Publications by authors named "Michael Wilschanski"

118 Publications

Drugs in Focus: Proton Pump Inhibitors.

J Pediatr Gastroenterol Nutr 2021 May;72(5):645-653

Neurogastroenterology and Motility Unit, Department of Paediatric Gastroenterology, Great Ormond Street Hospital NHS Foundation Trust.

Abstract: Proton pump inhibitors (PPIs) are amongst the most commonly prescribed drugs in infants and children with the last decades witnessing a dramatic rise in their utilization. Although PPIs are clearly effective when used appropriately and have been regarded as safe drugs, there is growing evidence regarding their potential adverse effects. Although, largely based on adult data it is clear that many of these are also relevant to pediatrics. PPI use potentially affects gastrointestinal microbiota composition and function, decreases defence against pathogens resulting in increased risk for infections, interferes with absorption of minerals and vitamins leading to specific deficiencies and increased risk for bone fractures as well as interferes with protein digestion resulting in increased risk of sensitization to allergens and development of allergic diseases and eosinophilic esophagitis. An association with gastric, liver and pancreatic cancer has also been inferred from adult data but is tenuous and causation is not proven. Overall, evidence for these adverse events is patchy and not always compelling. Overall, the use of PPIs, for selected indications with a good evidence base, has significant potential benefit but carries more caution in infants and children. Pediatricians should be aware of the concerns regarding the potential adverse events associated with their use.
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http://dx.doi.org/10.1097/MPG.0000000000003063DOI Listing
May 2021

The Use of Fecal Calprotectin Testing in Paediatric Disorders: A Position Paper of the European Society for Paediatric Gastroenterology and Nutrition Gastroenterology Committee.

J Pediatr Gastroenterol Nutr 2021 Apr;72(4):617-640

Neurogastroenterology and Motility, UCL Great Ormond Street Institute of Child Health and Department of Gastroenterology, Great Ormond Street Hospital, London, UK.

Objectives: The aim of the study was to review the evidence regarding the clinical use and value of fecal calprotectin (FC) measurements in different gastrointestinal disorders in children.

Methods: A literature search was conducted in the PubMed, MEDLINE, EMBASE, and Cochrane databases until October 31, 2019. Subtopics were identified and each assigned to individual authors.

Results: A total of 28 recommendations were voted on using the nominal voting technique. Recommendations are given related to sampling, measurement methods, and results interpretation. The 14 authors anonymously voted on each recommendation using a 9-point scale (1 strongly disagree to 9 fully agree). Consensus was considered achieved if at least 75% of the authors voted 6, 7, 8, or 9.

Conclusions: Consensus was reached for all recommendations. Limitations for the use of FC in clinical practice include variability in extraction methodology, performance of test kits as well as the need to establish local reference ranges because of the influence of individual factors, such as age, diet, microbiota, and drugs. The main utility of FC measurement at present is in the diagnosis and monitoring of inflammatory bowel disease (IBD) as well as to differentiate it from functional gastrointestinal disorders (FAPDs). FC, however, has neither utility in the diagnosis of infantile colic nor to differentiate between functional and organic constipation. A rise in FC concentration, may alert to the risk of developing necrotizing enterocolitis and help identifying gastrointestinal involvement in children with Henoch-Schönlein purpura. FC measurement is of little value in Cow's Milk Protein Allergy, coeliac disease (CD), and cystic fibrosis. FC does neither help to distinguish bacterial from viral acute gastroenteritis (AGE), nor to diagnose Helicobacter Pylori infection, small intestinal bacterial overgrowth (SIBO), acute appendicitis (AA), or intestinal polyps.
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http://dx.doi.org/10.1097/MPG.0000000000003046DOI Listing
April 2021

A Green Light for Stop Mutations.

Am J Respir Cell Mol Biol 2021 Mar 11. Epub 2021 Mar 11.

Hebrew University of Jerusalem, Faculty of Medicine, Jerusalem, Israel.

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http://dx.doi.org/10.1165/rcmb.2021-0060EDDOI Listing
March 2021

Management of Gastrointestinal and Nutritional Problems in Children With Neurological Impairment: A Survey of Practice.

J Pediatr Gastroenterol Nutr 2021 Apr;72(4):e97-e101

University Lille, CHU Lille, Inserm U1286 Infinite, Department of pediatric gastroenterology hepatology and nutrition, Lille, France.

Objectives: The main aim of this study was to determine the impact on clinical practice of the first European Society of Gastroenterology, Hepatology, and Nutrition (ESPGHAN) position paper on the diagnosis and management of nutritional and gastrointestinal problems in children with neurological impairment (NI).

Methods: In this pilot-study, a web-based questionnaire was distributed between November, 2019 and June, 2020, amongst ESPGHAN members using the ESPGHAN newsletter. Fifteen questions covered the most relevant aspects on nutritional management and gastrointestinal issues of children with NI. A descriptive analysis of responses was performed.

Results: A total of 150 health professionals from 23 countries responded to the survey. A considerable variation in clinical practice concerning many aspects of nutritional and gastrointestinal management of children with NI was observed. The most frequently used method for diagnosing oropharyngeal dysfunction was the direct observation of meals with or without the use of standardised scores (n = 103). Anthropometric measurements were the most commonly used tools for assessing nutritional status (n = 111). The best treatment for gastroesophageal reflux disease (GERD) was considered to be proton pump inhibitor therapy by most (n = 116) participants. Regarding tube feeding, nearly all respondents (n = 114) agreed that gastrostomy is the best enteral access to be used for long-term enteral feeding. Fundoplication was indicated at the time of gastrostomy placement especially in case of uncontrolled GERD.

Conclusions: More studies are required to address open questions on adequate management of children with NI. Identifying knowledge gaps paves the way for developing updated recommendations and improving patient care.
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http://dx.doi.org/10.1097/MPG.0000000000003020DOI Listing
April 2021

Ivacaftor in People with Cystic Fibrosis and a → or Residual Function Mutation.

Ann Am Thorac Soc 2021 03;18(3):433-441

Vertex Pharmaceuticals (Europe) Ltd., London, United Kingdom.

