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Pediatric hepatology

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https://www.readbyqxmd.com/read/29133255/high-prevalence-of-barrett-s-esophagus-and-esophageal-squamous-cell-carcinoma-after-repair-of-esophageal-atresia
#1
Floor W T Vergouwe, Hanneke IJsselstijn, Katharina Biermann, Nicole S Erler, René M H Wijnen, Marco J Bruno, Manon C W Spaander
BACKGROUND & AIMS: Esophageal atresia is rare, but improved surgical and intensive care techniques have increased rates of survival in children, so there are now many adults with this disorder. Many patients with esophageal atresia develop gastroesophageal reflux (GER), raising concerns about increased risk of Barrett's esophagus (BE; prevalence of 1.3%-1.6% in general population) and esophageal carcinoma. We assessed the prevalence of BE and esophageal carcinoma in this population. METHODS: We performed a prospective study of 289 patients with esophageal atresia at the Department of Gastroenterology and Hepatology at Erasmus MC University Medical Center in The Netherlands, from May 2012 through March 2017...
November 10, 2017: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/29113094/pediatric-chronic-intestinal-failure-in-italy-report-from-the-2016-survey-on-behalf-of-italian-society-for-gastroenterology-hepatology-and-nutrition-sigenp
#2
Antonella Diamanti, Teresa Capriati, Paolo Gandullia, Grazia Di Leo, Antonella Lezo, Laura Lacitignola, Maria Immacolata Spagnuolo, Simona Gatti, Lorenzo D'Antiga, Giovanna Verlato, Paola Roggero, Sergio Amarri, Maria Elisabetta Baldassarre, Francesco Cirillo, Domenica Elia, Renata Boldrini, Angelo Campanozzi, Carlo Catassi, Marina Aloi, Claudio Romano, Manila Candusso, Nicola Cecchi, Tommaso Bellini, Elaine Tyndall, Fabio Fusaro, Tamara Caldaro, Daniele Alberti, Piergiorgio Gamba, Mario Lima, Pietro Bagolan, Jean De Ville de Goyet, Luigi Dall'Oglio, Marco Spada, Francesca Grandi
BACKGROUND: Intestinal failure (IF) is the reduction in functioning gut mass below the minimal level necessary for adequate digestion and absorption of nutrients and fluids for weight maintenance in adults or for growth in children. There is a paucity of epidemiologic data on pediatric IF. The purpose of this study was to determine the prevalence, incidence, regional distribution and underlying diagnosis of pediatric chronic IF (CIF) requiring home parenteral nutrition (HPN) in Italy...
November 5, 2017: Nutrients
https://www.readbyqxmd.com/read/29098318/-wilson-s-disease-what-has-been-confirmed-in-diagnostic-and-therapy
#3
REVIEW
E-D Pfister
Wilson's disease (WD) is a rare autosomal recessive disorder characterized by abnormal copper accumulation. Presenting a broad variety of phenotypes and, thus, being a chameleon within the group of metabolic diseases, the manifold clinical symptoms of WD can include hepatologic, neurologic, and psychiatric manifestations. Early onset presentations in infancy and late-onset manifestations in adults older than 70 years of age have been described. If the typical laboratory blood test values are missing, the diagnosis of WD may be difficult and often involves a combination of different parameters...
November 2, 2017: Der Internist
https://www.readbyqxmd.com/read/29080241/next-generation-sequencing-in-pediatric-hepatology-and-liver-transplantation
#4
REVIEW
Emanuele Nicastro, Lorenzo D'Antiga
Next Generation Sequencing (NGS) has revolutionized the analysis of human genetic variations, offering a highly cost-effective way to diagnose monogenic diseases (MDs). Since nearly half of children with chronic liver disorders have a genetic cause and approximately 20% of pediatric liver transplants are performed in children with MDs, NGS offers the opportunity to significantly improve the diagnostic yield in this field. Among the NGS strategies, the use of targeted gene panels has proven useful to rapidly and reliably confirm a clinical suspicion, whereas the whole exome sequencing (WES) with variants filtering has been adopted to assist the diagnostic work up in unclear clinical scenarios...
October 28, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/29059122/response-to-the-letters-regarding-the-north-american-society-of-pediatric-gastroenterology-hepatology-and-nutrition-nafld-guidelines
#5
Miriam B Vos, Stephanie H Abrams, Sarah E Barlow, Sonia Caprio, Stephen R Daniels, Rohit Kohli, Marialena Mouzaki, Pushpa Sathya, Jeffery B Schwimmer, Shikha S Sundaram, Stavra A Xanthakos
No abstract text is available yet for this article.
