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Pediatric Liver

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https://www.readbyqxmd.com/read/28343205/-biliary-atresia-signs-and-symptoms-diagnosis-clinical-management
#1
Ewa Orłowska, Piotr Czubkowski, Piotr Socha
Biliary atresia is a chronic cholangiopathy leading to progressive fibrosis of both intra- and extrahepatic bile ducts. The cause of the condition is unknown. Fundamental management of biliary atresia is surgical intervention and the outcomes of the treatment depend on the child's age with best results when performed within the first 2 months of life. Thus, the main role of pediatric healthcare is an urgent differential diagnosis and prompt qualification for the surgery, optimal postoperative management and early qualification for the liver transplantation in patients with persistent cholestasis...
2017: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/28337087/medication-dosage-in-overweight-and-obese-children
#2
Kelly L Matson, Evan R Horton, Amanda C Capino
Approximately 31.8% of U.S. children ages 2 to 19 years are considered overweight or obese. This creates significant challenges to dosing medications that are primarily weight based (mg/kg) and in predicting pharmacokinetics parameters in pediatric patients. Obese individuals generally have a larger volume of distribution for lipophilic medications. Conversely, the Vd of hydrophilic medications may be increased or decreased due to increased lean body mass, blood volume, and decrease percentage of total body water...
January 2017: Journal of Pediatric Pharmacology and Therapeutics: JPPT: the Official Journal of PPAG
https://www.readbyqxmd.com/read/28333768/management-of-pediatric-non-alcoholic-fatty-liver-disease-by-academic-hepatologists-in-canada-a-nationwide-survey
#3
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/28333693/pediatric-intestinal-failure-associated-liver-disease
#4
Cathleen M Courtney, Brad W Warner
PURPOSE OF REVIEW: The goal of this review is to provide updates on the definition, pathophysiology, treatment, and prevention of intestinal failure-associated liver disease (IFALD) that are relevant to care of pediatric patients. RECENT FINDINGS: Current literature emphasizes the multifactorial nature of IFALD. The pathogenesis is still largely unknown; however, molecular pathways have been identified. Key to these pathways are proinflammatory cytokines involved in hepatic inflammation and bile acids synthesis such as Toll-like receptor 4 and farnesoid X receptor, respectively...
March 22, 2017: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/28333140/metabolic-and-molecular-insights-into-an-essential-role-of-nicotinamide-phosphoribosyltransferase
#5
Li Q Zhang, Leon Van Haandel, Min Xiong, Peixin Huang, Daniel P Heruth, Charlie Bi, Roger Gaedigk, Xun Jiang, Ding-You Li, Gerald Wyckoff, Dmitry N Grigoryev, Li Gao, Linheng Li, Min Wu, J Steven Leeder, Shui Qing Ye
Nicotinamide phosphoribosyltransferase (NAMPT) is a pleiotropic protein implicated in the pathogenesis of acute respiratory distress syndrome, aging, cancer, coronary heart diseases, diabetes, nonalcoholic fatty liver disease, obesity, rheumatoid arthritis, and sepsis. However, the underlying molecular mechanisms of NAMPT in these physiological and pathological processes are not fully understood. Here, we provide experimental evidence that a Nampt gene homozygous knockout (Nampt(-/-)) resulted in lethality at an early stage of mouse embryonic development and death within 5-10 days in adult mice accompanied by a 25...
March 23, 2017: Cell Death & Disease
https://www.readbyqxmd.com/read/28332273/reducing-the-incidence-of-hepatic-artery-thrombosis-in-pediatric-liver-transplantation-effect-of-microvascular-techniques-and-a-customized-anticoagulation-protocol
#6
William A Ziaziaris, Alexandre Darani, Andrew J A Holland, Angus Alexander, Jonathan Karpelowsky, Pasquale Barbaro, Michael Stormon, Edward O'Loughlin, Albert Shun, Gordon Thomas
We aimed to assess the incidence of HAT over three eras following implementation of microvascular techniques and a customized anticoagulation protocol in a predominantly cadaveric split liver transplant program. We retrospectively reviewed pediatric liver transplants performed between April 1986 and 2016 and analyzed the incidence HAT over three eras. In E1, 1986-2008, each patient received a standard dose of 5 U/kg/h of heparin and coagulation profiles normalized passively. In E2, 2008-2012, microvascular techniques were introduced...