Ivacaftor's clinical effects in the residual function mutations and warrant further characterization. To evaluate ivacaftor's effect in people with cystic fibrosis aged ≥6 years with or residual function mutations and to explore the correlation between ivacaftor-induced organoid-based cystic fibrosis transmembrane conductance regulator function measurements and clinical response to ivacaftor. Participants were randomized (1:1) in this placebo-controlled crossover study; each treatment sequence included two 8-week treatments with an 8-week washout period. The primary endpoint was absolute change in lung clearance index from baseline through Week 8. Additional endpoints included lung function, patient-reported outcomes, and intestinal organoid-based measurements of ivacaftor-induced cystic fibrosis transmembrane conductance regulator function. Of 38 participants, 37 completed the study. The primary endpoint was met; the Bayesian posterior probability of improvement in lung clearance index with ivacaftor versus placebo was >99%. Additional endpoints improved with ivacaftor. Safety findings were consistent with ivacaftor's known safety profile. Dose-dependent swelling was observed in 23 of 25 viable organoid cultures with ivacaftor treatment. Correlations between ivacaftor-induced organoid swelling and clinical endpoints were negligible to low. In people with cystic fibrosis aged ≥6 years with a or mutation, ivacaftor treatment improved clinical endpoints compared with placebo; however, there was no correlation between organoid swelling and change in clinical endpoints. The organoid assay may assist in identification of ivacaftor-responsive mutations but in this study did not predict magnitude of clinical benefit for individual people with cystic fibrosis with these two mutations.Clinical trial registered with ClinicalTrials.gov (NCT03068312).
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http://dx.doi.org/10.1513/AnnalsATS.202006-659OCDOI Listing
March 2021

Hijab Pin Ingestions.

Pediatrics 2020 06 8;145(6). Epub 2020 May 8.

Divisions of Pediatric Gastroenterology and

Objectives: To characterize the clinical manifestations, outcomes, and complications of hijab pin ingestion in adolescents and to identify risk factors for a need for intervention.

Methods: A retrospective review of patients <25 years of age who presented to our emergency department because of hijab pin ingestion between 2007 and 2018. Comparison was performed between impaled and nonimpaled pins.

Results: We reviewed 1558 foreign-body ingestion cases. Of these, 208 (13.3%) patients presented because of hijab pin ingestion, with a total of 225 ingested pins. The mean patient age was 14.7 ± 4.1 years, and 88% of patients were girls. Time from ingestion to presentation was 24 ± 49.5 hours. Most pins were located in the stomach (46.6%), and 18.6% of all pins were impaled. Location in the stomach (odds ratio = 4.3 [95% confidence interval: 1.9-9.2]; < .001) and abdominal tenderness on examination (odds ratio = 2.7 [95% confidence interval: 1.3-5.6]; = .007) were strong independent risk factors for an impaled pin. Time to intervention was 22.9 hours, and 41 endoscopies were performed. One patient required laparoscopic surgery. No complications were observed.

Conclusions: The hijab pin is an increasingly encountered foreign body in pediatric practice. Its specific clinical features distinguish it from other sharp objects. A delayed interventional approach in selected patients does not carry a higher risk of complications and results in significantly fewer interventions compared to existing guidelines. These findings will help guide pediatric specialists in this prevalent clinical scenario. Management recommendations are proposed.
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http://dx.doi.org/10.1542/peds.2019-3472DOI Listing
June 2020

Clinical and Practice Variations in Pediatric Acute Recurrent or Chronic Pancreatitis: Report From the INSPPIRE Study.

J Pediatr Gastroenterol Nutr 2020 07;71(1):112-118

Department of Pediatrics, University of Nebraska Medical Center, Omaha, NE.

Objective: The aim of the study was to determine whether clinical characteristics and management of pediatric acute recurrent pancreatitis (ARP) and chronic pancreatitis (CP) differ across INSPPIRE (INternational Study Group of Pediatric Pancreatitis: In Search for a cuRE) sites.

Study Design: Data were collected from INSPPIRE and analyzed per US regions and "non-US" sites. Between-group differences were compared by Pearson chi-square test. Differences in disease burden were compared by Kruskal-Wallis test.

Results: Out of the 479 subjects, 121 (25%) were enrolled in West, 151 (32%) Midwest, 45 Northeast (9%), 78 (16%) South, and 84 (18%) at non-US sites. Hispanic ethnicity was more common in South (P < 0.0001); white race in Northeast (P = 0.009). CP was less common and time from diagnosis of first acute pancreatitis to CP was longer in children at non-US sites (P = 0.0002 and P = 0.011, respectively). Genetic mutations were most common among all groups; PRSS1 variants predominated in Midwest (P = 0.002). Gallstones were more frequent in South (P = 0.002). Endoscopic retrograde cholangiopancreatography (ERCP) and computed tomography (CT) imaging were more commonly utilized in United States compared with non-United States (P < 0.0001), but there were no differences in the use of MRI/MRCP. Disease burden was highest in the West and Midwest, possibly as total pancreatectomy and islet autotransplantation (TPIAT) referral sites were located in these regions. All therapies were less commonly administered in non-US sites (P < 0.0001).

Conclusions: This is the first study to describe geographical variations in the INSPPIRE cohort, which possibly reflect variations in practice and referral patterns. The underlying reason behind the lower frequency of CP and fewer treatments in non-United States sites need to be further explored.
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http://dx.doi.org/10.1097/MPG.0000000000002661DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7305964PMC
July 2020

Insights into the variability of nasal potential difference, a biomarker of CFTR activity.

J Cyst Fibros 2020 07 4;19(4):620-626. Epub 2019 Nov 4.

Centre Maladies Rares Mucoviscidose, Hôpital Universitaire Necker-Enfants Malades, Assistance-Publique Hôpitaux de Paris, Paris, France; Université Paris Sorbonne, Paris, France; Institut Necker-Enfants Malades. INSERM U1151, Paris, France. Electronic address:

Background: Nasal potential difference (NPD) is used to evaluate CFTR function in vivo. We aimed to evaluate the intrasubject and intersubject variability of NPD measurements.

Methods: We reviewed NPD tracings of 116 patients with CF enrolled in the placebo arm of a multicenter study. Patients carried at least one nonsense mutation and underwent repeated NPD tests every 16 weeks. NPD parameters included basal potential difference (basal PD), inhibition of sodium absorption by amiloride (Δ Amiloride), chloride (Cl) transport in response to a Cl-free solution (Δ Low Cl), isoproterenol (Δ Isoproterenol), the sum of Δ Low Cl and Δ Isoproterenol (Δ Low Cl-Isoproterenol) and ATP (Δ ATP).

