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Paediatric pancreatitis

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https://www.readbyqxmd.com/read/30116405/serum-level-of-d-lactate-in-patients-with-cystic-fibrosis-preliminary-data
#1
Sabina Więcek, Jerzy Chudek, Halina Woś, Maria Bożentowicz-Wikarek, Bożena Kordys-Darmolinska, Urszula Grzybowska-Chlebowczyk
D-Lactate is produced by the intestinal biota and later absorbed into circulation. Some patients with cystic fibrosis (CF) develop exocrine pancreatic insufficiency that may disturb the gut microbiome and enhance the production of D-lactate. However, this concept has not been studied yet. The aim of the study was to assess D-lactate concentration in relation to the occurrence of clinical features, activity of CF, and diet composition in paediatric patients. Patients and Method . Serum concentrations of D-lactate were measured in 38 CF patients (19 girls and 19 boys) from 6 months to 18 years of age...
2018: Disease Markers
https://www.readbyqxmd.com/read/29907148/a-case-of-metaplastic-atrophic-gastritis-in-immune-dysregulation-polyendocrinopathy-enteropathy-x-linked-ipex-syndrome
#2
Youyou Luo, Jie Chen, Youhong Fang, Jingan Lou, Jindan Yu
BACKGROUND: Autoimmune metaplastic atrophic gastritis is a chronic progressive inflammatory condition. The clinical spectrum includes pernicious anemia, atrophic gastritis, antibodies to parietal cell antigens and intrinsic factor, achlorhydria, hypergastrinemia and carcinoma. It is rare in paediatric cohorts. CASE PRESENTATION: We present the case of a boy with metaplastic atrophic gastritis in whom immune dysregulation, polyendocrinopathy, enteropathy, X-linked(IPEX) syndrome was confirmed by FOXP3 gene mutation...
June 15, 2018: BMC Pediatrics
https://www.readbyqxmd.com/read/29767474/mesenchymal-stromal-cells-from-shwachman-diamond-syndrome-patients-fail-to-recreate-a-bone-marrow-niche-in-vivo-and-exhibit-impaired-angiogenesis
#3
Donatella Bardelli, Erica Dander, Cristina Bugarin, Claudia Cappuzzello, Alice Pievani, Grazia Fazio, Paolo Pierani, Paola Corti, Piero Farruggia, Carlo Dufour, Simone Cesaro, Marco Cipolli, Andrea Biondi, Giovanna D'Amico
Shwachman-Diamond syndrome (SDS) is a rare multi-organ recessive disease mainly characterised by pancreatic insufficiency, skeletal defects, short stature and bone marrow failure (BMF). As in many other BMF syndromes, SDS patients are predisposed to develop a number of haematopoietic malignancies, particularly myelodysplastic syndrome and acute myeloid leukaemia. However, the mechanism of cancer predisposition in SDS patients is only partially understood. In light of the emerging role of mesenchymal stromal cells (MSCs) in the regulation of bone marrow homeostasis, we assessed the ability of MSCs derived from SDS patients (SDS-MSCs) to recreate a functional bone marrow niche, taking advantage of a murine heterotopic MSC transplant model...
July 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29752349/paediatric-pancreatic-neuroendocrine-tumours-in-von-hippel-lindau-disease
#4
LETTER
Samuel M O'Toole, Anju Sahdev, Satya Bhattacharya, Roger Feakins, Evelien F Gevers, William M Drake
No abstract text is available yet for this article.
September 2018: Endocrine-related Cancer
https://www.readbyqxmd.com/read/29737317/first-robotic-pylorus-preserving-pancreaticoduodenectomy-for-frantz-s-tumour-in-an-adolescent-girl
#5
Palanivelu Chinnusamy, Sumanta Dey, Bhushan Chittawadagi, Srivatsan Gurumurthy, Sandeep Sabnis, Senthilnathan Palanisamy
Solid pseudopapillary tumour (SPT) is one of the uncommon benign cystic neoplasms of pancreas occurring predominantly in young females. Being benign in nature, surgical resection is the treatment of choice with excellent 5-year survival. A 14-year-old girl presented with pain abdomen for 1 week. On evaluation, she was found to have a large SPT involving head and uncinate process of Pancreas She underwent robotic pylorus preserving pancreaticoduodenectomy (R-PPPD) with da Vinci® Si Robotic System (Intuitive Surgical, Sunnyvale, CA, USA)...
