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Pancreatitis in childrens

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https://www.readbyqxmd.com/read/29773689/risk-factors-for-lung-disease-progression-in-children-with-cystic-fibrosis
#1
Marieke van Horck, Kim van de Kant, Bjorn Winkens, Geertjan Wesseling, Vincent Gulmans, Han Hendriks, Chris van der Grinten, Quirijn Jӧbsis, Edward Dompeling
To identify potential risk factors for lung disease progression in children with cystic fibrosis (CF), we studied the longitudinal data of all children with CF (aged ≥5 years) registered in the Dutch CF Registry (2009-2014). Lung disease progression was expressed as a decline in lung function (FEV1 %pred) and the pulmonary exacerbation rate (PEx). Potential risk factors at baseline included: sex, age, best FEV1%pred, best forced vital capacity (FVC)%pred, genotype, BMI z-score, pancreatic insufficiency, medication use (proton pump inhibitors [PPI], prophylactic antibiotics, and inhaled corticosteroids), CF-related diabetes, allergic bronchopulmonary aspergillosis, and colonisation with Pseudomonas aeruginosa The data of 545 children were analysed...
May 17, 2018: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/29768684/practical-approach-to-the-gastrointestinal-manifestations-of-cystic-fibrosis
#2
REVIEW
Rishi Bolia, Chee Y Ooi, Peter Lewindon, Jonathan Bishop, Sarath Ranganathan, Jo Harrison, Kristyn Ford, Natalie van der Haak, Mark R Oliver
Cystic fibrosis (CF) is the most common, life-shortening, genetic illness affecting children in Australia and New Zealand. The genetic abnormality results in abnormal anion transport across the apical membrane of epithelial cells in a number of organs, including the lungs, gastrointestinal tract, liver and genito-urinary tract. Thus, CF is a multi-system disorder that requires a multi-disciplinary approach. Respiratory disease is the predominant cause of both morbidity and mortality in patients with CF. However, there are significant and clinically relevant gastrointestinal, liver, pancreatic and nutritional manifestations that must be detected and managed in a timely and structured manner...
May 16, 2018: Journal of Paediatrics and Child Health
https://www.readbyqxmd.com/read/29762381/13c-labeled-starch-breath-test-in-congenital-sucrase-isomaltase-deficiency
#3
Claudia C Robayo-Torres, Marisela Diaz-Sotomayor, Bruce R Hamaker, Susan S Baker, Bruno P Chumpitazi, Antone R Opekun, Buford L Nichols
BACKGROUND AND HYPOTHESES: Human starch digestion is a multienzyme process involving 6 different enzymes: salivary and pancreatic α-amylase; sucrase and isomaltase (from sucrose-isomaltase [SI]), and maltase and glucoamylase (from maltase-glucoamylase [MGAM]). Together these enzymes cleave starch to smaller molecules ultimately resulting in the absorbable monosaccharide glucose. Approximately 80% of all mucosal maltase activity is accounted for by SI and the reminder by MGAM. Clinical studies suggest that starch may be poorly digested in those with congenital sucrase-isomaltase deficiency (CSID)...
June 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29753677/multicenter-phase-1-2-application-of-adenovirus-specific-t-cells-in-high-risk-pediatric-patients-after-allogeneic-stem-cell-transplantation
#4
Winnie Ip, Juliana M F Silva, Hubert Gaspar, Arindam Mitra, Shreenal Patel, Kanchan Rao, Robert Chiesa, Persis Amrolia, Kimberly Gilmour, Gul Ahsan, Mary Slatter, Andrew R Gennery, Robert F Wynn, Paul Veys, Waseem Qasim
BACKGROUND: Adenovirus (ADV) reactivation can cause significant morbidity and mortality in children after allogeneic stem cell transplantation. Antiviral drugs can control viremia, but viral clearance requires recovery of cell-mediated immunity. METHOD: This study was an open-label phase 1/2 study to investigate the feasibility of generating donor-derived ADV-specific T cells (Cytovir ADV, Cell Medica) and to assess the safety of pre-emptive administration of ADV-specific T cells in high-risk pediatric patients after allogeneic hematopoietic stem cell transplantation (HSCT) to treat adenoviremia...