Results: Basal PD and Δ Amiloride displayed the highest variabilities, mainly stemming from intercenter and intrasubject effect. Δ Low Cl, Δ Isoproterenol and Δ Low Cl-Isoproterenol demonstrated a large intrasubject variability but a smaller intersubject variability. The intrasubject measurement variability for Δ Low Cl-Isoproterenol, was within ± 7.2 mV with 95% probability. It was greater in patients reporting ongoing pulmonary exacerbations.

Conclusions: The large intercenter variability of basal PD and Δ Amiloride highlights the operator-dependent aspect of these measurements. A difference greater than 7.2 mV in Δ Low Cl-Isoproterenol in a given patient on CFTR modulator can be attributed, with 95% probability, to a treatment effect rather than to the variability inherent in the measurement.
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http://dx.doi.org/10.1016/j.jcf.2019.09.015DOI Listing
July 2020

Diabetes Mellitus in Children with Acute Recurrent and Chronic Pancreatitis: Data From the INternational Study Group of Pediatric Pancreatitis: In Search for a CuRE Cohort.

J Pediatr Gastroenterol Nutr 2019 11;69(5):599-606

University of Iowa, Iowa City, IA.

Objectives: Adults with chronic pancreatitis (CP) have a high risk for developing pancreatogenic diabetes mellitus (DM), but little is known regarding potential risk factors for DM in children with acute recurrent pancreatitis (ARP) or CP. We compared demographic and clinical features of children with ARP or CP, with and without DM, in the INternational Study Group of Pediatric Pancreatitis: In Search for a CuRE (INSPPIRE) registry.

Methods: We reviewed the INSPPIRE database for the presence or absence of physician-diagnosed DM in 397 children, excluding those with total pancreatectomy with islet autotransplantation, enrolled from August 2012 to August 2017. Patient demographics, BMI percentile, age at disease onset, disease risk factors, disease burden, and treatments were compared between children with DM (n = 24) and without DM (n = 373).

Results: Twenty-four children (6% of the cohort) had a diagnosis of DM. Five of 13 tested were positive for beta cell autoantibodies. The DM group was 4.2 years [95% confidence interval (CI) 3-5.4] older at first episode of acute pancreatitis, and tended to more often have hypertriglyceridemia [odds ratio (OR) 5.21 (1.33-17.05)], coexisting autoimmune disease [OR 3.94 (0.88-13.65)] or pancreatic atrophy [OR 3.64 (1.13, 11.59)].

Conclusion: Pancreatic atrophy may be more common among children with DM, suggesting more advanced exocrine disease. However, data in this exploratory cohort also suggest increased autoimmunity and hypertriglyceridemia in children with DM, suggesting that risk factors for type 1 and type 2 DM, respectively may play a role in mediating DM development in children with pancreatitis.
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http://dx.doi.org/10.1097/MPG.0000000000002482DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6834233PMC
November 2019

Factors Associated With Frequent Opioid Use in Children With Acute Recurrent and Chronic Pancreatitis.

J Pediatr Gastroenterol Nutr 2020 01;70(1):106-114

Stead Family Department of Pediatrics, University of Iowa, Iowa City, IA.

Objectives: The aim of the study was to understand the association of frequent opioid use with disease phenotype and pain pattern and burden in children and adolescents with acute recurrent (ARP) or chronic pancreatitis (CP).

Methods: Cross-sectional study of children <19 years with ARP or CP, at enrollment into the INSPPIRE cohort. We categorized patients as opioid "frequent use" (daily/weekly) or "nonfrequent use" (monthly or less, or no opioids), based on patient and parent self-report.

Results: Of 427 children with ARP or CP, 17% reported frequent opioid use. More children with CP (65%) reported frequent opioid use than with ARP (41%, P = 0.0002). In multivariate analysis, frequent opioid use was associated with older age at diagnosis (odds ratio [OR] 1.67 per 5 years, 95% confidence interval [CI] 1.13-2.47, P = 0.01), exocrine insufficiency (OR 2.44, 95% CI 1.13-5.24, P = 0.02), constant/severe pain (OR 4.14, 95% CI 2.06-8.34, P < 0.0001), and higher average pain impact score across all 6 functional domains (OR 1.62 per 1-point increase, 95% CI 1.28-2.06, P < 0.0001). Children with frequent opioid use also reported more missed school days, hospitalizations, and emergency room visits in the past year than children with no frequent use (P < 0.0002 for each). Participants in the US West and Midwest accounted for 83% of frequent opioid users but only 56% of the total cohort.

Conclusions: In children with CP or ARP, frequent opioid use is associated with constant pain, more healthcare use, and higher levels of pain interference with functioning. Longitudinal and prospective research is needed to identify risk factors for frequent opioid use and to evaluate nonopioid interventions for reducing pain and disability in these children.
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http://dx.doi.org/10.1097/MPG.0000000000002502DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6934913PMC
January 2020

Prevention of malnutrition in cystic fibrosis.

Curr Opin Pulm Med 2019 11;25(6):674-679

Department of Pediatrics, Pediatric Gastroenterology, Hepatology and Nutrition, Hadassah Medical Center, Jerusalem, Israel.

Purpose Of Review: Malnutrition is one of the major burdens of disease in cystic fibrosis. The prevention of malnutrition remains a priority throughout the life of a patient with cystic fibrosis. Literature and guidelines on the management of nutrition in cystic fibrosis have been published; however, here we review updated findings in cystic fibrosis nutrition as well as the role of novel treatments.

Recent Findings: We review the latest studies on the importance and consequences of nutrition in cystic fibrosis. Novel findings on specific nutrients such as vitamin D and sodium can improve our care and thereby health and growth outcomes. The role of exercise has been further studied. In the field of new treatments, we review the role of cystic fibrosis transmembrane-conductance regulator potentiators and modulators in cystic fibrosis nutrition. A new feeding tube fat-digesting device has been developed and shows promise in cystic fibrosis enteral nutrition.

Summary: Advances in the nutritional care of cystic fibrosis are forming and believed to further develop in the near future, adding to the recent progress in cystic fibrosis patients' health, survival, and quality of life.
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http://dx.doi.org/10.1097/MCP.0000000000000629DOI Listing
November 2019

Nasal potential difference in suspected cystic fibrosis patients with 5T polymorphism.

