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Pediatric hepatology

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https://www.readbyqxmd.com/read/28837507/clinical-report-intestinal-rehabilitation-programs-in-the-management-of-pediatric-intestinal-failure-and-short-bowel-syndrome
#1
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 (IR) programs are best suited to provide such complex care with the goal of achieving enteral autonomy and oral feeding with or without intestinal transplantation...
August 23, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28837442/diagnosis-follow-up-and-treatment-of-cystic-fibrosis-related-liver-disease
#2
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
#3
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
#4
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
#5
Marco Maglione, Daniela Finizio, Gabor Veres, Tudor Lucian Pop, Grazia Isabella Continisio, Alexandra Papadopoulou, Alfredo Guarino
The basic knowledge necessary for a European pediatric gastroenterologist/hepatologist/nutritionist (PGHN) 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...
June 21, 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
#6
Katharina J Werkstetter, Ilma R 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, Ranaan 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...
June 15, 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
#7
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
#8
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
#9
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
https://www.readbyqxmd.com/read/28471111/intelligent-diagnosis-of-jaundice-with-dynamic-uncertain-causality-graph-model
#10
Shao-Rui Hao, Shi-Chao Geng, Lin-Xiao Fan, Jia-Jia Chen, Qin Zhang, Lan-Juan Li
Jaundice is a common and complex clinical symptom potentially occurring in hepatology, general surgery, pediatrics, infectious diseases, gynecology, and obstetrics, and it is fairly difficult to distinguish the cause of jaundice in clinical practice, especially for general practitioners in less developed regions. With collaboration between physicians and artificial intelligence engineers, a comprehensive knowledge base relevant to jaundice was created based on demographic information, symptoms, physical signs, laboratory tests, imaging diagnosis, medical histories, and risk factors...
May 2017: Journal of Zhejiang University. Science. B
https://www.readbyqxmd.com/read/28437324/noncirrhotic-portal-fibrosis-in-pediatric-population
#11
Vikrant Sood, Bikrant B Lal, Rajeev Khanna, Dinesh Rawat, Chhagan Bihari, Seema Alam
OBJECTIVES: Noncirrhotic portal fibrosis (NCPF) has been classically described as a disease of young to middle age with limited literature regarding its occurrence, onset, or clinical presentation in children. We hereby present a series of 19 patients diagnosed and managed as NCPF in pediatric age group. METHODS: A retrospective review of all the patients presenting to the pediatric hepatology department (age <18 years) and diagnosed as NCPF was done and data were evaluated...
May 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28396105/eosinophilic-esophagitis-a-case-study-of-a-12-year-old-boy
#12
Katherine Kiely
Eosinophilic esophagitis is an underrecognized chronic condition in which symptoms of gastroesophageal reflux are accompanied by eosinophilia in the esophagus. This disorder affects both children and adults and is associated with a history of other atopic disorders, particularly food allergies. This case study discusses the epidemiology of eosinophilic esophagitis and the proper procedures for diagnosis, referral, and management using the dietary restrictions and pharmacologic treatment included in the clinical recommendations issued by the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition; the American Academy of Allergy, Asthma, and Immunology; and the American College of Gastroenterologists...
April 7, 2017: Journal of Pediatric Health Care
https://www.readbyqxmd.com/read/28333768/management-of-pediatric-non-alcoholic-fatty-liver-disease-by-academic-hepatologists-in-canada-a-nationwide-survey
#13
Marialena Mouzaki, Simon C Ling, Richard A Schreiber, Binita M Kamath
BACKGROUND: The literature on the optimal clinical management of pediatric patients with non-alcoholic fatty liver disease (NAFLD) is limited. The objective of this study was to identify discrepancies in the care provided to patients with NAFLD by hepatologists practicing in academic centers across Canada. METHODS: A nationwide survey was distributed electronically to all pediatric hepatologists practicing in university-affiliated hospitals using the infrastructure of the Canadian Pediatric Hepatology Research Group...
March 22, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28317399/recent-advances-in-pediatric-celiac-disease
#14
Grace J Lee, John Y Kao
The incidence of celiac disease (CD) has increased over the last half-century, resulting in rising interest in identifying risk factors for CD. The necessity of duodenal biopsies in the diagnosis of CD has recently been challenged. Areas covered: This review covers the recent literature regarding the role of infant feeding practices, including breastfeeding and timing of gluten introduction, and the microbiota in the development of CD. Additionally, the application of the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition guidelines for a non-biopsy approach to the diagnosis of CD is reviewed...