May 9, 2018: Cytotherapy
https://www.readbyqxmd.com/read/29751784/henoch-sch%C3%A3-nlein-purpura-with-acute-pancreatitis-analysis-of-13-cases
#5
Qin Zhang, Qi Guo, Ming Gui, Zhenhua Ren, Bo Hu, Ling Lu, Fang Deng
BACKGROUND: Henoch-Schönlein purpura is a common small vessel vasculitis in children. Acute pancreatitis rarely presents as a complication of Henoch-Schönlein purpura and has not been well characterized. METHODS: We retrospectively reviewed 13 cases of Henoch-Schönlein purpura with acute pancreatitis among 3212 patients who attended our hospital between January 2003 and June 2016 and analyzed their clinical characteristics, laboratory findings, imaging findings, treatment and overall prognosis...
May 11, 2018: BMC Pediatrics
https://www.readbyqxmd.com/read/29750397/fdg-pet-ct-in-the-evaluation-of-primary-and-secondary-pancreatic-malignancies
#6
Casey E Bohl, Sara M Federico, Giles W Robinson, Armita Bahrami, Barry L Shulkin
PURPOSE: Primary pancreatic carcinoma and pancreatic metastases are rare in the pediatric population. Pancreatoblastoma is the most common pancreatic malignant tumor in young children and solid-pseudopapillary tumor in teenagers. Pancreatic adenocarcinoma is extremely rare under the age of 40 and is usually associated with underlying genetic abnormalities. Secondary malignancies of the pancreas occur more frequently than primary pancreatic malignancies in children and are most commonly seen with non-Hodgkin lymphomas (NHL) and mesenchymal sarcomas...
May 11, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29746344/nutritional-considerations-in-pediatric-pancreatitis-a-position-paper-from-the-naspghan-pancreas-committee-espghan-pancreas-working-group
#7
Maisam Abu-El-Haija, Aliye Uc, Steven L Werlin, A Jay Freeman, Miglena Georgieva, Danijela Jojkić - Pavkov, Daina Kalnins, Brigitte Kochavi, Bart G P Koot, Stephanie Van Biervliet, Jaroslaw Walkowiak, Michael Wilschanski, Veronique D Morinville
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...
May 8, 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29737796/pancreatic-enzyme-replacement-therapy-pert-in-children-with-persistent-diarrhea-avoidance-of-elemental-diet-need-accessibility-and-costs
#8
Ariani Dewi Widodo, Rianto Setiabudy, Ina S Timan, Saptawati Bardosono, Widdy Winarta, Agus Firmansyah
BACKGROUND AND OBJECTIVES: Persistent diarrhea has been proven to cause pancreatic exocrine insufficiency, due to decreased stimulation to the pancreas caused by prolonged mucosal injury. Pancreatic enzyme replacement therapy (PERT) given in conjunction to regular treatment is thought to be beneficial in replacing this pancreatic enzyme deficiency, avoiding the need of elemental diet. This study aims to evaluate the benefit of PERT in chil-dren with persistent diarrhea. METHODS AND STUDY DESIGN: This is a randomized, two double-blind parallel group, placebo-controlled clinical trial to evaluate the effects of pancreatic enzyme supplementation in persistent diar-rhea...
2018: Asia Pacific Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/29735580/natural-history-clinical-manifestations-and-pathogenesis-of-hepatitis-e-virus-genotype-1-and-2-infections
#9
Rakesh Aggarwal, Amit Goel
Infection with genotype 1 or 2 hepatitis E virus (HEV) results primarily from human-to-human transmission through the fecal-oral route in low-resource countries. It presents primarily as "acute viral hepatitis" syndrome, usually a self-limiting illness. A few cases progress to acute liver failure, a serious illness with high fatality. Clinical disease is infrequent among children. Infection during pregnancy is associated with a higher risk of symptomatic disease, severe liver injury, and mortality...
May 7, 2018: Cold Spring Harbor Perspectives in Medicine
https://www.readbyqxmd.com/read/29735574/management-of-type-1-diabetes-with-a-very-low-carbohydrate-diet
#10
Belinda S Lennerz, Anna Barton, Richard K Bernstein, R David Dikeman, Carrie Diulus, Sarah Hallberg, Erinn T Rhodes, Cara B Ebbeling, Eric C Westman, William S Yancy, David S Ludwig
OBJECTIVES: To evaluate glycemic control among children and adults with type 1 diabetes mellitus (T1DM) who consume a very low-carbohydrate diet (VLCD). METHODS: We conducted an online survey of an international social media group for people with T1DM who follow a VLCD. Respondents included adults and parents of children with T1DM. We assessed current hemoglobin A1c (HbA1c) (primary measure), change in HbA1c after the self-reported beginning of the VLCD, total daily insulin dose, and adverse events...