J Cyst Fibros 2020 07 19;19(4):627-631. Epub 2019 Jul 19.

Department of Pulmonology, University Medical Center Utrecht, Postbus 85500, 3508, GA, Utrecht, the Netherlands.

Background: 5T polymorphism is a CFTR mutation with unclear clinical consequences: the phenotype varies from healthy individuals to Cystic Fibrosis (CF). The aim of this study was to evaluate if nasal potential difference (NPD) and sweat testing correlate with symptoms and CF diagnosis in 5T patients.

Methods: 86 patients with 5T who had undergone NPD measurement, were included (6 homozygous (5T/5T), 41 with a PI-CF causing mutation in trans (5T/PI-CF), 11 with a PS-CF causing mutation in trans (5T/PS-CF) and 28 without a known mutation in trans (5T/?). Data including age, phenotype, sweat chloride and follow up were collected.

Results: 33% of the 5T/5T patients had abnormal NPD results, compared to 70% in 5T/PI-CF; 33% in 5T/PS-CF and 29% in 5T/?. The percentage of high or borderline sweat chloride was highest in 5T/PI-CF, and 5T/?, compared to 5T/5T and 5T/PS-CF (91, 96, 80, and 63%, respectively). TGm (number of TG repeats in intron 8) analysis was performed in 21 5T/PI-CF patients. TG11 was associated with lower sweat chloride, lower percentage of abnormal NPD and less progression of symptoms compared to TG12 and TG13.

Conclusion: There is much variation in clinical status among 5T patients. All patients in this study with 5T/PS CF, all patients with both normal NPD and sweat test, and most patients with TG11 were stable or improving over time. Therefore, NPD measurement and TGm status aid to assess if a patient is at high risk for developing CF or CFTR-related disease and if specific follow up in a CF center is required.
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http://dx.doi.org/10.1016/j.jcf.2019.07.001DOI Listing
July 2020

Initial Development and Validation of a Transition Readiness Scale for Adolescents with Inflammatory Bowel Disease.

Gastroenterol Res Pract 2019 18;2019:5062105. Epub 2019 Jun 18.

Institute of Gastroenterology and Liver Diseases, Hadassah-Hebrew University Medical Center, POB 12000, Jerusalem 91120, Israel.

Background And Aims: To date, there are no validated measures in IBD to assess the level of preparedness for transition into adult health care. The purpose of this study was to develop and assess the reliability and validity of a "Transition Readiness" (TR) measure for adolescents with IBD, as well as to evaluate the level of TR synchronicity between adolescents themselves, their parents, and their pediatric gastroenterologists.

Methods: A self-assessment tool was created to evaluate TR. Items were reviewed for face validation by IBD experts, and an exploratory factor analysis was performed which yielded 3 distinct domains. The study cohort included adolescents aged 12-21 yrs, their parents, and their physicians in pediatric IBD centers. Correlations between patient/parent/physician TR between each of the domains and the overall TR score to age were assessed.

Results: 63 subjects (average age 16.6 yrs/79% Crohn's disease/44% male) participated in this study. There was a significant correlation between the scoring of adolescents and parents on all three domains. The correlation between adolescents and physicians, as well as between parents and physicians, was only consistent for self-efficacy. Self-efficacy significantly correlated with age, while the correlations between perceived knowledge and perception of medical care with age were not significant.

Conclusion: Validation of a novel TR measurement for adolescents with IBD demonstrated a good correlation between patients and parents. Out of the three proposed constructs, perceived self-efficacy is the most salient measure.
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http://dx.doi.org/10.1155/2019/5062105DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6604282PMC
June 2019

The Use of Jejunal Tube Feeding in Children: A Position Paper by the Gastroenterology and Nutrition Committees of the European Society for Paediatric Gastroenterology, Hepatology, and Nutrition 2019.

J Pediatr Gastroenterol Nutr 2019 08;69(2):239-258

Neurogastroenterology and Motility Unit, Department of Pediatric Gastroenterology, Great Ormond Street Hospital NHS Foundation Trust, London, UK.

Objectives: Jejunal tube feeding (JTF) is increasingly becoming the standard of care for children in whom gastric tube feeding is insufficient to achieve caloric needs. Given a lack of a systematic approach to the care of JTF in paediatric patients, the aim of this position paper is to provide expert guidance regarding the indications for its use and practical considerations to optimize its utility and safety.

Methods: A group of members of the Gastroenterology and Nutrition Committees of the European Society of Paediatric Gastroenterology Hepatology and Nutrition and of invited experts in the field was formed in September 2016 to produce this clinical guide. Seventeen clinical questions treating indications and contraindications, investigations before placement, techniques of placement, suitable feeds and feeding regimen, weaning from JTF, complications, long-term care, and ethical considerations were addressed.A systematic literature search was performed from 1982 to November 2018 using PubMed, the MEDLINE, and Cochrane Database of Systematic Reviews. Grading of Recommendations, Assessment, Development, and Evaluation was applied to evaluate the outcomes.During a consensus meeting, all recommendations were discussed and finalized. In the absence of evidence from randomized controlled trials, recommendations reflect the expert opinion of the authors.

Results: A total of 33 recommendations were voted on using the nominal voting technique.

Conclusions: JTF is a safe and effective means of enteral feeding when gastric feeding is insufficient to meet caloric needs or is not possible. The decision to place a jejunal tube has to be made by close cooperation of a multidisciplinary team providing active follow-up and care.
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http://dx.doi.org/10.1097/MPG.0000000000002379DOI Listing
August 2019

Risk Factors for Rapid Progression From Acute Recurrent to Chronic Pancreatitis in Children: Report From INSPPIRE.

J Pediatr Gastroenterol Nutr 2019 08;69(2):206-211

University of Iowa Stead Family Children's Hospital, Iowa City, IA.

Objective: The aim of the study was to determine the rate of progression from acute recurrent pancreatitis (ARP) to chronic pancreatitis (CP) in children and assess risk factors.

Study Design: Data were collected from the INternational Study group of Pediatric Pancreatitis: In search for a cuRE (INSPPIRE) cohort. Kaplan-Meier curves were constructed to calculate duration of progression from initial attack of acute pancreatitis (AP) to CP. Log-rank test was used to compare survival (nonprogression) probability distribution between groups. Cox proportional hazard regression models were fitted to obtain hazard ratio (with 95% confidence interval [CI]) of progression for each risk variable.

