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Showing papers in "Journal of Pediatric Gastroenterology and Nutrition in 2012"


Journal ArticleDOI
TL;DR: The aim of the new guidelines for coeliac disease was to achieve a high diagnostic accuracy and to reduce the burden for patients and their families.
Abstract: Objective: Diagnostic criteria for coeliac disease (CD) from the European Society for Paediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN) were published in 1990. Since then, the autoantigen in CD, tissue transglutaminase, has been identified; the perception of CD has changed from that of a rather uncommon enteropathy to a common multiorgan disease strongly dependent on the haplotypes human leukocyte antigen (HLA)-DQ2 and HLA-DQ8; and CD-specific antibody tests have improved. Methods: A panel of 17 experts defined CD and developed new diagnostic criteria based on the Delphi process. Two groups of patients were defined with different diagnostic approaches to diagnose CD: children with symptoms suggestive of CD (group 1) and asymptomatic children at increased risk for CD (group 2). The 2004 National Institutes of Health/ Agency for Healthcare Research and Quality report and a systematic literature search on antibody tests for CD in paediatric patients covering the years 2004 to 2009 was the basis for the evidence-based recommendations on CD-specific antibody testing. Results: In group 1, the diagnosis of CD is based on symptoms, positive serology, and histology that is consistent with CD. If immunoglobulin A anti-tissue transglutaminase type 2 antibody titers are high (>10 times the upper limit of normal), then the option is to diagnose CD without duodenal biopsies by applying a strict protocol with further laboratory tests. In group 2, the diagnosis of CD is based on positive serology and histology. HLADQ2 and HLA-DQ8 testing is valuable because CD is unlikely if both haplotypes are negative. Conclusions: The aim of the new guidelines was to achieve a high diagnostic accuracy and to reduce the burden for patients and their families. The performance of these guidelines in clinical practice should be evaluated prospectively. (JPGN 2012;54: 136–160)

2,242 citations


Journal ArticleDOI
TL;DR: This guideline provides recommendations for the diagnosis and management of suspected cow's-milk protein allergy (CMPA) in Europe and presents a practical approach with a diagnostic algorithm and is based on recently published evidence-based guidelines on CMPA.
Abstract: Objectives: This guideline provides recommendations for the diagnosis and management of suspected cow’s-milk protein allergy (CMPA) in Europe. It presents a practical approach with a diagnostic algorithm and is based on recently published evidence-based guidelines on CMPA. Diagnosis: If CMPA is suspected by history and examination, then strict allergen avoidance is initiated. In certain circumstances (eg, a clear history of immediate symptoms, a life-threatening reaction with a positive test for CMP–specific IgE), the diagnosis can be made without a milk challenge. In all other circumstances, a controlled oral food challenge (open or blind) under medical supervision is required to confirm or exclude the diagnosis of CMPA. Treatment: In breast-fed infants, the mother should start a strict CMPfree diet. Non–breast-fed infants with confirmed CMPA should receive an extensively hydrolyzed protein-based formula with proven efficacy in appropriate clinical trials; amino acids–based formulae are reserved for certain situations. Soy protein formula, if tolerated, is an option beyond 6 months of age. Nutritional counseling and regular monitoring of growth are mandatory in all age groups requiring CMP exclusion. Reevaluation: Patients should be reevaluated every 6 to 12 months to assess whether they have developed tolerance to CMP. This is achieved in >75% by 3 years of age and >90% by 6 years of age. Inappropriate or overly long dietary eliminations should be avoided. Such restrictions may impair the quality of life of both child and family, induce improper growth, and incur unnecessary health care costs.

495 citations


Journal ArticleDOI
TL;DR: Indications for liver biopsy include the following: to rule out other treatable diseases, in cases of clinically suspected advanced liver disease, before pharmacological/surgical treatment, and as part of a structured intervention protocol or clinical research trial.
Abstract: Nonalcoholic fatty liver disease (NAFLD) is the most common cause of chronic liver disease in children and adolescents in the United States, and most probably also in the rest of the industrialized world.As the prevalence of NAFLD in childhood increases with the worldwide obesity epidemic, there is an urgent need for diagnostic standards that can be commonly used by pediatricians and hepatologists. To this end, we performed a PubMed search of the adult and pediatric literature on NAFLD diagnosis through May 2011 using Topics and/or relevant Authors as search words. According to the present literature, NAFLD is suspected based on the association of fatty liver combined with risk factors (mainly obesity), after the exclusion of other causes of liver disease. The reference but imperfect standard for confirming NAFLD is liver histology. The following surrogate markers are presently used to estimate degree of steatosis and liver fibrosis and risk of progression to end-stage liver disease: imaging by ultrasonography or magnetic resonance imaging, liver function tests, and serum markers of liver fibrosis.NAFLD should be suspected in all of the overweight or obese children and adolescents older than 3 years with increased waist circumference especially if there is a NAFLD history in relatives. The typical presentation, however, is in children ages 10 years and older. The first diagnostic step in these children should be abdominal ultrasound and liver function tests, followed by exclusion of other liver diseases. Overweight/obese children with normal ultrasonographic imaging and normal liver function tests should still be monitored due to the poor sensitivity of these tests at a single assessment.Indications for liver biopsy include the following: to rule out other treatable diseases, in cases of clinically suspected advanced liver disease, before pharmacological/surgical treatment, and as part of a structured intervention protocol or clinical research trial.