November 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29048383/is-there-a-causal-relationship-between-intussusception-and-food-allergy
#6
Emrah Aydin, Omer F Beşer, Esra Ozek, Soner Sazak, Ensar Duras
Although intussusception and food allergy are common health problems in childhood, the relation between these two diseases remain obscure. The aim of this study is to investigate the relationship between food allergy and intussusception, and the factors associated with both. Patients diagnosed with intussusception by the Brighton Collaboration Intussusception Working Group criteria were prospectively investigated for food allergy per the European Society for Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) Guideline...
October 19, 2017: Children
https://www.readbyqxmd.com/read/28983682/biliary-atresia-unity-in-diversity
#7
REVIEW
Claus Petersen
Biliary atresia (BA) is a rare disease of unknown origin and unsatisfying outcome. Single, multicenter and national evaluations of epidemiological and outcome data on BA have been periodically published over the course of decades. However, the diversity of the registered parameters and outcome measures impede comparability and cumulative analysis of these very worthwhile studies. Taking into account the fact that BA is a good example of translational research and transition of patients from pediatric surgery and hepatology to transplant surgery and hepatology in general, the interdisciplinary community should make every effort to develop a common platform upon which further activities are conducted...
December 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28977113/primary-sclerosing-cholangitis-in-children-and-adolescents
#8
Eleonora Druve Tavares Fagundes, Alexandre Rodrigues Ferreira, Caroline Caldeira Hosken, Thaís Costa Nascentes Queiroz
BACKGROUND: Primary sclerosing cholangitis is a rare disease, but its prevalence has been underestimated in children and adolescents due to broad variation in clinical presentation as well as diagnostic challenges in this life period. OBJECTIVE: To evaluate children and adolescents with primary sclerosing cholangitis and to describe their clinical, laboratorial, histopathological, and cholangiography conditions. METHODS: This is an observational descriptive research that took place from 2005 to 2016 and included all the patients seen in the Outpatient Unit for Pediatric Hepatology of Hospital das Clinicas of UFMG who had been diagnosed with primary sclerosing cholangitis before the age of 18...
October 2, 2017: Arquivos de Gastroenterologia
https://www.readbyqxmd.com/read/28968008/-profile-of-gastrointestinal-diseases-in-a-pediatric-gastroenterology-center-in-colombia-15-years-of-follow-up
#9
Wilson Daza, Silvana Dadan, Michelle Higuera
INTRODUCTION: The profile of gastrointestinal diseases is constantly changing globally and locally affecting suspected diagnosis and medical methods. OBJECTIVE: To establish the main diagnoses in a gastroenterology, hepatology, and pediatric nutrition unit in Bogota between 2009 and 2013, and to compare with findings from the same unit during the two prior five-year periods (1997 to 2006). MATERIALS AND METHODS: We conducted a retrospective descriptive study...
September 1, 2017: Biomédica: Revista del Instituto Nacional de Salud
https://www.readbyqxmd.com/read/28950706/efficacy-of-a-point-of-care-test-based-on-deamidated-gliadin-peptides-for-the-detection-of-celiac-disease-in-pediatric-patients
#10
Isabel Polanco, Thabata Koester Weber, Eva Martínez-Ojinaga, Manuel Molina, Jesús Sarria
OBJECTIVE: The objective of the study was to assess the effectiveness of a point-of-care test (POCT) based on deamidated gliadin peptides (DGP) compared to the European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) criteria diagnosis in the early detection of celiac disease (CD) in pediatric patients. METHODS: One hundred children (≤ 18 years) with suspected CD were selected, including siblings of celiac children that underwent gastroscopy for other gastrointestinal conditions...
November 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/28927776/-gluten-is-the-information-available-on-the-internet-valid
#11
T Banti, L Fievet, A Fabre
BACKGROUND: Internet provides easy access to health information, but the quality and validity of this information vary. OBJECTIVES: Evaluate the quality of website structures and the information provided on celiac disease (CD), gluten sensitivity (GS), and wheat allergy (WA). MATERIALS AND METHODS: The websites addressing CD, GS, and WA appearing on the first two pages of Google, Yahoo, and Bing from seven selected queries were investigated...