Results: Of 442 children, 251 had ARP and 191 had CP. The median time of progression from initial attack of AP to CP was 3.79 years. The progression was faster in those ages 6 years or older at the first episode of AP compared to those younger than 6 years (median time to CP: 2.91 vs 4.92 years; P = 0.01). Children with pathogenic PRSS1 variants progressed more rapidly to CP compared to children without PRSS1 variants (median time to CP: 2.52 vs 4.48 years; P = 0.003). Within 6 years after the initial AP attack, cumulative proportion with exocrine pancreatic insufficiency was 18.0% (95% CI: 12.4%, 25.6%); diabetes mellitus was 7.7% (95% CI: 4.2%, 14.1%).

Conclusions: Children with ARP rapidly progress to CP, exocrine pancreatic insufficiency, and diabetes. The progression to CP is faster in children who were 6 years or older at the first episode of AP or with pathogenic PRSS1 variants. The factors that affect the aggressive disease course in childhood warrant further investigation.
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http://dx.doi.org/10.1097/MPG.0000000000002405DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6699635PMC
August 2019

Chronic Pancreatitis: Pediatric and Adult Cohorts Show Similarities in Disease Progress Despite Different Risk Factors.

J Pediatr Gastroenterol Nutr 2019 04;68(4):566-573

Department of Medicine.

Objectives: The aim of the present study was to investigate the natural history of chronic pancreatitis (CP); patients in the North American Pancreatitis Study2 (NAPS2, adults) and INternational Study group of Pediatric Pancreatitis: In search for a cuRE (INSPPIRE, pediatric) were compared.

Methods: Demographics, risk factors, disease duration, management and outcomes of 224 children and 1063 adults were compared using appropriate statistical tests for categorical and continuous variables.

Results: Alcohol was a risk in 53% of adults and 1% of children (P < 0.0001); tobacco in 50% of adults and 7% of children (P < 0.0001). Obstructive factors were more common in children (29% vs 19% in adults, P = 0.001). Genetic risk factors were found more often in children. Exocrine pancreatic insufficiency was similar (children 26% vs adult 33%, P = 0.107). Diabetes was more common in adults than children (36% vs 4% respectively, P < 0.0001). Median emergency room visits, hospitalizations, and missed days of work/school were similar across the cohorts. As a secondary analysis, NAPS2 subjects with childhood onset (NAPS2-CO) were compared with INSPPIRE subjects. These 2 cohorts were more similar than the total INSPPIRE and NAPS2 cohorts, including for genetic risk factors. The only risk factor significantly more common in the NAPS2-CO cohort compared with the INSPPIRE cohort was alcohol (9% NAPS2-CO vs 1% INSPPIRE cohorts, P = 0.011).

Conclusions: Despite disparity in age of onset, children and adults with CP exhibit similarity in demographics, CP treatment, and pain. Differences between groups in radiographic findings and diabetes prevalence may be related to differences in risk factors associated with disease and length of time of CP.
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http://dx.doi.org/10.1097/MPG.0000000000002279DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6492264PMC
April 2019

Celiac Disease and Celiac Antibodies in DM1 Patients: When Are Screening and Biopsy Recommended?

Dig Dis Sci 2019 02 30;64(2):487-492. Epub 2018 Oct 30.

Faculty of Medicine, Hebrew University Medical School, Jerusalem, Israel.

Introduction: The prevalence of celiac disease (CD) is increased in diabetes mellitus type 1 (DM1) patients. In most cases, CD is diagnosed in asymptomatic patients and hence periodic screening tests are recommended, but the timing, frequency of tests and indication for duodenal biopsy is unclear. The purpose of this study was to investigate the dynamics of CD serology in DM1 and identify risk factors for CD.

Methods: Celiac serology and duodenal biopsy results from 1990 until 2015 were collected from patients with DM1. The outcome of positive celiac serology, the incidence and risk factors for CD in DM1 patients were investigated.

Results: A total of 314 DM1 patients who had celiac serology were identified, with follow-up period up to 23 years. Of 31 patients (9.9%) with positive celiac serology, 11(35.4%) had spontaneous normalization after various time periods. Eighteen patients were diagnosed with CD (58.1% of positive celiac serology, 5.73% of the study cohort). Age under 4.5 years was a risk factor for CD, but not family background of autoimmune diseases or gender. All patients with CD diagnosis were diagnosed during the first 6 years following DM1 diagnosis.

Conclusion: Screening asymptomatic DM1 patients for CD beyond 6 years after diagnosis is not recommended. Spontaneous normalization of CD serology occurs, and hence, serologic follow-up may be performed. In children with DM1 diagnosis under the age of 4.5 years or with positive CD serology at DM1 diagnosis, there is an increased risk for CD and therefore positive serology should lead to biopsy.
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http://dx.doi.org/10.1007/s10620-018-5353-4DOI Listing
February 2019

INternational Study Group of Pediatric Pancreatitis: In Search for a CuRE Cohort Study: Design and Rationale for INSPPIRE 2 From the Consortium for the Study of Chronic Pancreatitis, Diabetes, and Pancreatic Cancer.

Pancreas 2018 Nov/Dec;47(10):1222-1228

Department of Pediatrics, Washington University School of Medicine, St Louis, MO.

We created the INternational Study Group of Pediatric Pancreatitis: In Search for a CuRE (INSPPIRE 2) cohort to study the risk factors, natural history, and outcomes of pediatric acute recurrent pancreatitis and chronic pancreatitis (CP). Patient and physician questionnaires collect information on demographics, clinical history, family and social history, and disease outcomes. Health-related quality of life, depression, and anxiety are measured using validated questionnaires. Information entered on paper questionnaires is transferred into a database managed by Consortium for the Study of Chronic Pancreatitis, Diabetes, and Pancreatic Cancer's Coordinating and Data Management Center. Biosamples are collected for DNA isolation and analysis of most common pancreatitis-associated genes.Twenty-two sites (18 in the United States, 2 in Canada, and 1 each in Israel and Australia) are participating in the INSPPIRE 2 study. These sites have enrolled 211 subjects into the INSPPIRE 2 database toward our goal to recruit more than 800 patients in 2 years. The INSPPIRE 2 cohort study is an extension of the INSPPIRE cohort study with a larger and more diverse patient population. Our goals have expanded to include evaluating risk factors for CP, its sequelae, and psychosocial factors associated with pediatric acute recurrent pancreatitis and CP.
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http://dx.doi.org/10.1097/MPA.0000000000001172DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6195325PMC
March 2019

Standardized Measurement of Nasal Membrane Transepithelial Potential Difference (NPD).