March 22, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28332028/infection-profile-in-chronic-granulomatous-disease-a-23-year-experience-from-a-tertiary-care-center-in-north-india
#7
Amit Rawat, Pandiarajan Vignesh, Avinash Sharma, Jitendra K Shandilya, Madhubala Sharma, Deepti Suri, Anju Gupta, Vikas Gautam, Pallab Ray, Shivaprakash M Rudramurthy, Arunaloke Chakrabarti, Kohsuke Imai, Shigeaki Nonoyama, Osamu Ohara, Yu L Lau, Surjit Singh
PURPOSE: Chronic granulomatous disease (CGD) is an inherited phagocytic disorder characterized by recurrent infections with usually catalase-positive organisms. Infections in CGD from developing countries are expected to be different from those in the Western countries. We report the profile of infections in children diagnosed with CGD from a tertiary care center in North India. METHODOLOGY: Case records of children diagnosed with CGD at Pediatric Immunodeficiency Clinic, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India, from August 1993 to April 2016 (23 years) were analyzed...
March 22, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28331447/novel-biliary-reconstruction-techniques-during-liver-transplantation
#8
Ian C Carmody, John Romano, Humberto Bohorquez, Emily Bugeaud, David S Bruce, Ari J Cohen, John Seal, Trevor W Reichman, George E Loss
BACKGROUND: Biliary complications remain a significant problem following liver transplantation. Several surgical options can be used to deal with a significant size mismatch between the donor and recipient bile ducts during the biliary anastomosis. We compared biliary transposition to recipient biliary ductoplasty in cadaveric liver transplant. METHODS: A total of 33 reconstructions were performed from January 1, 2005 to December 31, 2013. In the biliary transposition group (n=23), 5 reconstructions were performed using an internal stent (5 or 8 French pediatric feeding tube), and 18 were performed without...
2017: Ochsner Journal
https://www.readbyqxmd.com/read/28331334/safety-efficacy-and-clinical-utility-of-asparaginase-in-the-treatment-of-adult-patients-with-acute-lymphoblastic-leukemia
#9
REVIEW
Jamie Koprivnikar, James McCloskey, Stefan Faderl
Adults with acute lymphoblastic leukemia (ALL) are known to have inferior outcomes compared to the pediatric population. Although the reasons for this are likely manyfold, the agents utilized and the increased intensity of pediatric treatments compared to adult treatments are likely significant contributing factors. Asparaginase, an enzyme that converts asparagine to aspartic acid, forms the backbone of almost all pediatric regimens and works by depleting extracellular asparagine, which ALL cells are unable to synthesize...
2017: OncoTargets and Therapy
https://www.readbyqxmd.com/read/28331102/clinical-features-of-women-with-turner-syndrome-experiencing-transition-period-in-japan
#10
Satsuki Nishigaki, Takashi Hamazaki, Akitoshi Tsuruhara, Toshiko Yoshida, Takuji Imamura, Hiroshi Inada, Keinosuke Fujita, Haruo Shintaku
Turner syndrome results from the entire or partial loss of the second X chromosome, and is associated with a number of medical problems. Affected women require long-term medical follow-up. This study investigated the status of medical follow-up focusing on the transition for young adult women with Turner syndrome (TS). The clinical profiles of 63 women with TS over the age of 16 were retrospectively examined. Thirty-three women are continuously followed by pediatric endocrinologists at our pediatric division...