420 citations


Journal ArticleDOI
TL;DR: It was found that children with pancreatitis were primarily managed by pediatric gastroenterologists, unless the etiology was known, and initial investigations included serum liver enzymes, triglycerides, calcium, and abdominal ultrasound.
Abstract: Objectives There is limited literature on acute pancreatitis (AP), acute recurrent pancreatitis (ARP) and chronic pancreatitis (CP) in children. The INSPPIRE (International Study group of Pediatric Pancreatitis: In search for a cure) consortium was formed to standardize definitions, develop diagnostic algorithms, investigate disease pathophysiology and design prospective multicenter studies in pediatric pancreatitis.

350 citations


Journal ArticleDOI
TL;DR: These guidelines provide clinically useful points to guide the management of UC in children and offer a standardized protocol that allows effective, timely management and monitoring of the disease course, while acknowledging that each patient is unique.
Abstract: Background and Aims: Pediatric ulcerative colitis (UC) shares many features with adult-onset disease but there are some unique considerations; therefore, therapeutic approaches have to be adapted to these particular needs. We aimed to formulate guidelines for managing UC in children based on a systematic review (SR) of the literature and a robust consensus process. The present article is a product of a joint effort of the European Crohn’s and Colitis Organization (ECCO) and the European Society for Paediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN). Methods: A group of 27 experts in pediatric IBD participated in an iterative consensus process including 2 face-to-face meetings, following an open call to ESPGHAN and ECCO members. A list of 23 predefined questions were addressed by working subgroups based on a SR of the literature. Results: A total of 40 formal recommendations and 68 practice points were endorsed with a consensus rate of at least 89% regarding initial evaluation, how to monitor disease activity, the role of endoscopic evaluation, medical and surgical therapy, timing and choice of each medication, the role of combined therapy, and when to stop medications. A management flowchart, based on the Pediatric Ulcerative Colitis Activity Index (PUCAI), is presented.

334 citations


Journal ArticleDOI
TL;DR: IgA-EmA and IgA-anti-TG2/EmA tests appear highly accurate to diagnose coeliac disease in children and may help in excluding CD, while POC tests show inferior accuracy.
Abstract: Objective The aim of this study was to summarise the evidence from 2004 to September 2009 on the performance of laboratory-based serological and point of care (POC) tests for diagnosing coeliac disease (CD) in children using histology as reference standard. Patients and methods We searched MEDLINE and EMBASE for studies reporting on children for tests based on IgA and IgG anti-gliadin (AGA), endomysial (EmA), anti-transglutaminase-2 (TG2), and anti-deamidated gliadin peptides (DGP) antibodies or POC tests. For inclusion, histological analysis of duodenal biopsies and sensitivity and specificity for index tests had to be reported. Data were pooled and summary measures calculated for sensitivity, specificity, positive and negative likelihood ratios ("LR+", "LR-"), and diagnostic odds ratios (DOR). In case of elevated statistical heterogeneity, studies reaching 90% sensitivity or specificity were reported. Results A total of 2510 articles were reviewed; 16 entered meta-analysis, reporting on 3110 patients (1876 with CD, 1234 without CD). For IgA-EmA, sensitivity was ≥90% in 7/11 studies and pooled specificity 98.2%. For IgA-anti-TG2, 11/15 studies yielded sensitivities ≥90% and 13/15 specificities ≥90%. For IgA-DGP, sensitivity ranged between 80.7% and 95.1% (specificity 86.3%-93.1%); for IgG-DGP between 80.1% and 98.6% (specificity 86.0-96.9%). IgA-EmA had the highest pooled DOR (554) and LR+ (31.8) for a laboratory test, followed by IgA-anti-TG2, IgG-DGP, IgA-DGP and IgA-AGA. POC tests showed a pooled sensitivity of 96.4% for IgA-TG2 (specificity 97.7%). Conclusions IgA-EmA and IgA-anti-TG2 tests appear highly accurate to diagnose CD. IgG-anti-DGP tests may help in excluding CD. IgA-AGA and IgA-DGP tests show inferior accuracy. POC tests may achieve high accuracy in the hands of experienced readers, but IgA-anti-TG2/EmA were superior.