September 15, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28837507/intestinal-rehabilitation-programs-in-the-management-of-pediatric-intestinal-failure-and-short-bowel-syndrome
#12
Russell J Merritt, Valeria Cohran, Bram P Raphael, Timothy Sentongo, Diana Volpert, Brad W Warner, Praveen S Goday
Intestinal failure is a rare, debilitating condition that presents both acute and chronic medical management challenges. The condition is incompatible with life in the absence of the safe application of specialized and individualized medical therapy that includes surgery, medical equipment, nutritional products, and standard nursing care. Intestinal rehabilitation programs are best suited to provide such complex care with the goal of achieving enteral autonomy and oral feeding with or without intestinal transplantation...
November 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28837442/diagnosis-follow-up-and-treatment-of-cystic-fibrosis-related-liver-disease
#13
Ivo P van de Peppel, Anna Bertolini, Johan W Jonker, Frank A J A Bodewes, Henkjan J Verkade
PURPOSE OF REVIEW: To provide an insight and overview of the challenges in the diagnosis, follow-up and treatment of cystic fibrosis-related liver disease (CFLD). RECENT FINDINGS: The variable pathophysiology of CFLD complicates its diagnosis and treatment. A 'gold standard' for CFLD diagnosis is lacking. Over the past years, new techniques to diagnose features of CFLD, such as transient elastography, have been investigated. Although most of these tests confirm cystic fibrosis-related liver involvement (CFLI), they are, however, not suitable to distinguish various phenotypical presentations or predict progression to clinically relevant cirrhosis or portal hypertension...
August 23, 2017: Current Opinion in Pulmonary Medicine
https://www.readbyqxmd.com/read/28776642/diagnosis-of-monogenic-liver-diseases-in-childhood-by-next-generation-sequencing
#14
Amelie Stalke, Britta Skawran, Bernd Auber, Thomas Illig, Brigitte Schlegelberger, Norman Junge, Imeke Goldschmidt, Christoph Leiskau, Nils von Neuhoff, Ulrich Baumann, Eva-Doreen Pfister
Next-Generation Sequencing (NGS) has opened up novel diagnostic opportunities for children with unidentified, but suspected inherited diseases. We describe our single-center experience with NGS diagnostics in standard clinical scenarios in pediatric hepatology. We investigated 135 children with suspected inherited hepatopathies, where initially no causative pathogenic variant had been identified, with an amplicon-based NGS panel of 21 genes associated with acute and chronic hepatopathies. In 23 of these patients we detected pathogenic or likely pathogenic variants in ten different genes...
August 4, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28709446/omitting-duodenal-biopsy-in-children-with-suspected-celiac-disease-and-extra-intestinal-symptoms
#15
Mauro Bozzola, Cristina Meazza, Chiara Gertosio, Sara Pagani, Daniela Larizza, Valeria Calcaterra, Ombretta Luinetti, Giovanni Farello, Carmine Tinelli, Lorenzo Iughetti
BACKGROUND: The aim of our study is to evaluate if in children with highly positive serology and HLA-DQ2/DQ8 (triple test, TT) and only extra-intestinal symptoms, it is possible to omit performing an intestinal biopsy for celiac disease (CD) diagnosis, as suggested by the new European Society for Pediatric Gastroenterology, Hepatology and Nutrition ESPGHAN guidelines. METHODS: In this retrospective study a total of 105 patients, suspected of having CD because of extra-intestinal symptoms and showing serum tissue transglutaminase antibody (anti-tTG) and anti-endomysial antibody (EMA) measurements and HLA genotyping, were considered for the final analysis (33 boys and 72 girls; age range 1...
July 15, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/28644369/european-society-for-pediatric-gastroenterology-hepatology-and-nutrition-s-educational-offer-and-the-training-syllabus
#16
Marco Maglione, Daniela Finizio, Gabor Veres, Tudor L Pop, Grazia I Continisio, Alexandra Papadopoulou, Alfredo Guarino
The basic knowledge necessary for a European pediatric gastroenterologist/hepatologist/nutritionist is set-out in the training syllabus (TS) of the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN). We retrospectively compared the topics covered in ESPGHAN's training events between 2013 and 2016 with the basic knowledge TS items. Thirty-six initiatives including e-learning were identified. Twelve (33%) courses focused on gastroenterology, 9 (25%) on hepatology, and 10 (28%) on nutrition...