May 4, 2018: Journal of Minimal Access Surgery
https://www.readbyqxmd.com/read/29666173/hereditary-pancreatitis-in-paediatrics-the-causative-role-of-p-leu104pro-mutation-of-cationic-trypsinogen-gene-also-in-young-subjects
#6
Ausilia Enea, Antonio Pizzol, Michele Pinon, Fabio Cisarò, Francesco Tandoi, Carlo Arduino, Pier Luigi Calvo
No abstract text is available yet for this article.
April 17, 2018: Gut
https://www.readbyqxmd.com/read/29657614/acute-pancreatitis-in-children
#7
Urszula Grzybowska-Chlebowczyk, Martyna Jasielska, Anna Flak-Wancerz, Sabina Więcek, Katarzyna Gruszczyńska, Wojciech Chlebowczyk, Halina Woś
Introduction: The acute pancreatitis is a rare disease, but it has started to be diagnosed more often in children. Aim: The aim of the study was single-centre, retrospective analysis of the incidence, aetiology, and clinical course of acute pancreatitis in children. Material and methods: We analysed the medical records of patients with acute pancreatitis hospitalised in the Gastroenterology Unit of the Paediatrics Department, Medical University of Silesia from Jan 2004 to Dec 2013...
2018: Przegla̜d Gastroenterologiczny
https://www.readbyqxmd.com/read/29560645/comparison-of-side-viewing-duodenoscope-and-single-balloon-enteroscope-to-perform-ercp-in-patients-with-billroth-ii-gastrectomy
#8
MULTICENTER STUDY
G Mbatshi, E J Macken, H U De Schepper, H Piessevaux, P H Deprez, T G Moreels
Endoscopic retrograde cholangiopancreatography (ERCP) in Billroth II patients is challenging and different endoscopes can be used. We retrospectively analysed 67 ERCP procedures in 38 Billroth II patients focussing on endoscope type and respective technical success and adverse event rate. 33 (49.2 %) ERCPs were performed using a duodenoscope, 87.9 % were successful and 3 were completed with the single-balloon enteroscope. 28 (41.8 %) ERCPs were performed with the single-balloon enteroscope, 82.1 % were successful and 2 were completed with a paediatric colonoscope...
October 2017: Acta Gastro-enterologica Belgica
https://www.readbyqxmd.com/read/29434062/glucose-tolerance-insulin-secretion-sensitivity-characteristics-in-indian-children-with-cystic-fibrosis-a-pilot-study
#9
Vandana Jain, Santosh Kumar, Naval K Vikram, Mani Kalaivani, Surya Prakash Bhatt, Rajni Sharma, Kumar Kabra Sushil
Background & objectives: Cystic fibrosis (CF) is a life-limiting genetic condition resulting in chronic respiratory infections, pancreatic enzyme insufficiency and associated complications. This pilot study was undertaken to assess the glucose tolerance and insulin secretion and sensitivity among Indian children with CF. Methods: Children with CF under regular follow up at the Paediatric Pulmonology Clinic of a tertiary care hospital in New Delhi, India, were enrolled...
October 2017: Indian Journal of Medical Research
https://www.readbyqxmd.com/read/29426291/paediatric-pancreaticobiliary-endoscopy-a-21-year-experience-from-a-tertiary-hepatobiliary-centre-and-systematic-literature-review
#10
REVIEW
Margaret G Keane, Mayur Kumar, Natascha Cieplik, Douglas Thorburn, Gavin J Johnson, George J Webster, Michael H Chapman, Keith J Lindley, Stephen P Pereira
BACKGROUND: In adults ERCP and endoscopic ultrasound (EUS) are standard methods of evaluating and treating many hepatopancreaticobiliary (HPB) conditions. HPB disease is being diagnosed with increasing frequency in children but information about role of ERCP and EUS and their outcomes in this population remain limited. Therefore the aims of this study were to describe the paediatric ERCP and EUS experience from a large tertiary referral HPB centre, and to systematically compare outcomes with those of other published series...