239 citations


Journal ArticleDOI
TL;DR: The study provides evidence for the specific relation between HMO-grown bifidobacteria and intestinal epithelial cells interaction, and is the first study describing H MO-induced changes in the bifIDobacteria–intestinal cells interaction.
Abstract: Objectives Human milk oligosaccharides (HMO) are the third most abundant component of breast milk Our laboratory has previously revealed gene clusters specifically linked to HMO metabolism in select bifidobacteria isolated from fecal samples of infants Our objective was to test the hypothesis that growth of select bifidobacteria on HMO stimulates the intestinal epithelium

214 citations


Journal ArticleDOI
TL;DR: Administration of a follow-on formula with L fermentum CECT5716 may be useful for the prevention of community-acquired gastrointestinal and upper respiratory infections.
Abstract: Objectives:The aim of the study was to examine the effects of a follow-on formula containing Lactobacillus fermentum CECT5716 (L fermentum) on the incidence of infections in infants between the ages of 6 and 12 months.Patients and Methods:A randomized double-blinded controlled study includin

212 citations


Journal ArticleDOI
TL;DR: The guideline details the epidemiology and natural history of HCV infection in children, the diagnostic workup, monitoring and treatment of disease, and provides an update on future treatment options and areas of research.
Abstract: Hepatitis C virus (HCV) is an RNA virus that affects >180 million individuals worldwide with a high propensity for chronic infection. Children with HCV infection differ from adults in several ways including some modes of transmission, rates of clearance, progression of fibrosis, and the duration of potential chronic infection when acquired at birth. Since the discovery of HCV in 1989, there have been significant advances in the understanding of the virology and natural history of chronic HCV infection in children. In addition, there are now several treatment options for children with chronic hepatitis C infection and many new therapies on the horizon. As a consequence, the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition brought together experts in pediatric hepatology to review the available data in children and provide clinicians with approaches to the diagnosis, management, and prevention of HCV infection in children and adolescents. The guideline details the epidemiology and natural history of HCV infection in children, the diagnostic workup, monitoring and treatment of disease, and provides an update on future treatment options and areas of research.

176 citations


Journal ArticleDOI
TL;DR: An algorithm that uses the number of magnets ingested, location of magnets, and the timing of ingestion before intervention helps to delineate the roles of the pediatric gastroenterologists and surgeons in the management of ingested rare-earth magnets in children.
Abstract: We describe a comprehensive algorithm for the management of ingested rare-earth magnets in children. These newer and smaller neodymium magnets sold as adult toys are much stronger than the traditional magnets, and can attract each other with formidable forces. If >1 magnet is swallowed at the same time, or a magnet is co-ingested with another metallic object, the loops of intestine can be squeezed between them resulting in bowel damage including perforations. An algorithm that uses the number of magnets ingested, location of magnets, and the timing of ingestion before intervention helps to delineate the roles of the pediatric gastroenterologists and surgeons in the management of these cases.

153 citations


Journal ArticleDOI
TL;DR: The gut microbiota of 6-month-old infants in a low- Income country differs significantly from that in a high-income country, which may have an effect on both the energy harvest from the diet typifying malnutrition and diarrheal diseases in low-income countries and Western lifestyle diseases in high- income countries.
Abstract: Background and Objective:The intestinal microbiota composition in infants reflects the early environment. Our objective was to compare the gut microbiota in 6-month-old infants living in rural Malawi with children of the same age living in urban Finland, both being breast-fed and having an a

Journal ArticleDOI
TL;DR: The present guideline is intended to aid physicians in developing an enteral nutrition therapy program and potentially promote its use.
Abstract: Exclusive enteral nutrition is an effective yet often underused therapy for the induction of remission in pediatric Crohn disease. The North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition formed the Enteral Nutrition Working Group to review the use of enteral nutrition therapy in pediatric Crohn disease. The group was composed of 5 pediatric gastroenterologists and 1 pediatric nutritionist, all with an interest and/or expertise in exclusive enteral nutrition. Specific attention was placed upon review of the evidence for efficacy of therapy, assessment of the variations in care, identification of barriers to its widespread use, and compilation of the necessary components for a successful program. The present guideline is intended to aid physicians in developing an enteral nutrition therapy program and potentially promote its use.

