualitative study.

AIM: Hirschsprung's disease is a congenital disorder requiring surgery. Most children operated on for Hirschsprung's disease experience postoperative bowel dysmotility. Although various food is known to influence bowel motility, evidence of diet's role and dietary guidelines in treatment of bowel dysfunction in Hirschsprung's disease is lacking. The aim was to explore parental experiences of dietary effects on bowel function in children with Hirschsprung's disease. METHODS: A qualitative study including three focus groups with ten parents of children with Hirschsprung's disease at a national Hirschsprung's disease centre. Data were analysed through content analysis. RESULTS: Parents emphasised diet as a strong influencer on their child's bowel function in Hirschsprung's disease. They expressed great concerns about their responsibility and strived hard to explore and adjust dietary habits to control the child's bowel function. Families' daily and social lives were influenced by the child's diet and bowel function. The parents desired dietary support and guidelines to improve their confidence in self-treatment of Hirschsprung's disease. CONCLUSION: Dietary habits play a key role in parental self-treatment of bowel function in their children with Hirschsprung's disease. Dietary guidelines for patients with Hirschsprung's disease are anticipated.

EPCAM mutation update: Variants associated with congenital tufting enteropathy and Lynch syndrome.

The epithelial cell adhesion molecule gene (EPCAM, previously known as TACSTD1 or TROP1) encodes a membrane-bound protein that is localized to the basolateral membrane of epithelial cells and is overexpressed in some tumors. Biallelic mutations in EPCAM cause congenital tufting enteropathy (CTE), which is a rare chronic diarrheal disorder presenting in infancy. Monoallelic deletions of the 3' end of EPCAM that silence the downstream gene, MSH2, cause a form of Lynch syndrome, which is a cancer predisposition syndrome associated with loss of DNA mismatch repair. Here, we report 13 novel EPCAM mutations from 17 CTE patients from two separate centers, review EPCAM mutations associated with CTE and Lynch syndrome, and structurally model pathogenic missense mutations. Statistical analyses indicate that the c.499dupC (previously reported as c.498insC) frameshift mutation was associated with more severe treatment regimens and greater mortality in CTE, whereas the c.556-14A>G and c.491+1G>A splice site mutations were not correlated with treatments or outcomes significantly different than random simulation. These findings suggest that genotype-phenotype correlations may be useful in contributing to management decisions of CTE patients. Depending on the type and nature of EPCAM mutation, one of two unrelated diseases may occur, CTE or Lynch syndrome.

Factors influencing iron nutrition among one-year-old healthy children in Sweden.

AIMS AND OBJECTIVES: To describe possible social, nutritional and biological factors influencing iron intake and iron status among healthy one-year-old children in southern Sweden. BACKGROUND: Iron deficiency is one of the most important nutritional disorders and increases the risk of delayed mental and motor development. Children are at risk because of rapid growth, which entails relatively high requirements of iron. DESIGN: A prospective study using survey methods. METHOD: Randomly selected one-year-old children (n = 90) and their parents participated. Parents answered a questionnaire enquiring about demographic data and the child's feeding and health during the first year. The child's total food intake and blood samples (haemoglobin, mean corpuscular volume, S-ferritin and transferring receptor) were obtained. Results. Twenty-seven per cent of the children had an iron intake below the Nordic Nutrition Recommendations of 8 mg/day (NNR 2004). Follow-on formula and iron-fortified porridge contributed to 64% of the child's total iron intake. Partial breastfeeding and low maternal education correlated negatively with iron intake from complementary food. In total, 10·3% (n = 9) of the children were found to be iron-depleted (S-ferritin ≤ 12 µg/l), and 2·3% (n = 2) had iron deficiency with or without anaemia (Hb ≤ 100 g/l). CONCLUSIONS: One-year-old children in Sweden may be at risk of developing iron deficiency, but information about iron-rich food can improve iron status. RELEVANCE TO CLINICAL PRACTICE: Knowledge about factors influencing children's iron intake and iron status may improve the nutritional advice and education from the Child Health Services to prevent or detect iron deficiency.

Effects of high protein intakes.

Among other nutrients of breast milk, the amino acid pattern is considered normative throughout infancy. Exclusive breastfeeding by a healthy mother should be the standard from birth to 6 months. During the breastfeeding period the protein intake is low in the human being compared too many other animals. The protein content in breast milk is about 1 g/100 ml and the daily protein intake approximately 1 g/kg/day. When other foods are introduced during the weaning period the protein intake increases remarkably to 3-4 g/kg/day in spite of the fact that the protein requirement is decreasing. The long-term consequences of this phenomenon are obscure. A high protein intake has endocrine effects, such as the high levels of insulin and insulin-like growth factor-1. Furthermore, the metabolic effects with high levels of urea in serum and urine, and the high levels of many amino acids may exceed the capacity of the hepatic and renal systems to metabolize and excrete the excess of nitrogen. This may lead to acidosis and hypernatremic dehydration during periods of fever and diarrhea. Whether the risk of obesity later in life is decreased because of a low intake of protein during the breastfeeding period is still obscure.