March 22, 2017: Endocrine Journal
https://www.readbyqxmd.com/read/28329452/effect-of-prime-blood-storage-duration-on-clinical-outcome-after-pediatric-cardiac-surgery
#11
Arvind Kumar Bishnoi, Pankaj Garg, Kartik Patel, Parth Solanki, Jigar Surti, Atul Solanki, Komal Shah, Sanjay Patel
BACKGROUND: In this study, we tested the hypothesis that in pediatric patients undergoing cardiac surgery using cardiopulmonary bypass (CPB) with blood prime, the storage duration of the packed red blood cells (PRBCs) used in prime led to differences in postoperative complications and metabolic profiles of the patients. METHODS: For this prospective observational study we included 400 pediatric patients undergoing cardiac operations using CPB and requiring PRBCs prime...
March 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28328064/a-bibliometric-analysis-of-pediatric-liver-transplantation-publications
#12
Dermot T McDowell, Alexandre Darani, Albert Shun, Gordon Thomas, Andrew J A Holland
Citation counts can identify landmark papers. The aim of this study was to identify and characterize the top-cited articles in the pediatric liver transplantation literature. A search strategy for the Scopus(®) database was designed for pediatric liver transplantation publications from 1945 to 2014. The 50 top-cited articles were analyzed. Author co-citation analysis was performed using VOSviewer techniques. There were 2896 articles published between 1969 and 2015. The mean citation count of the top 50 cited articles was 166 (range 95-635)...
March 22, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28327291/solid-pseudopapillary-neoplasm-of-the-pancreas-a-clinicopathological-review-of-20-cases-including-rare-examples
#13
Ayca Ersen, Anil Aysal Agalar, Erdener Ozer, Cihan Agalar, Tarkan Unek, Tufan Egeli, Mucahit Ozbilgin, Ibrahim Astarcioglu, Mustafa Olguner, Funda Obuz, Ozgul Sagol
AIMS: Solid-pseudopapillary neoplasm (SPN) is an uncommon malignant tumor of the pancreas with a favorable prognosis unlike other pancreatic neoplasms. We investigated the clinicopathological features of 20 patients with SPN in details. METHODS: The patients diagnosed as SPN in Dokuz Eylul University Hospital between January 2005 and March 2016 were reviewed in terms of clinical and histopathological data. RESULTS: Mean age of the patients was 33...
November 2016: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28326955/a-challenging-case-of-severe-infantile-cholestasis-in-alpha-1-antitrypsin-deficiency
#14
Zahida Khan, Veena L Venkat, Kyle A Soltys, Donna B Stolz, Sarangarajan Ranganathan
Jaundice in the newborn period can be physiologic and is often due to benign causes. Jaundice due to conjugated hyperbilirubinemia extending beyond the second week of life may be an early sign of several cholestatic or metabolic liver diseases, and it requires logical and timely analysis so that specific treatments can be initiated. Alpha-1 antitrypsin deficiency is the most common genetic cause of pediatric liver disease and transplantation, and it must be considered when evaluating cholestatic infants. Here, we present an unusual case of alpha-1 antitrypsin deficiency with severe infantile cholestasis and rapid decompensation in the first 4 months of life, where in-depth but timely diagnosis was crucial for the appropriate intervention to take place...
March 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28325786/impact-on-hospital-resources-of-systematic-evaluation-and-management-of-suspected-nonaccidental-trauma-in-patients-less-than-4-years-of-age
#15
Bethann M Pflugeisen, Mauricio A Escobar, Dustin Haferbecker, Yolanda Duralde, Elizabeth Pohlson
OBJECTIVE: There has been an increasing movement worldwide to create systematic screening and management procedures for atypical injury patterns in children with the hope of better detecting and evaluating nonaccidental trauma (NAT). A legitimate concern for any hospital considering implementation of a systematic evaluation process is the impact on already burdened hospital resources. We hypothesized that implementation of a guideline that uses red flags related to history, physical, or radiologic findings to trigger a standardized NAT evaluation of patients <4 years would not negatively affect resource utilization at our level II pediatric trauma center...