J Vis Exp 2018 09 13(139). Epub 2018 Sep 13.

Service de Pneumologie et Allergologie Pédiatriques and Center de Ressources et de Compétence de la Mucoviscidose, Hôpital Necker Enfants Malades; INSERM U 1151, Institut Necker Enfants Malades;

We describe a standardized measurement of nasal potential difference (NPD). In this technique, cystic fibrosis transmembrane conductance regulator (CFTR) and the epithelial sodium channel (ENaC) function are monitored by the change in voltage across the nasal epithelium after the superfusion of solutions that modify ion channel activity. This is enabled by the measurement of the potential difference between the subcutaneous compartment and the airway epithelium in the nostril, utilizing a catheter in contact with the inferior nasal turbinate. The test allows the measurement of the stable baseline voltage and the successive net voltage changes after perfusion of 100 µM amiloride, an inhibitor of Na reabsorption in Ringer's solution; a chloride-free solution containing amiloride to drive chloride secretion and 10 µM isoproterenol in a chloride-free solution with amiloride to stimulate the cyclic adenosine monophosphate (cAMP)-dependent chloride conductance related to CFTR. This technique has the advantage of demonstrating the electrophysiological properties of two key components establishing the hydration of the airway surface liquid of the respiratory epithelium, ENaC, and CFTR. Therefore, it is a useful research tool for phase 2 and proof of concept trials of agents that target CFTR and ENaC activity for the treatment of cystic fibrosis (CF) lung disease. It is also a key follow-up procedure to establish CFTR dysfunction when genetic testing and sweat testing are equivocal. Unlike sweat chloride, the test is relatively more time consuming and costly. It also requires operator training and expertise to conduct the test effectively. Inter- and intra-subject variability has been reported in this technique especially in young or uncooperative subjects. To assist with this concern, interpretation has been improved through a recently validated algorithm.
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http://dx.doi.org/10.3791/57006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6235174PMC
September 2018

Probiotics in cystic fibrosis patients: A double blind crossover placebo controlled study: Pilot study from the ESPGHAN Working Group on Pancreas/CF.

Clin Nutr ESPEN 2018 10 20;27:59-65. Epub 2018 Jul 20.

Hadassah University Hospitals, Pediatric Gastroenterology, CF Centre, Jerusalem, Israel.

Background: A potential positive effect of probiotics in cystic fibrosis (CF) on fecal calprotectin (FCP), pulmonary exacerbations and weight has been described in small controlled trials.

Methods: A double-blind multicenter cross-over study (2 × 4 m) was performed looking at abdominal pain, nutritional status, pulmonary function, pulmonary exacerbation, FCP and lactulose/mannitol gut permeability test. Patients kept a diary with daily scoring of abdominal pain, stool frequency and consistency as well as treatment changes.

Results: 31 CF patients entered the study of which 25 finished it. At start patients aged 9.3yrs (6.9-12.2), had a median BMI z-score of -0.5 (-1.5-0.08), height z-score of -0.4 (-1.1-0.05) and FEV1% of 100% (87.2-106.6). Median FCP at start was 61 μg/g (17-108) and gut permeability 0.079 (0.051-0.122). No significant changes were observed in the clinical parameters (BMI, FEV1%, abdominal pain, exacerbations). Despite being frequently abnormal (17/28 (61%) >50 mg/kg), FCP did not change significantly with probiotics. The proportion of patients with normal permeability was 8% during placebo and 32% during probiotic treatment (p = 0.031). FCP correlated to BMI z-score (p = 0.043) and gut permeability to abdominal pain (p = 0.015). The microbiome revealed a high predominance of Actinobacteria and Proteobacteriae. Probiotic supplementation did not result in a shift at the phylum nor at phylogenetic level.

Conclusion: Normalization of gut permeability was observed in 13% of patients during probiotic treatment. However, none of the previously described effects could be confirmed.
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http://dx.doi.org/10.1016/j.clnesp.2018.06.008DOI Listing
October 2018

Increasing Vitamin D Serum Levels Is Associated With Reduced Pulmonary Exacerbations in Patients With Cystic Fibrosis.

J Pediatr Gastroenterol Nutr 2019 01;68(1):110-115

CF Center, Hadassah-University Medical Center, Jerusalem, Israel.

Context: In 2012, The North American Cystic Fibrosis Foundation (NACFF) published new guidelines for the treatment of vitamin D deficiency in individuals with cystic fibrosis (CF).

Objective: The objectives of our study were to assess the efficacy of these guidelines, and to test the effect of increasing vitamin D dosage on pulmonary function and exacerbations.

Design: Pulmonary function tests and serum concentrations of 25-hydroxyvitamin D [25(OH)D] were measured 1 year before increasing vitamin D dosage according to the guidelines and at least 1 year later. In addition, days of hospitalization and pulmonary exacerbations were counted and an average per year (average number of days of hospitalization and average number of pulmonary exacerbations [PEA], respectively) was calculated.

Setting And Participants: A total of 90 patients from The Cystic Fibrosis Clinic at Hadassah Mount-Scopus Hospital, Jerusalem, Israel.

Results: The mean serum concentration of vitamin D increased significantly from 20.97 ng/mL (52.34 nmol/L) at baseline to 25.41 ng/mL (63.42 nmol/L) at the end of follow-up (P < 0.001). The number of PEA decreased significantly from 2.79 ± 3.96 to 2.15 ± 2.91 (P = 0.007). The change in vitamin D levels was correlated with a decrease in PEA (correlation coefficient = -0.318, P = 0.002).

Conclusions: The NACFF guidelines for management of vitamin D deficiency improve vitamin D levels in patients with CF but did not reach the normal values in most patients. The increase in vitamin D serum levels was, however, associated with a decrease in number of pulmonary exacerbations.
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http://dx.doi.org/10.1097/MPG.0000000000002126DOI Listing
January 2019

Impact of Obesity on Pediatric Acute Recurrent and Chronic Pancreatitis.