Feeding preterm infants after hospital discharge: a commentary by the ESPGHAN Committee on Nutrition.

Survival of small premature infants has markedly improved during the last few decades. These infants are discharged from hospital care with body weight below the usual birth weight of healthy term infants. Early nutrition support of preterm infants influences long-term health outcomes. Therefore, the ESPGHAN Committee on Nutrition has reviewed available evidence on feeding preterm infants after hospital discharge. Close monitoring of growth during hospital stay and after discharge is recommended to enable the provision of adequate nutrition support. Measurements of length and head circumference, in addition to weight, must be used to identify those preterm infants with poor growth that may need additional nutrition support. Infants with an appropriate weight for postconceptional age at discharge should be breast-fed when possible. When formula-fed, such infants should be fed regular infant formula with provision of long-chain polyunsaturated fatty acids. Infants discharged with a subnormal weight for postconceptional age are at increased risk of long-term growth failure, and the human milk they consume should be supplemented, for example, with a human milk fortifier to provide an adequate nutrient supply. If formula-fed, such infants should receive special postdischarge formula with high contents of protein, minerals and trace elements as well as an long-chain polyunsaturated fatty acid supply, at least until a postconceptional age of 40 weeks, but possibly until about 52 weeks postconceptional age. Continued growth monitoring is required to adapt feeding choices to the needs of individual infants and to avoid underfeeding or overfeeding.

Prevalence of celiac disease: before and after a national change in feeding recommendations.

OBJECTIVE: A national change in infant feeding recommendations was proposed in 1996 in Sweden: a slow introduction to gluten during weaning was stressed, the recommendation being introduction at 4 instead of 6 months of age. The aim of the present study was to compare the prevalence of celiac disease in healthy young children born before and after the new feeding recommendations in 1996. MATERIAL AND METHODS: Sera from 679 children at a median age of 2.9 years (range 2.5-4.2 years) born between January 1996 and November 1997 were investigated with IgA-antigliadin antibodies (AGA) and IgA-endomysial autoantibodies (EMA) and compared with 690 age-matched children born between July 1992 and June 1993. Children with a positive test for EMA and AGA or EMA only were re-tested, and if positive at follow up, investigated with intestinal biopsy. RESULTS: At baseline, 2.2% (15/679) children were positive for EMA and another 0.6% (4/679) for both EMA and AGA. One child refused to be re-tested and eight children were still EMA positive at follow-up. Intestinal biopsy was performed in seven children (one declined biopsy), of whom three showed total villous atrophy. Two children with EMA titers 1:640, respectively, refused further participation in the study, but were strongly suspected to have celiac disease. In total, 0.7% (5/679) (95% confidence interval (CI) = 0.1-1.4%) were considered to have celiac disease compared with 1.3% (9/690) (95% CI = 0.4-2.2%) in the control group (p=0.4217). In addition, 0.3% of the children were diagnosed with symptomatic celiac disease compared with 0.7% in controls (p=0.0134). CONCLUSIONS: The prevalence of symptomatic celiac disease declined after the infant dietary recommendations were introduced in 1996, but we could not find any difference in undiagnosed celiac disease between the screened children born before and those born after 1996.

Soy protein infant formulae and follow-on formulae: a commentary by the ESPGHAN Committee on Nutrition.

This comment by the European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) Committee on Nutrition summarizes available information on the composition and use of soy protein formulae as substitutes for breastfeeding and cows' milk protein formulae as well as on their suitability and safety for supporting adequate growth and development in infants. Soy is a source of protein that is inferior to cows' milk, with a lower digestibility and bioavailability as well as a lower methionine content. For soy protein infant formulae, only protein isolates can be used, and minimum protein content required in the current European Union legislation is higher than that of cows' milk protein infant formulae (2.25 g/100 kcal vs. 1.8 g/100kcal). Soy protein formulae can be used for feeding term infants, but they have no nutritional advantage over cows' milk protein formulae and contain high concentrations of phytate, aluminum, and phytoestrogens (isoflavones), which might have untoward effects. There are no data to support the use of soy protein formulae in preterm infants. Indications for soy protein formulae include severe persistent lactose intolerance, galactosemia, and ethical considerations (e.g., vegan concepts). Soy protein formulae have no role in the prevention of allergic diseases and should not be used in infants with food allergy during the first 6 months of life. If soy protein formulae are considered for therapeutic use in food allergy after the age of 6 months because of their lower cost and better acceptance, tolerance to soy protein should first be established byclinical challenge. There is no evidence supporting the use of soy protein formulae for the prevention or management of infantile colic, regurgitation, or prolonged crying.

Mothers' experiences of feeding situations - an interview study.