Journal ArticleDOI
TL;DR: Gastrointestinal and nutritional problems perceived by parents are prevalent throughout life in girls and women with RTT and may pose a substantial medical burden for their caregivers, and physician awareness of these features of RTT may improve the health and quality of life of individuals affected with this disorder.
Abstract: Objective:We conducted a nationwide survey to determine the prevalence of common gastrointestinal and nutritional disorders in Rett syndrome (RTT) based on parental reporting and related the occurrence of these problems to age and methyl-CpG-binding protein 2 (MECP2) gene status.Methods:We d

Journal ArticleDOI
TL;DR: MII-pH recording is a promising procedure needing further validation and development to increase its additional benefit over conventional investigation techniques.
Abstract: Aim: The aim of the study was to provide an updated position statement from the ESPGHAN European Pediatric Impedance Working Group on different technical aspects such as indications, methodology, and interpretation of multichannel intraluminal impedance-pH monitoring (MII-pH). Methods: Evidence was used where available, but the article is based mainly on expert opinion and consensus. Results: MII-pH provides more information than simple pH monitoring because reflux detection is not limited to acid reflux. Different companies provide commercialized MII-pH recording systems, making the method widely available and useable in daily clinical practice; however, the technique still has limitations: high cost, limited additional value regarding therapeutic implications, and lack of evidence-based parameters for the assessment of gastroesophageal reflux and symptom association in children. Conclusions: MII-pH recording is a promising procedure needing further validation and development to increase its additional benefit over conventional investigation techniques. The added value of the technique regards mainly clinical circumstances in which nonacid or weakly acid reflux may be relevant such as persisting symptoms during antireflux treatment with proton pump inhibitors and feeding-related reflux; and assessing specific discontinuous symptoms thought to be associated with gastroesophageal reflux; and research.

Journal ArticleDOI
TL;DR: Elimination of cow's milk–induced clinical and histological remission in 65% (95% confidence interval 42%–88%) of children with EoE in whom it was attempted.
Abstract: Objectives:Cow's-milk protein is one of the food antigens responsible for causing eosinophilic esophageal inflammation in a majority of children. We describe our experience with treating eosinophilic esophagitis (EoE) in children by eliminating only cow's milk from their diets.Methods:This r

Journal ArticleDOI
TL;DR: It was not possible to detect any beneficial effect of the probiotic intervention with Ls-33 on inflammatory markers or parameters related to the MS in adolescents with obesity being in a state of low-grade systemic inflammation.
Abstract: Objectives:The connections between gut microbiota, energy homeostasis, and inflammation and its role in the pathogenesis of obesity-related disorders are increasingly recognized. We aimed to investigate the effect of the probiotic strain Lactobacillus salivarius Ls-33 on a series of biomarke

Journal ArticleDOI
TL;DR: In children with NAFLD, reported sugar-sweetened beverage consumption is low; however, uric acid, which may reflect total fructose consumption, was significantly associated with NASH and should be further evaluated.
Abstract: OBJECTIVES Nonalcoholic fatty liver disease (NAFLD) is the most common chronic liver disease in children in the United States. Although changes in diet are often recommended to improve NAFLD, little is known regarding the influence of diet on histologic features of the disease. SUBJECTS AND METHODS This was a prospective, cross-sectional registry-based study. Children (n = 149) enrolled in the multicenter nonalcoholic steatohepatitis (NASH) Clinical Research Network had demographic, anthropometric, clinical, laboratory, and histology data obtained, including the Block Brief Food Questionnaire. Subjects were grouped by presence or absence of steatohepatitis and grades of histologic features according to NASH Clinical Research Network criteria. RESULTS No significant differences were found between children with steatosis compared with steatohepatitis for fraction of energy from fat, carbohydrates, and protein. Sugar-sweetened beverage consumption was low and did not correlate with histologic features, although uric acid, a surrogate marker for fructose intake, was significantly increased in those with definite NASH (P = 0.008). For all groups, vitamin E consumption was insufficient compared with the recommended daily allowance. Median consumption of vitamin E was lower in children with higher grade of steatosis (8.4 vs 6.1 vs 6.9 for grades I, II, and III, respectively, P = 0.05). Those consuming less vitamin C had increased ballooning degeneration (P = 0.05). CONCLUSIONS Children with NAFLD have a diet that is insufficient in vitamin E and this may contribute to the pathophysiology of NAFLD. In children with NAFLD, reported sugar-sweetened beverage consumption is low; however, uric acid, which may reflect total fructose consumption, was significantly associated with NASH and should be further evaluated.