June 2017: Expert Review of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28293079/pediatric-gastrointestinal-bleeding-perspectives-from-the-italian-society-of-pediatric-gastroenterology
#15
REVIEW
Claudio Romano, Salvatore Oliva, Stefano Martellossi, Erasmo Miele, Serena Arrigo, Maria Giovanna Graziani, Sabrina Cardile, Federica Gaiani, Gian Luigi de'Angelis, Filippo Torroni
There are many causes of gastrointestinal bleeding (GIB) in children, and this condition is not rare, having a reported incidence of 6.4%. Causes vary with age, but show considerable overlap; moreover, while many of the causes in the pediatric population are similar to those in adults, some lesions are unique to children. The diagnostic approach for pediatric GIB includes definition of the etiology, localization of the bleeding site and determination of the severity of bleeding; timely and accurate diagnosis is necessary to reduce morbidity and mortality...
February 28, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28292383/mucopolysaccharidoses-clinical-spectrum-and-frequency-of-different-types
#16
Huma Arshad Cheema, Hassan Suleman Malik, Muhammad Almas Hashmi, Zafar Fayyaz, Iqra Mushtaq, Nagina Shahzadi
OBJECTIVE: To determine the relative frequency and clinical features of different varieties of mucopolysaccharidosis. STUDY DESIGN: Descriptive study. PLACE AND DURATION OF STUDY: Department of Pediatric Gastroenterology, Hepatology and Nutrition, The Children's Hospital and The Institute of Child Health, Lahore, from January 2013 to December 2015. METHODOLOGY: All patients who had any feature suggestive of mucopolysaccharidosis were screened with detailed history, clinical examination and skeletal survey...
February 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28242319/white-paper-aga-power-practice-guide-on-obesity-and%C3%A2-weight-management-education-and-resources
#17
REVIEW
Andres Acosta, Sarah Streett, Mathew D Kroh, Lawrence J Cheskin, Katherine H Saunders, Marina Kurian, Marsha Schofield, Sarah E Barlow, Louis Aronne
The epidemic of obesity continues at alarming rates, with a high burden to our economy and society. The American Gastroenterological Association understands the importance of embracing obesity as a chronic, relapsing disease and supports a multidisciplinary approach to the management of obesity. Because gastrointestinal disorders resulting from obesity are more frequent and often present sooner than type 2 diabetes mellitus and cardiovascular disease, gastroenterologists have an opportunity to address obesity and provide an effective therapy early...
May 2017: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28230608/specialized-imaging-and-procedures-in-pediatric-pancreatology-a-north-american-society-for-pediatric-gastroenterology-hepatology-and-nutrition-clinical-report
#18
Tom K Lin, David M Troendle, Daniel B Wallihan, Bradley Barth, Victor L Fox, Douglas S Fishman, Veronique D Morinville
OBJECTIVES: An increasing number of children are being diagnosed with pancreatitis and other pancreatic abnormalities. Dissemination of the information regarding existing imaging techniques and endoscopic modalities to diagnose and manage pancreatic disorders in children is sorely needed. METHODS: We conducted a review of the medical literature on the use of the following imaging and procedural modalities in pediatric pancreatology: transabdominal ultrasonography (TUS), computed tomography (CT), magnetic resonance imaging (MRI)/magnetic resonance cholangiopancreatography (MRCP), endoscopic ultrasonography (EUS), and endoscopic retrograde cholangiopancreatography (ERCP)...
March 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28199778/a-national-retrospective-study-of-paediatric-end-stage-liver-disease-as-a-predictor-of-change-to-second-line-therapy-in-children-with-autoimmune-hepatitis
#19
Andréanne N Zizzo, Carolina Jimenez-Rivera, Joseph Kim, Richard A Schreiber, Simon C Ling, Jason Yap, Jeff Critch, Najma Ahmed, Fernando Alvarez, Binita M Kamath
BACKGROUND & AIMS: Adult studies of autoimmune hepatitis (AIH) have shown that the model of end-stage liver disease is associated with resistance to first-line treatment. Using a multicentre retrospective database, we sought to determine if the paediatric end-stage liver disease (PELD) score would similarly predict treatment resistance in paediatric AIH. METHODS: One hundred and seventy-one children from 13 Canadian centres who fulfilled the International Autoimmune Hepatitis Group (IAIHG) criteria were included and assessed for change to second-line therapy within 24 months of primary treatment onset...
February 15, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28177956/optimizing-the-diagnosis-of-celiac-disease
#20
Michelle Shui Yee Lau, David S Sanders
PURPOSE OF REVIEW: The diagnostic approach in celiac disease is continuously evolving as our understanding of its pathophysiology improves. This review aims to provide a summary of contemporary work that supports optimization of the diagnosis of this common yet underdiagnosed condition. RECENT FINDINGS: The recently updated National Institute of Clinical Excellence and European Society for Pediatric Gastroenterology, Hepatology, and Nutrition guidelines and the contentious biopsy-free diagnostic approach will be discussed...
May 2017: Current Opinion in Gastroenterology
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