Pancreas 2018 09;47(8):967-973

Department of Pediatrics, Washington University School of Medicine, St Louis, MO.

Objective: The aim of this study was to assess the impact of obesity on pediatric acute recurrent pancreatitis or chronic pancreatitis (CP).

Methods: We determined body mass index (BMI) status at enrollment in INSPPIRE (INternational Study group of Pediatric Pancreatitis: In search for a cuRE) cohort using CDC criteria for pediatric-specific BMI percentiles. We used the Cochran-Armitage test to assess trends and the Jonckheere-Terpstra test to determine associations.

Results: Of 446 subjects (acute recurrent pancreatitis, n = 241; CP, n = 205), 22 were underweight, 258 normal weight, 75 overweight, and 91 were obese. The BMI groups were similar in sex, race, and age at presentation. Hypertriglyceridemia was more common in overweight or obese. Obese children were less likely to have CP and more likely to have acute inflammation on imaging. Compared with children with normal weight, obese or overweight children were older at first acute pancreatitis episode and diagnosed with CP at an older age. Obese or overweight children were less likely to undergo medical or endoscopic treatment, develop exocrine pancreatic insufficiency, and require total pancreatectomy with islet autotransplantation. Diabetes was similar among all groups.

Conclusions: Obesity or overweight seems to delay the initial acute pancreatitis episode and diagnosis of CP compared with normal weight or underweight. The impact of obesity on pediatric CP progression and severity deserves further study.
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http://dx.doi.org/10.1097/MPA.0000000000001120DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6095802PMC
September 2018

Nutritional Considerations in Pediatric Pancreatitis: A Position Paper from the NASPGHAN Pancreas Committee and ESPGHAN Cystic Fibrosis/Pancreas Working Group.

J Pediatr Gastroenterol Nutr 2018 07;67(1):131-143

Division of Pediatric Gastroenterology and Nutrition, Montreal Children's Hospital, McGill University Health Centre, Montreal, Quebec, Canada.

Objectives: Wide variations exist in how physicians manage the nutritional aspects of children affected by acute pancreatitis (AP), acute recurrent pancreatitis (ARP), and chronic (CP) pancreatitis. Better consensus for optimal management is needed.

Methods: This consensus statement on nutrition in pediatric pancreatic diseases was developed through a joint ESPGHAN-NASPGHAN working group that performed an evidence-based search of the literature on nutrition in AP, ARP, and CP with a focus on pediatrics. The literature was summarized, quality of evidence reviewed, and expert recommendations developed. The authorship met to discuss the evidence and statements. Voting on recommendations occurred over 2 rounds based on feedback. A consensus of at least 75% was required to approve a recommendation. Areas requiring further research were identified.

Results And Discussion: The literature on nutrition in pediatric pancreatitis is limited. Children with mild AP benefit from starting an early nutritional regimen in the course of the attack. Early nutrition should be attempted in severe AP when possible; enteral nutrition is preferred over parenteral nutrition. Children with ARP are likely to tolerate and benefit from a regular diet. Children with CP need ongoing assessment for growth and nutritional deficiencies, exocrine and endocrine insufficiencies.

Conclusions: This document presents the first authoritative recommendations on nutritional considerations in pediatric pancreatitis. Future research should address the gaps in knowledge particularly relating to optimal nutrition for AP in children, role of diet or dietary supplements on recurrent attacks of pancreatitis and pain episodes, monitoring practices to detect early growth and nutritional deficiencies in CP and identifying risk factors that predispose children to these deficiencies.
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http://dx.doi.org/10.1097/MPG.0000000000002023DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6020697PMC
July 2018

Pancreas Divisum in Pediatric Acute Recurrent and Chronic Pancreatitis: Report From INSPPIRE.

J Clin Gastroenterol 2019 07;53(6):e232-e238

Department of Pediatrics, Stead Family Children's Hospital, University of Iowa, Iowa City, IA.

Introduction: The significance of pancreas divisum (PD) as a risk factor for pancreatitis is controversial. We analyzed the characteristics of children with PD associated with acute recurrent or chronic pancreatitis to better understand its impact.

Patients And Methods: We compared children with or without PD in the well-phenotyped INSPPIRE (INternational Study group of Pediatric Pancreatitis: In search for a cuRE) cohort. Differences were analyzed using 2-sample t test or Wilcoxon rank sum test for continuous variables, Pearson χ or Fisher exact test for categorical variables.

Results: PD was found in 52 of 359 (14.5%) subjects, a higher prevalence than the general population (∼7%). Females more commonly had PD (71% vs. 55%; P=0.02). Children with PD did not have a higher incidence of mutations in SPINK1, CFTR, CTRC compared with children with no PD. Children with PD were less likely to have PRSS1 mutations (10% vs. 34%; P<0.01) or a family history of pancreatitis (P<0.05), and more likely to have hypertriglyceridemia (11% vs. 3%; P=0.03). Children with PD underwent significantly more endoscopic procedures and pancreatic sphincterotomy. Patients with PD had fewer attacks of acute pancreatitis (P=0.03) and were less likely to develop exocrine pancreatic insufficiency (P=0.01). Therapeutic endoscopic retrograde cholangiopancreatography was considered most helpful if pancreatic duct was impacted with stones (83% helpful).

Conclusions: PD is likely a risk factor for acute recurrent pancreatitis and chronic pancreatitis in children that appears to act independently of genetic risk factors. Patients with PD and stones obstructing the pancreatic duct benefit most from therapeutic endoscopic retrograde cholangiopancreatography.
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http://dx.doi.org/10.1097/MCG.0000000000001063DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6275137PMC
July 2019

Primary sclerosing cholangitis is associated with abnormalities in CFTR.

J Cyst Fibros 2018 09 26;17(5):666-671. Epub 2018 May 26.

Laboratory of Molecular Genetics and Histocompatibility, University Hospital of Brest, Institut National de la Santé et de la Recherche Médicale, U1078 Brest, France.

Background: The etiology of primary sclerosing cholangitis (PSC) is unknown. PSC and Cystic Fibrosis related liver disease have common features: chronic inflammation, biliary damage and similar cholangiographic findings. It is unknown whether or not PSC is related to cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction. We hypothesize that a sub-group of PSC patients may be a "single-organ" presentation of CF.