Journal ArticleDOI
TL;DR: Patients with positivity of anti-TTG ≥7-fold cutoff, confirmed by positivity to EMA, have a high-degree probability of duodenal damage and may not require the biopsy for final diagnosis.
Abstract: Objectives Positivity of both immunoglobulin A anti-tissue transglutaminase (TTG) and anti-endomysium antibodies (EMA) has a positive predictive value of nearly 100% for celiac disease (CD). The objective of the present study was to evaluate whether patients of any age, with high pretest probability of CD and high titre of anti-TTG and EMA positivity, have a high probability of intestinal damage and may not require the biopsy for final diagnosis. Methods A retrospective analysis of 412 consecutively referred patients, age range 10 months to 72 years, who underwent small-bowel biopsy for suspicion of CD and positivity to both anti-TTG and EMA, was performed at 4 Italian centers. Biopsies were evaluated independently by 2 pathologists using Marsh modified classification; in cases of dissimilar results, a third pathologist examined the biopsy. The final histological finding diagnosis was expressed as the prevalent or highest score assigned by the pathologist board. Results Three hundred ninety-six patients (96.1%) had histological findings consistent with CD (grade 2 and 3a, 3b, or 3c of modified Marsh classification). An anti-TTG ratio ≥ 7 was able to identify with the 3 assays used (Celikey, anti-TTG immunoglobulin A, EuTTG) all of the patients with significant mucosal damage (Marsh ≥ 2) independent of age and sex; specificity and positive predictive value were 100%. An anti-TTG ratio >20 was more specific (99.8%) for identification of patients with villous atrophy (Marsh 3 a, b, or c). Conclusions Patients with positivity of anti-TTG ≥ 7-fold cutoff, confirmed by positivity to EMA, have a high-degree probability of duodenal damage. In selected conditions, a duodenal biopsy may be avoided and a confirmed greatly positive anti-TTG result could be the basis to prescribe a gluten-free diet.

Journal ArticleDOI
TL;DR: The goal of this article is to educate and assist pediatricians and adult gastroenterology physicians caring for children with IBD and in doing so, help to develop more collaborative care plans between primary care and subspecialty providers.
Abstract: Ulcerative colitis (UC) and Crohn disease (CD), collectively referred to as inflammatory bowel disease (IBD), are chronic inflammatory disorders that can affect the gastrointestinal tract of children and adults. Like other autoimmune processes, the cause(s) of these disorders remain unknown but likely involves some interplay between genetic vulnerability and environmental factors. Children, in particular with UC or CD, can present to their primary care providers with similar symptoms, including abdominal pain, diarrhea, weight loss, and bloody stool. Although UC and CD are more predominant in adults, epidemiologic studies have demonstrated that a significant percentage of these patients were diagnosed during childhood. The chronic nature of the inflammatory process observed in these children and the waxing and waning nature of their clinical symptoms can be especially disruptive to their physical, social, and academic development. As such, physicians caring for children must consider these diseases when evaluating patients with compatible symptoms. Recent research efforts have made available a variety of more specific and effective pharmacologic agents and improved endoscopic and radiologic assessment tools to assist clinicians in the diagnosis and interval assessment of their patients with IBD; however, as the level of complexity of these interventions has increased, so too has the need for practitioners to become familiar with a wider array of treatments and the risks and benefits of particular diagnostic testing. Nonetheless, in most cases, and especially when frequent visits to subspecialty referral centers are not geographically feasible, primary care providers can be active participants in the management of their pediatric patients with IBD. The goal of this article is to educate and assist pediatricians and adult gastroenterology physicians caring for children with IBD, and in doing so, help to develop more collaborative care plans between primary care and subspecialty providers.