May 7, 2018: Pediatrics
https://www.readbyqxmd.com/read/29731964/total-body-irradiation-and-iron-chelation-treatment-are-associated-with-pancreatic-injury-following-pediatric-hematopoietic-stem-cell-transplantation
#11
Natalia Maximova, Massimo Gregori, Roberto Simeone, Aurelio Sonzogni, Davide Zanon, Giulia Boz, Lorenzo D'Antiga
Whereas many studies have addressed the risk of organ dysfunction following hematopoietic stem cell transplantation (HSCT), little is known about pancreatic susceptibility in this setting. We aimed to investigate the effect of iron overload (IO) and total body irradiation (TBI) on pancreatic function of children undergoing HSCT. We retrospectively evaluated children admitted between 2012-2016 fulfilling the following criteria: normal pancreatic iron concentration (PIC), regular pancreatic function before HSCT, availability of abdominal magnetic resonance imaging with gradient-recalled-echo sequences and a full set of biochemical markers of IO and pancreatic function performed before HSCT and at discharge...
April 13, 2018: Oncotarget
https://www.readbyqxmd.com/read/29706289/urinary-antimicrobial-peptides-potential-novel-biomarkers-of-obstructive-uropathy
#12
S Gupta, A R Jackson, D G DaJusta, D J McLeod, S A Alpert, V R Jayanthi, K McHugh, A R Schwaderer, B Becknell, C B Ching
INTRODUCTION: Antimicrobial peptides (AMPs) have historically been evaluated for their role in protecting against uropathogens. However, there is mounting evidence to support their expression in noninfectious injury, with unclear meaning as to their function. It is possible that AMPs represent urothelial injury. Urinary tract obstruction is known to alter the urothelium; however, AMPs have not been evaluated for expression in this noninfectious injury. OBJECTIVE: A pilot study to compare urinary AMP expression in children undergoing surgical intervention for ureteropelvic junction obstruction (UPJO) with nonobstructed controls...
March 29, 2018: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/29701109/attitudes-toward-return-of-genetic-research-results-to-relatives-including-after-death-comparison-of-cancer-probands-blood-relatives-and-spouse-partners
#13
Carmen Radecki Breitkopf, Susan M Wolf, Kari G Chaffee, Marguerite E Robinson, Noralane M Lindor, Deborah R Gordon, Barbara A Koenig, Gloria M Petersen
Genetic research generates results with implications for relatives. Recommendations addressing relatives' access to a participant's genetic research findings include eliciting participant preferences about access and choosing a representative to make decisions about access upon participant incapacity/death. Representatives are likely to be blood relatives or spouse/partners (who may share genetically related children). This raises the question of whether relatives hold similar attitudes about access or divergent attitudes that may yield conflict...
April 1, 2018: Journal of Empirical Research on Human Research Ethics: JERHRE
https://www.readbyqxmd.com/read/29700810/refining-the-phenotype-associated-with-biallelic-dnajc21-mutations
#14
G D'Amours, F Lopes, J Gauthier, V Saillour, C Nassif, R Wynn, N Alos, T Leblanc, Y Capri, S Nizard, E Lemyre, J L Michaud, V-A Pelletier, Y D Pastore, J-F Soucy
Inherited bone marrow failure syndromes (IBMFS) are caused by mutations in genes involved in genomic stability. Although they may be recognized by the association of typical clinical features, variable penetrance and expressivity are common, and clinical diagnosis is often challenging. DNAJC21, which is involved in ribosome biogenesis, was recently linked to bone marrow failure. However, the specific phenotype and natural history remain to be defined. We correlate molecular data, phenotype, and clinical history of five unreported affected children and all individuals reported in the literature...
April 26, 2018: Clinical Genetics
https://www.readbyqxmd.com/read/29699766/factors-associated-with-preservation-of-c-peptide-levels-at-the-diagnosis-of-type-1-diabetes
#15
Agnieszka Szypowska, Lidia Groele, Marta Wysocka-Mincewicz, Artur Mazur, Lucyna Lisowicz, Iwona Ben-Skowronek, Joanna Sieniawska, Bożenna Klonowska, Dorota Charemska, Jolanta Nawrotek, Irena Jałowiec, Artur Bossowski, Klaudyna Noiszewska, Beata Pyrżak, Izabela Rogozińska, Mieczysław Szalecki
AIMS: The level of C-peptide can identify individuals most likely to respond to immune interventions carried out to prevent pancreatic β-cell damage. The aim of the study was to evaluate factors associated with C-peptide levels at type 1 diabetes (T1D) diagnosis. METHODS: This study included 1098 children aged 2-17 with newly recognized T1D. Data were collected from seven Polish hospitals. The following variables were analyzed: date of birth, fasting C-peptide, HbA1c, sex, weight, height, pH at diabetes onset...