Methods: Patients with PSC underwent nasal potential difference (NPD) measurement, sweat chloride measurement and complete CFTR sequencing by new generation sequencing.

Results: 6/32 patients aged 46 ± 13 yrs. had CFTR causing mutations on one allele and 19 had CFTR polymorphisms; 6/23 tested had abnormal and 21 had intermediate sweat tests; 4/32 patients had abnormal NPD. One patient had chronic pancreatitis and was infertile.

Conclusions: 19% of PSC patients had features of CFTR related disorder, 19% carry CFTR mutations and 50% had CFTR polymorphisms. In some patients, PSC may be a single organ presentation of CF or a CFTR-related disorder.
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http://dx.doi.org/10.1016/j.jcf.2018.04.005DOI Listing
September 2018

Recommendations for Diagnosis and Management of Autoimmune Pancreatitis in Childhood: Consensus From INSPPIRE.

J Pediatr Gastroenterol Nutr 2018 08;67(2):232-236

Hospital for Sick Children, Toronto, Ontario, Canada.

Objectives: Autoimmune pancreatitis (AIP) represents a complex immune-mediated pancreas disorder. Pediatric AIP (P-AIP) is rare. We have recently summarized the characteristic features of P-AIP. We now aim to develop recommendation statements to standardize the diagnostic and therapeutic approach to P-AIP and facilitate future research in the field.

Methods: A panel of pediatric gastroenterologists participating in the International Study Group of Pediatric Pancreatitis: In search for a cuRE was formed to discuss and then vote on 15 recommendation statements. A consensus of at least 80% was obtained following 3 voting rounds and revision of the statements.

Results: We have now generated 15 statements to help standardize the approach to diagnosis and management of P-AIP.

Conclusions: The first P-AIP recommendation statements developed by the International Study Group of Pediatric Pancreatitis: In search for a cuRE group are intended to bring standardization to the diagnosis and treatment of this rare childhood disorder. These statements may help guide a uniform approach to patient care and facilitate future research studies.
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http://dx.doi.org/10.1097/MPG.0000000000002028DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6059991PMC
August 2018

Cystic fibrosis: a gastrointestinal cancer syndrome.

Lancet Oncol 2018 06 26;19(6):719-720. Epub 2018 Apr 26.

Pediatric Gastroenterology, Hadassah-Hebrew University Medical Center, Jerusalem 24035, Israel; Cystic Fibrosis Center, Hadassah-Hebrew University Medical Center, Jerusalem 24035, Israel. Electronic address:

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http://dx.doi.org/10.1016/S1470-2045(18)30250-XDOI Listing
June 2018

Obesogenic habits among children and their families in response to initiation of gluten-free diet.

Eur J Pediatr 2018 Jun 29;177(6):859-866. Epub 2018 Mar 29.

Pediatric Gastroenterology Unit, Hebrew University-Hadassah Medical Center, Jerusalem, Israel.

Initiation of a lifelong, gluten-free diet (GFD) in children with celiac disease (CD) influences the child's life in many ways. The aim of this study was to assess the influence of GFD on the child and his/her family's eating habits and lifestyle behaviors. To study this, we asked children and their parents completed the Family Eating and Activity Habits Questionnaire (FEAHQ) at the time of diagnosis of CD and at least 6 months after initiation of GFD and a questionnaires assessing symptoms related to CD and adherence to the GFD diet. We analyzed questionnaires from 40 children with CD and their families. There were 21 females, ranging in age from 4 to 15.7 years (median age 7.4 years±2.8 years). The control group comprised 15 healthy children. After initiation of GFD the family ate more junk food including snacks and candies (p = 0.05), with the significant change reported by children and fathers (p = 0.001 and 0.03 respectively). All family members in the control group had significantly less snacks. Parents and children reported a significant increase in obesogenic eating styles, such as eating from the cooking pot and eating while doing other activities (mothers, p = 0.001; fathers, 0.02; and children, 0.02 respectively).

Conclusions: Our study shows that initiation of GFD in children with CD leads to changes in eating habits and staple food eating that may lead to a more obesogenic environment. Care givers, pediatricians, gastroenterologists, and dieticians alike should be aware of these implications and educate families towards a healthier lifestyle and diet beyond the GFD itself. What's Known: • Gluten-free diet has been shown to affect various psychosocial aspects of children with celiac disease. • Obesity and celiac are associated. What is New: • Initiation of gluten-free diet led to increased eating of junk food both in the patient and his/her family. • After initiation of GFD pro-obesogenic eating habits is increased.
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http://dx.doi.org/10.1007/s00431-018-3128-8DOI Listing
June 2018

EPC/HPSG evidence-based guidelines for the management of pediatric pancreatitis.

Pancreatology 2018 Mar 4;18(2):146-160. Epub 2018 Jan 4.

Institute for Translational Medicine, University of Pécs, Pécs, Hungary; First Department of Medicine, University of Szeged, Szeged, Hungary. Electronic address:

Background: Pediatric pancreatitis is an underdiagnosed disease with variable etiology. In the past 10-15 years the incidence of pediatric pancreatitis has increased, it is now 3.6-13.3 cases per 100,000 children. Up-to-date evidence based management guidelines are lacking for the pediatric pancreatitis. The European Pancreatic Club, in collaboration with the Hungarian Pancreatic Study Group organized a consensus guideline meeting on the diagnosis and management of pancreatitis in the pediatric population.

Methods: Pediatric Pancreatitis was divided into three main clinical categories: acute pancreatitis, acute recurrent pancreatitis and chronic pancreatitis. Fifteen relevant topics (acute pancreatitis: diagnosis; etiology; prognosis; imaging; complications; therapy; biliary tract management; acute recurrent pancreatitis: diagnosis; chronic pancreatitis: diagnosis, etiology, treatment, imaging, intervention, pain, complications; enzyme replacement) were defined. Ten experts from the USA and Europe reviewed and summarized the available literature. Evidence was classified according to the GRADE classification system.

Results: Within fifteen topics, forty-seven relevant clinical questions were defined. The draft of the updated guideline was presented and discussed at the consensus meeting held during the 49th Meeting of European Pancreatic Club, in Budapest, on July 1, 2017.

Conclusions: These evidence-based guidelines provides the current state of the art of the diagnosis and management of pediatric pancreatitis.
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http://dx.doi.org/10.1016/j.pan.2018.01.001DOI Listing
March 2018