Journal ArticleDOI
TL;DR: The quality of diagnostic workup in paediatric patients with IBD increased steadily between 2004 and 2009, and small-bowel imaging by magnetic resonance imaging superseded the use of small-Bowel follow-through.
Abstract: OBJECTIVE In 2005, the Inflammatory Bowel Disease (IBD) Working Group of the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition published consensus guidelines on the diagnostic workup of paediatric IBD, the Porto criteria. According to these guidelines, children suspected of having IBD should undergo an oesophagogastroduodenoscopy (OGD), ileocolonoscopy, and (except in cases of definitive ulcerative colitis) adequate imaging of the small bowel. To audit and evaluate the diagnostic workup of paediatric patients with IBD in Europe, the Working Group created EUROKIDS, a prospective, Web-based registry of newly diagnosed paediatric patients with IBD. METHODS Patients with IBD (ages 0-18 years) were registered in 44 centres in 18 countries. Data on diagnostic workup were analysed according to the year of diagnosis, type of IBD, and centre size. Diagnostic yield of OGD and ileal intubation were evaluated. RESULTS Between 2004 and 2009, 2087 newly diagnosed patients were correctly registered. Both OGD and ileocolonoscopy had been performed in 64% of all of the patients and increased significantly from year 1 (52 %) to 5 (71%, P < 0.001). Small-bowel follow-through use decreased during the years (year 1 n = 213, year 5 n = 108; P < 0.001), whereas magnetic resonance imaging use increased (year 1 n = 25, year 5 n = 171; P < 0.001). Patients diagnosed as having Crohn disease (CD, 59%) and ulcerative colitis (58%) were more likely to have had a complete diagnostic workup than patients diagnosed as having IBD unclassified (45%). In CD, the diagnostic yield of OGD was 7.5% and the yield of ileal intubation was 13%. CONCLUSIONS The quality of diagnostic workup in paediatric patients with IBD increased steadily between 2004 and 2009. Small-bowel imaging by magnetic resonance imaging superseded the use of small-bowel follow-through. OGD and ileal intubation contributed to a definitive diagnosis of CD.

Journal ArticleDOI
TL;DR: Clinical decision making in ARPKD should reflect an understanding of the potential issues emanating from CHF, and accepted pediatric specific approaches to portal hypertension are warranted but must take into consideration the stage of renal insufficiency and potential plans for renal transplantation.
Abstract: Autosomal recessive polycystic kidney disease (ARPKD)/congenital hepatic fibrosis (CHF) is a fibrocystic disease affecting mainly the kidneys and liver. It is characterized by fusiform dilatations of the renal collecting duct and ductal plate malformation of the liver. The incidence is approximately 1:20,000 live births. The majority of cases are caused by mutations in the PKHD1 gene, which encodes for fibrocystin/polycystin. This protein is expressed by the renal and biliary epithelium and is postulated to maintain 3-dimensional tubular architecture (1). ARPKD can be divided into 4 subgroups (2). The first group, “perinatal,” presents in infancy with severe renal disease, congestive heart failure, and pulmonary hypoplasia. The second group, “neonatal,” has progressive renal disease, whereas the third (“infantile”) and fourth (“juvenile”) groups have less severe renal disease and more complications from CHF. CHF typically occurs in ARPKD (3). Two registries have examined the nature and prevalence of liver disease in ARPKD (4,5). CHF can be an isolated lesion (isolated CHF, iCHF) or can occur in ARPKD, Caroli syndrome, and other inherited and noninherited conditions (6). CHF in the setting of ARPKD (and related conditions) can lead to complications that are related primarily to portal hypertension and recurrent cholangitis (1,3). There is growing concern about the role of CHF in the development of sepsis for patients with ARPKD who have undergone renal transplantation (7). There is also the potential risk of development of cholangiocarcinoma in individuals with chronic biliary disease. Because CHF in ARPKD is “curable” via liver transplantation, combined liver-kidney transplantation as a potentially more definitive therapy has been suggested. Clinical decision making in ARPKD/CHF is particularly difficult given the interrelation of the 2 diseased organ systems. The indications for and timing of liver transplantation or nontransplant surgeries such as portosystemic shunting in ARPKD are not clear. A clearer understanding of the natural history of CHF may inform this particularly difficult clinical decision making.

Journal ArticleDOI
TL;DR: Frequent occurrence of IFALD among neonates on PN displays an association to duration of PN and markedly increased serum PS, especially stigmasterol, in comparison to healthy neonates and children on Pn.
Abstract: Objectives:We prospectively evaluated incidence of prolonged (>28 days) parenteral nutrition (PN), associated complications, and significance of parenteral plant sterols (PS) in neonatal intestinal failure–associated liver disease (IFALD) compared with children.Methods:We recruited 28 neonat