February 9, 2018: BMC Pediatrics
https://www.readbyqxmd.com/read/29398271/tread-carefully-a-functional-variant-in-the-human-nadph-oxidase-4-nox4-is-not-disease-causing
#11
Michael Nafisinia, Minal Juliet Menezes, Wendy Anne Gold, Lisa Riley, Joshua Hatch, John Cardinal, David Coman, John Christodoulou
In this study, we report a paediatric patient with a lethal phenotype of respiratory distress, failure to thrive, pancreatic insufficiency, liver dysfunction, hypertrophic cardiomyopathy, bone marrow suppression, humoral and cellular immune deficiency. To identify the genetic basis of this unusual clinical phenotype and potentially make available the option of future prenatal testing, whole exome sequencing (WES) was used followed by functional studies in a bid to confirm pathogenicity. The WES we identified a homozygous novel variant, AK298328; c...
March 2018: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/29302507/blunt-trauma-pancreas-in-children-is-non-operative-management-appropriate-for-all-grades
#12
Ravi Kumar Garg, Jai Kumar Mahajan
Purpose: Blunt trauma of pancreas in children is uncommon and its management varies from observational to early operative intervention. We analysed the feasibility and outcome of non-operative management in all grades of paediatric pancreatic injuries. Methods: A total of 15 patients of pancreatic trauma seen in a Paediatric Surgery Unit were retrospectively analyzed. Results: Age of the patients ranged from 3-11 years (mean, 7.7 years). The mode of injury was local trauma in 9 children...
December 2017: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/29285688/pancreatoblastoma-an-unusual-diagnosis-in-an-adult-patient
#13
Gonçalo Nunes, Hélder Coelho, Marta Patita, Rita Barosa, Pedro Pinto Marques, Lídia Roque Ramos, Maria José Brito, Aurora Tomaz, Jorge Fonseca
The authors describe a 31-year-old man admitted due to progressive weight loss, diarrhea and massive hepatomegaly. Laboratory data showed anemia (haemoglobin 11.7 g/dl), abnormal liver tests (total bilirubin 1.4 g/dl, aspartate aminotransferase 70 U/l, alanine aminotransferase 37 U/l and alkaline phosphatase 520 U/l). Abdominal ultrasound (US) displayed a large heterogeneous liver with a segment IV 25 mm nodule. Magnetic resonance revealed a 4 cm pancreatic tail mass and several liver nodules consistent with metastasis...
April 2018: Clinical Journal of Gastroenterology
https://www.readbyqxmd.com/read/29228226/vedolizumab-associated-pancreatitis-in-paediatric-ulcerative-colitis-functional-selectivity-of-the-%C3%AE-4%C3%AE-7integrin-and-madcam-1-pathway
#14
Robert N Lopez, Nitin Gupta, Daniel A Lemberg
No abstract text is available yet for this article.
March 28, 2018: Journal of Crohn's & Colitis
https://www.readbyqxmd.com/read/29193669/serial-lipase-for-pancreatitis-not-enough-evidence
#15
REVIEW
Tishanthan Pathmarajah, Marwan Idrees, Sana Nasim, Dieter G Weber
BACKGROUND: Lipase is both a sensitive and specific marker for diagnosing pancreatitis. However, the benefit of serial lipase in both monitoring and defining prognosis remains undetermined. This systematic review was conducted to further evaluate this potential application. In addition, this review also looked into the benefits of serial lipase in the subgroup of traumatic pancreatitis. METHODS: PubMed, Cochrane Library and Google Scholar were systematically searched for related articles, between January 1995 and December 2015, according to Preferred Reporting Items for Systematic Reviews and Meta-analyses standards...
June 2018: ANZ Journal of Surgery
https://www.readbyqxmd.com/read/29123588/infliximab-treatment-in-a-paediatric-patient-with-ulcerative-colitis-who-developed-acute-pancreatitis-due-to-azathioprine-during-follow-up
#16
Ahmet Basturk, Aygen Yilmaz, Meryem Keceli, Reha Artan
No abstract text is available yet for this article.