March 23, 2018: Journal of Diabetes and its Complications
https://www.readbyqxmd.com/read/29697579/acid-suppressing-drugs-and-a-low-1-level-of-antithrombin-as-risk-factors-for-l-asparaginase-associated-pancreatitis-a-case-control-study-in-the-japan-association-of-childhood-leukemia-study-jacls
#16
Yoshiko Hashii, Makoto Yoshida, Junichi Hara, Shinichiro Nishimura, Keiko Yumura-Yagi, Keizo Horibe, Tatsutoshi Nakahata
L-Asparaginase has significantly improved outcome for children with acute lymphoblastic leukemia and has become an essential component of multiagent chemotherapy. However, there are many adverse events due to L-asparaginase, including acute pancreatitis. The pathology of L-asparaginase-associated pancreatitis (AAP) remains unclear. We compared patients who developed AAP (n=29) and random matched controls (n=36) who had been enrolled in the Japan Association of Childhood Leukemia Study of the ALL-02 protocol...
April 24, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29697193/solid-pseudopapillary-and-malignant-pancreatic-tumors-in-childhood-a-systematic-review-and-evidence-quality-assessment
#17
REVIEW
Konstantinos S Mylonas, Ilias P Doulamis, Diamantis I Tsilimigras, Dimitrios Nasioudis, Dimitrios Schizas, Peter T Masiakos, Cassandra M Kelleher
A systematic review of 32 studies reporting on 489 children with pancreatic tumors was performed. The most prevalent histologic subtype was solid pseudopapillary tumor (SPT) (61.3%). Pancreaticoduodenectomy was the most commonly performed operation (48%). Neoadjuvant chemo/radiotherapy was utilized in 3.9, 36.2, 25, and 27.8% of patients with SPTs, pancreatoblastomas (PBLs), neuroendocrine tumors, and exocrine carcinomas, respectively. Adjuvant chemotherapy (75.6%) and radiation (34%) were most commonly utilized in PBLs...
April 26, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29683955/successful-management-of-a-child-with-drug-induced-necrotizing-pancreatitis-during-acute-lymphoblastic-leukemia-therapy-a-case-report
#18
Anna Płotka, Agnieszka Wziątek, Jacek Wachowiak, Katarzyna Derwich
Acute pancreatitis in children acute lymphoblastic leukemia is commonly caused by drugs, for example, L-Asparaginase, pegapargase, steroids. The incidence of this complication is estimated at 6.7% to 18%. Although the majority of drug-induced acute pancreatitis cases are mild, severe cases can rarely occur. This work presents a case of successful management of a child with drug-induced necrotizing pancreatitis during acute lymphoblastic leukemia therapy. This case illustrates that comprehensive care and immediate intensive treatment can rescue patient despite poor prognosis...
April 20, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29667750/association-between-body-mass-index-and-pancreatitis-in-children-with-acute-lymphoblastic-leukemia
#19
Pernille R Mogensen, Benjamin O Wolthers, Kathrine Grell, Kjeld Schmiegelow, Thomas L Frandsen
No abstract text is available yet for this article.
April 18, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29660323/nationwide-trends-in-acute-and-chronic-pancreatitis-among-privately-insured-children-and-non-elderly-adults-in-the-united-states-2007-2014
#20
Zachary M Sellers, Donna MacIsaac, Helen Yu, Melody Dehghan, Ke-You Zhang, Rachel Bensen, Jessie J Wong, Cindy Kin, K T Park
BACKGROUND & AIMS: Epidemiologic analyses of acute and chronic pancreatitis (AP and CP) provide insight into causes and strategies for prevention, and affect allocation of resources to its study and treatment. We sought to determine current and accurate incidences of AP and CP, along with the prevalence of CP, in children and adults in the United States. METHODS: We collected data from the Truven MarketScan Research Databases of commercial inpatient and outpatient insurance claims in the United States from 2007 through 2014 (patients 0-64 years old)...
April 13, 2018: Gastroenterology
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