Journal ArticleDOI
TL;DR: There are design and administration features of child/adolescent FFQs that should be considered to obtain reliable and valid estimates of dietary intake in this population.
Abstract: Objective: Summarize the validity and reliability of child/adolescent foodfrequency questionnaires (FFQs) that assess food and/or food groups.Methods: We performed a systematic review of child/adolescent (6–18 years) FFQ studies published between January 2001 and December2010 using MEDLINE, Cochrane Library, PsycINFO, and Google Scholar.Main inclusion criteria were peer reviewed, written in English, and reportedreliability or validity of questionnaires that assessed intake of food/foodgroups. Studies were excluded that focused on diseased people or used acombined dietary assessment method. Two authors independently selectedthe articles and extracted questionnaire characteristics such as number ofitems, portion size information, time span, category intake frequencies, andmethodof administration.Validityand reliability coefficients wereextractedand reported for food categories and averaged across food categories foreach study.Results: Twenty-one studies were selected from 873, 18 included validitydata, and 14 included test-retest reliability data. Publications were fromthe United States, Europe, Africa, Brazil, and the south Pacific. Validitycorrelations ranged from 0.01 to 0.80, and reliability correlations rangedfrom 0.05 to 0.88. The highest average validity correlations were obtainedwhen the questionnaire did not assess portion size, measured a shorter timespan (ie, previous day/week), was of medium length(ie, 20–60 items),andwas not administered to the child’s parents.Conclusions: There are design and administration features of child/adolescent FFQs that should be considered to obtain reliable and validestimates of dietary intake in this population.Key Words: nutrition surveys, questionnaires, reliability, validity

Journal ArticleDOI
TL;DR: In conclusion, abnormal gut integrity is associated with reduced linear growth in a population of rural African preschool-age children.
Abstract: The aim of the present study was to investigate the relation of environmental enteropathy, as measured by the dual sugar absorption test, to linear growth faltering in 2- to 5-year-old Malawian children. Dietary quality, food insecurity, anthropometry, and site-specific sugar testing were measured in 418 children, and anthropometry was reassessed 3 months later. A linear regression model predicting linear growth was created. Better growth was associated with less urinary lactulose excretion, more clean water usage, not sleeping with animals, and no previous history of malnutrition. Eighty-seven percent of children studied demonstrated evidence of environmental enteropathy. In conclusion, abnormal gut integrity is associated with reduced linear growth in a population of rural African preschool-age children.

Journal ArticleDOI
TL;DR: Despite a lack of RCTs containing all aspects of effectiveness and safety, the present evidence indicates propofol-based PS to be the best practice for PS in children undergoing GIE.
Abstract: Objective The aim of the study was to assess, by a review of published evidence, the safest and most effective way to provide procedural sedation (PS) in children undergoing gastrointestinal endoscopy (GIE). Methods The databases MEDLINE, Cochrane Library, and Embase were used. Search terms "endoscopy, gastrointestinal" or "endoscopy, digestive system" were combined with "sedation," "conscious sedation," "moderate sedation," "deep sedation," and "hypnotics and sedatives." The final review was restricted to studies reporting specifically on safety (incidences of adverse events) and/or effectiveness (time characteristics, need for supplemental sedation, need for restraint, procedural success, provider satisfaction, and patient comfort) of PS for GIE in children younger than 18 years. Results The search yielded 182 references and the final selection included 11 randomized controlled trials (RCTs) and 15 non-RCTs. Six sedation categories were identified: propofol, opioid/benzodiazepine, premedication, ketamine-, sevoflurane-, and midazolam-based. Only a few RCTs have compared different categories. Opioid/benzodiazepine- and propofol-based PS have a similar safety profile and a low incidence of major adverse events. Propofol-based sedation turned out to be the most effective regimen, with effectiveness comparable to general anesthesia. The addition of midazolam, fentanyl, remifentanil, and/or ketamine to propofol may increase the effectiveness without creating more adverse events. Data on midazolam-, ketamine- and sevoflurane-based sedation were generally too limited to draw conclusions. Conclusions Despite a lack of RCTs containing all aspects of effectiveness and safety, the present evidence indicates propofol-based PS to be the best practice for PS in children undergoing GIE. Propofol can be safely administered by specifically trained nonanesthesiologists.

Journal ArticleDOI
TL;DR: Altered gene expression of TLR2, TLR9, and TOLLIP in small intestinal biopsies in celiac disease suggests that microbiota-associated factors may be important in the development of the disease.
Abstract: Objectives:Less than one-tenth of the carriers of the risk genes HLA-DQ2 or HLA-DQ8 develop celiac disease, suggesting that other genetic and environmental factors are important in the pathogenesis. The role of gut microbiota has been addressed previously with inconsistent findings. Our aim