November 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28624578/accuracy-in-diagnosis-of-celiac-disease-without-biopsies-in%C3%A2-clinical-practice
#17
MULTICENTER STUDY
Katharina Julia Werkstetter, Ilma Rita Korponay-Szabó, Alina Popp, Vincenzo Villanacci, Marianna Salemme, Gabriele Heilig, Søren Thue Lillevang, Maria Luisa Mearin, Carmen Ribes-Koninckx, Adrian Thomas, Riccardo Troncone, Birgit Filipiak, Markku Mäki, Judit Gyimesi, Mehri Najafi, Jernej Dolinšek, Stine Dydensborg Sander, Renata Auricchio, Alexandra Papadopoulou, Andreas Vécsei, Peter Szitanyi, Ester Donat, Rafaella Nenna, Philippe Alliet, Francesca Penagini, Hélène Garnier-Lengliné, Gemma Castillejo, Kalle Kurppa, Raanan Shamir, Almuthe Christine Hauer, Françoise Smets, Susana Corujeira, Myriam van Winckel, Stefan Buderus, Sonny Chong, Steffen Husby, Sibylle Koletzko
BACKGROUND & AIMS: The guidelines of the European Society of Pediatric Gastroenterology, Hepatology, and Nutrition allow for diagnosis of celiac disease without biopsies in children with symptoms and levels of immunoglobulin A against tissue-transglutaminase (TGA-IgA) 10-fold or more the upper limit of normal (ULN), confirmed by detection of endomysium antibodies (EMA) and positivity for HLA-DQ2/DQ8. We performed a large, international prospective study to validate this approach. METHODS: We collected data from consecutive pediatric patients (18 years or younger) on a gluten-containing diet who tested positive for TGA-IgA from November 2011 through May 2014, seen at 33 pediatric gastroenterology units in 21 countries...
October 2017: Gastroenterology
https://www.readbyqxmd.com/read/28604510/safety-and-efficacy-of-the-push-endoscopic-technique-in-the-management-of-esophageal-food-bolus-impactions-in-children
#18
Jamal Kriem, Riad Rahhal
OBJECTIVES: Adult-based guidelines support the use of the pull (extraction) endoscopic technique in managing esophageal food bolus impactions (FBIs) but allow the consideration of the push (advancement) technique with caution based on available published literature. The North American Society of Pediatric Gastroenterology, Hepatology and Nutrition guidelines mention the use of gentle endoscopic pushing for disimpaction but elaborate that this technique has not been studied in children...
June 9, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28541262/joint-espghan-naspghan-guidelines-for-the-management-of-helicobacter-pylori-in-children-and-adolescents-update-2016
#19
Nicola L Jones, Sibylle Koletzko, Karen Goodman, Patrick Bontems, Samy Cadranel, Thomas Casswall, Steve Czinn, Benjamin D Gold, Jeannette Guarner, Yoram Elitsur, Matjaž Homan, Nicolas Kalach, Michal Kori, Armando Madrazo, Francis Megraud, Alexandra Papadopoulou, Marion Rowland
BACKGROUND: Because of the changing epidemiology of Helicobacter pylori infection and low efficacy of currently recommended therapies, an update of the European Society for Paediatric Gastroenterology Hepatology and Nutrition/North American Society for Pediatric Gastroenterology, Hepatology and Nutrition recommendations for the diagnosis and management of H pylori infection in children and adolescents is required. METHODS: A systematic review of the literature (time period: 2009-2014) was performed...
June 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28487842/dietary-primary-prevention-of-allergic-diseases-in-children-the-philippine-guidelines
#20
REVIEW
Marysia Stella T Recto, Maria Lourdes G Genuino, Mary Anne R Castor, Roxanne J Casis-Hao, Diana R Tamondong-Lachica, Maria Imelda V Sales, Marilou G Tan, Karen S Mondonedo, Regina C Dionisio-Capulong
Allergic diseases, such as asthma, allergic rhinitis, eczema, and food allergy, are preventable diseases. Primary prevention strategies of allergic diseases have been in scrutiny. Effective prevention strategies maybe started prenatally, postnatally, during infancy, and even during childhood. These guidelines have been prepared by the Philippine Society of Allergy, Asthma and Immunology and the Philippine Society for Pediatric Gastroenterology, Hepatology and Nutrition. They aim to provide evidence-based recommendations for the dietary primary prevention of allergic diseases in children...
April 2017: Asia Pacific Allergy
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