2017: Przegla̜d Gastroenterologiczny
https://www.readbyqxmd.com/read/28968289/chymotrypsinogen-c-genetic-variants-including-c-180tt-are-strongly-associated-with-chronic-pancreatitis-in-pediatric-patients
#17
Alicja Monika Grabarczyk, Grzegorz Oracz, Katarzyna Wertheim-Tysarowska, Aleksandra Anna Kujko, Karolina Wejnarska, Elwira Kolodziejczyk, Jerzy Bal, Dorota Koziel, Artur Kowalik, Stanislaw Gluszek, Agnieszka Magdalena Rygiel
OBJECTIVES: Genetic studies in adults/adolescent patients with chronic pancreatitis (CP) identified chymotrypsinogen C (CTRC) genetic variants but their association with CP risk has been difficult to replicate. To evaluate the risk of CP associated with CTRC variants in CP pediatric patients-control study. METHODS: The distribution of CTRC variants in CP pediatric cohort (n = 136, median age at CP onset 8 years) with no history of alcohol/smoking abuse was compared with controls (n = 401, median age 45)...
December 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28855921/diagnosis-and-treatment-of-hyperinsulinaemic-hypoglycaemia-and-its-implications-for-paediatric-endocrinology
#18
REVIEW
Huseyin Demirbilek, Sofia A Rahman, Gonul Gulal Buyukyilmaz, Khalid Hussain
Glucose homeostasis requires appropriate and synchronous coordination of metabolic events and hormonal activities to keep plasma glucose concentrations in a narrow range of 3.5-5.5 mmol/L. Insulin, the only glucose lowering hormone secreted from pancreatic β-cells, plays the key role in glucose homeostasis. Insulin release from pancreatic β-cells is mainly regulated by intracellular ATP-generating metabolic pathways. Hyperinsulinaemic hypoglycaemia (HH), the most common cause of severe and persistent hypoglycaemia in neonates and children, is the inappropriate secretion of insulin which occurs despite low plasma glucose levels leading to severe and persistent hypoketotic hypoglycaemia...
2017: International Journal of Pediatric Endocrinology
https://www.readbyqxmd.com/read/28320633/nutritional-status-nutrient-intake-and-use-of-enzyme-supplements-in-paediatric-patients-with-cystic-fibrosis-a-european-multicentre-study-with-reference-to-current-guidelines
#19
Joaquim Calvo-Lerma, Jessie M Hulst, Inês Asseiceira, Ine Claes, Maria Garriga, Carla Colombo, Victoria Fornés, Sandra Woodcock, Tiago Martins, Mieke Boon, Mar Ruperto, Sylvia Walet, Chiara Speziali, Peter Witters, Etna Masip, Celeste Barreto, Kris de Boeck, Carmen Ribes-Koninckx
BACKGROUND: The New European guidelines have established the most updated recommendations on nutrition and pancreatic enzyme replacement therapy (PERT) in CF. In the context of MyCyFAPP project - a European study in children with CF aimed at developing specific tools for improvement of self-management - the objective of the current study was to assess nutritional status, daily energy and macronutrient intake, and PERT dosing with reference to these new guidelines. METHODS: Cross sectional study in paediatric patients with CF from 6 European centres...
July 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28216481/autoimmune-pancreatitis-in-the-paediatric-population-review-of-the-literature-and-own-experience
#20
REVIEW
Elwira Kołodziejczyk, Karolina Wejnarska, Grzegorz Oracz
Autoimmune pancreatitis (AIP) is a rare chronic inflammatory autoimmune disease of the pancreas. It is estimated that it accounts for 2-6% of all the chronic pancreatitis (CP) cases in adult patients. AIP is usually characterized by sudden onset. The presenting symptoms most commonly include painless jaundice, general weakness and loss of weight. Imaging studies often reveal diffuse enlargement or focal changes of pancreatic parenchyma. In view of the clinical manifestation and changes documented on imaging, AIP should be considered after performing a differential diagnosis from pancreatic cancer or inflammatory masses in the course of CP...
2016: Developmental Period Medicine
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