Journal ArticleDOI
TL;DR: ELBW infants who received early and high IV AA during the first week of life were associated with poor overall growth at 2 years, and Cumulative and single plasma AA concentrations correlated negatively with MDI and postnatal growth.
Abstract: OBJECTIVE The aim of the present study was to examine the effects of early and high intravenous (IV) amino acid (AA) supplementation on growth, health, and neurodevelopment of extremely-low-birth-weight (ELBW) infants throughout their first 2 years of life. METHODS Infants were prospectively randomized in a double-masked fashion and treated for 7 days with either IV AA starting at 0.5 g · kg (-1) · day(-1) and increased by 0.5 g · kg(-1) every day to 3 g · kg(-1)· day(-) or starting at 2 g · kg (-1) · day(-1) of IV AA and advanced by 1 g · kg(-1) every day to 4 g · kg (-1) · day(-1). Plasma AA concentrations were determined by reverse-phase high-performance liquid chromatography. Survivors were longitudinally assessed with Bayley II Scales of Infant Development and physical, social, and global health. RESULTS Forty-three of 51 survivors were studied. Mental Developmental Index (MDI) and Psychomotor Developmental Index were similar between groups; however, the early and high AA group had a lower MDI at 18 months. This difference disappeared at 2 years of age. The early and high AA group z score means for weight, length, and head circumferences were significantly lower than the standard AA group at most visits. Cumulative and single plasma AA concentrations correlated negatively with MDI and postnatal growth. CONCLUSIONS ELBW infants who received early and high IV AA during the first week of life were associated with poor overall growth at 2 years.

Journal ArticleDOI
TL;DR: The authors agree with the Advisory Committee members that PPIs should not be administered to treat the symptoms of GERD in the otherwise healthy infant without the evidence of acid-induced disease.
Abstract: The Food and Drug Administration has completed its review of 4 clinical trials evaluating the use of proton pump inhibitors (PPIs) in infants (ages 1 month to <12 months) for the treatment of gastroesophageal reflux disease (GERD). An Advisory Committee meeting was held in November 2010 to discuss the potential reasons why PPI use in these trials failed to show a benefit in infants with GERD, and directions for future study. The present review summarizes the findings from the clinical trials. Potential mechanisms for the failed clinical trials are discussed. The safety of long-term use is also discussed. As a result of our analysis and review, the authors agree with the Advisory Committee members that PPIs should not be administered to treat the symptoms of GERD in the otherwise healthy infant without the evidence of acid-induced disease.

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TL;DR: Taurolidine line lock was associated with a decreased incidence of catheter-related bloodstream infections, and this finding supports its use in patients with a history of septicemia on treatment with cyclical PN.
Abstract: Objective:The aim of this study was to review the incidence and type of central venous catheter–related bloodstream infection in children on treatment with home parenteral nutrition (PN) before and after the introduction of taurolidine. Taurolidine is a catheter lock solution that prevents b

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TL;DR: Investigating the effect of infant formula with polydextrose and galacto-oligosaccharides on fecal microbiota and secretory IgA found it to produce soft stools and a bifidogenic effect closer to breast milk than formula without PDX/GOS.
Abstract: Objective: The aim of the study was to evaluate the effect of infant formula with polydextrose (PDX) and galacto-oligosaccharides (GOS) on fecal microbiota and secretory IgA (sIgA). Materials and Methods: In the present double-blind, randomized study, term infants received control (Enfamil Lipil) or the same formula with PDX/GOS (4 g/L, 1:1 ratio; PDX/GOS) for 60 days; a reference breast-fed group was included. Formula intake, tolerance, and stool characteristics were collected via electronic diary and analyzed by repeated measures analysis of variance. Anthropometric measurements and stool samples were obtained at baseline and after 30 and 60 days of feeding. Fecal sIgA was measured by enzyme-linked immunosorbent assay and fecal bacteria by fluorescent in situ hybridization and quantitative real-time polymerase chain reaction (qPCR); both were analyzed by Wilcoxon rank sum test. Results: Two hundred thirty infants completed the study. Infants consuming PDX/GOS had softer stools than control at all times (P <0.001). Using qPCR, counts in PDX/GOS were closer to the breast-fed group, tended to be higher than control for total bifidobacteria (P=0.069) and Bifidobacterium longum (P=0.057) at 30 days, and were significantly higher for total bifidobacteria and B longum at 60 days and B infantis at 30 days (P = 0.002). No significant differences were detected between PDX/GOS and control in changes from baseline to 30 or 60 days for sIgA or total bifidobacteria by fluorescent in situ hybridization or qPCR; however, significantly higher changes from baseline were detected between PDX/GOS and control for B infantis at 30 days and B longum at 60 days (P Conclusions: Infant formula with PDX/GOS produces soft stools and a bifidogenic effect closer to breast milk than formula without PDX/GOS.