AIM: The aim of the study was to describe parents' experiences concerning feeding situations and their contact with the nurse at the Child Health Service (CHS). BACKGROUND: Some of the most important tasks for the nurse at the CHS are to monitor growth, detect feeding difficulties and give advice concerning food intake and feeding practices. METHOD: Eighteen mothers differing in age, education, ethnicity and number of children and recruited from different CHS were interviewed. The narratives were transcribed verbatim and analysed by content analysis at manifest and latent levels. RESULT: All mothers' described that food and feeding were essential parts of their lives requiring a great deal of time and involvement. Two major categories of mothers' attitudes in feeding situations were identified - a flexible attitude and a controlling attitude. Mothers with a flexible attitude were sensitive to the child's signals and responded to them in order to obtain good communication. Mothers who expressed a need for control established rules and routines regarding the feeding situations. Mothers with a controlling attitude expressed receiving inadequate support from the nurse at the CHS. CONCLUSION AND CLINICAL IMPLICATION: This study shows that some mothers experience inadequate support from the nurse at the CHS. Knowledge about mothers' experiences of feeding situations and their different attitudes towards the child during feeding might improve the CHS nurses' knowledge and help them understand and more adequately support mothers who experience feeding difficulties.

Prebiotic oligosaccharides in dietetic products for infants: a commentary by the ESPGHAN Committee on Nutrition.

This article by the ESPGHAN Committee on Nutrition summarizes available information on the effects of adding prebiotic oligosaccharides to infant and follow-on formulae. Currently there are only limited studies evaluating prebiotic substances in dietetic products for infants. Although administration of prebiotic oligosaccharides has the potential to increase the total number of bifidobacteria in feces and may also soften stools, there is no published evidence of clinical benefits of adding prebiotic oligosaccharides to dietetic products for infants. Data on oligosaccharide mixtures in infant formulae do not demonstrate adverse effects, but further evaluation is recommended. Combinations and dosages in addition to those so far studied need to be fully evaluated with respect to both safety and efficacy before their use in commercial infant food products. Well-designed and carefully conducted randomized controlled trials with relevant inclusion/exclusion criteria, adequate sample sizes and validated clinical outcome measures are needed both in preterm and term infants. Future trials should define optimal quantity and types of oligosaccharides with prebiotic function, optimal dosages and duration of intake, short and long term benefits and safety. At the present time, therefore, the Committee takes the view that no general recommendation on the use of oligosaccharide supplementation in infancy as a prophylactic or therapeutic measure can be made.

Preparation and handling of powdered infant formula: a commentary by the ESPGHAN Committee on Nutrition.

Powdered infant formulae are not sterile and may contain pathogenic bacteria. In addition, milk products are excellent media for bacterial proliferation. Multiplication of Enterobacter sakazakii in prepared formula feeds can cause devastating sepsis, particularly in the first 2 months of life. In approximately 50 published case reports of severe infection, there are high rates of meningitis, brain abscesses and necrotizing enterocolitis, with an overall mortality from 33% to 80%. Breast feeding provides effective protection against infection, one of the many reasons why it deserves continued promotion and support. To minimize the risk of infection in infants not fully breastfed, recommendations are made for preparation and handling of powdered formulae for children younger than 2 months of age. In the home setting, powdered infant formulae should be freshly prepared for each feed. Any milk remaining should be discarded rather than used in the following feed. Infant feeds should never be kept warm in bottle heaters or thermoses. In hospitals and other institutions written guidelines for preparation and handling of infant formulae should be established and their implementation monitored. If formula needs to be prepared in advance, it should be prepared on a daily basis and kept at 4 degrees C or below. Manufacturers of infant formulae should make every effort to minimize bacterial contamination of powdered products.

Nondigestible carbohydrates in the diets of infants and young children: a commentary by the ESPGHAN Committee on Nutrition.

The consumption of nondigestible carbohydrates is perceived as beneficial by health professionals and the general public, but the translation of this information into dietary practice, public health recommendations, and regulatory policy has proved difficult. Nondigestible carbohydrates are a heterogeneous entity, and their definition is problematic. Without a means to characterize the dietary components associated with particular health benefits, specific attributions of these cannot be made. Food labeling for "fiber" constituents can be given only in a general context, and the development of health policy, dietary advice, and education, and informed public understanding of nondigestible carbohydrates are limited. There have, however, been several important developments in our thinking about nondigestible carbohydrates during the past few years. The concept of fiber has expanded to include a range of nondigestible carbohydrates. Their fermentation, fate, and effects in the colon have become a defining characteristic; human milk, hitherto regarded as devoid of nondigestible carbohydrates, is now recognized as a source for infants, and the inclusion of nondigestible carbohydrates in the diet has been promoted for their "prebiotic" effects. Therefore, a review of the importance of nondigestible carbohydrates in the diets of infants and young children is timely. The aims of this commentary are to clarify the current definitions of nondigestible carbohydrates, to review published evidence for their biochemical, physiologic, nutritional, and clinical effects, and to discuss issues involved in defining dietary guidelines for infants and young children.