March 21, 2017: Hospital Pediatrics
https://www.readbyqxmd.com/read/28324110/effects-of-metreleptin-in-pediatric-patients-with-lipodystrophy
#16
Rebecca J Brown, Cristina Adelia Meehan, Elaine Cochran, Kristina I Rother, David E Kleiner, Mary Walter, Phillip Gorden
Context: Lipodystrophy syndromes are rare disorders of deficient adipose tissue. Metreleptin, a human analog of leptin, improved metabolic abnormalities in mixed cohorts of leptin-deficient children and adults with lipodystrophy. Objective: Determine effects of metreleptin on diabetes, hyperlipidemia, non-alcoholic fatty liver disease (NAFLD), growth, and puberty in leptin-deficient pediatric patients with lipodystrophy. Design: Prospective, single-arm, open-label studies with continuous enrollment since 2000...
January 23, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28322576/the-effects-of-exercise-on-abdominal-fat-and-liver-enzymes-in-pediatric-obesity-a-systematic-review-and-meta-analysis
#17
Katherine González-Ruiz, Robinson Ramírez-Vélez, Jorge Enrique Correa-Bautista, Mark D Peterson, Antonio García-Hermoso
BACKGROUND: Despite the prevalence of obesity and the multiple position stands promoting exercise for the treatment of obesity and hepatic function, a meta-analytic approach has not previously been used to examine the effects in the pediatric population. The aim of the study was to determine the effectiveness of exercise interventions on abdominal fat, liver enzymes, and intrahepatic fat in overweight and obese youth. MATERIALS AND METHODS: A computerized search was made using three databases...
March 21, 2017: Childhood Obesity
https://www.readbyqxmd.com/read/28321975/the-medication-level-variability-index-mlvi-predicts-poor-liver-transplant-outcomes-a-prospective-multi-site-study
#18
Eyal Shemesh, John C Bucuvalas, Ravinder Anand, George V Mazariegos, Estella M Alonso, Robert S Venick, Miguel Reyes-Mugica, Rachel A Annunziato, Benjamin L Shneider
Nonadherence to immunosuppressant medications is a leading cause of poor long-term outcomes in transplant recipients. The Medication Level Variability Index (MLVI) provides a vehicle for transplant outcome risk-stratification through continuous assessment of adherence. The MALT (Medication Adherence in children who had a Liver Transplant) prospective multi-site study evaluated whether MLVI predicts Late Acute Rejection (LAR). 400 pediatric (1-17 year old) liver transplant recipients were enrolled and followed for 2 years...
March 20, 2017: American Journal of Transplantation
https://www.readbyqxmd.com/read/28321802/sepsis-risk-factors-in-infants-with-congenital-diaphragmatic-hernia
#19
Michaël Levy, Nolwenn Le Sache, Mostafa Mokhtari, Guy Fagherazzi, Gaelle Cuzon, Benjamin Bueno, Virginie Fouquet, Alexandra Benachi, Sergio Eleni Dit Trolli, Pierre Tissieres
BACKGROUND: Congenital diaphragmatic hernia (CDH) is a rare congenital anomaly and remains among the most challenging ICU-managed disease. Beside severe pulmonary hypertension, lung hypoplasia and major abdominal surgery, infective complications remain major determinants of outcome. However, the specific incidence of sepsis as well as associated risk factors is unknown. METHODS: This prospective, 4-year observational study took place in the pediatric intensive care and neonatal medicine department of the Paris South University Hospitals (Le Kremlin-Bicêtre, France), CDH national referral center and involved 62 neonates with CDH...
December 2017: Annals of Intensive Care
https://www.readbyqxmd.com/read/28321162/mortality-associated-with-gastrointestinal-bleeding-in-children-a-retrospective-cohort-study
#20
Thomas M Attard, Mikaela Miller, Chaitanya Pant, Ashwath Kumar, Mike Thomson
AIM: To determine the clinical characteristics of children with gastrointestinal bleeding (GIB) who died during the course of their admission. METHODS: We interrogated the Pediatric Hospital Information System database, including International Classification of Diseases, Current Procedural Terminology and Clinical Transaction Classification coding from 47 pediatric tertiary centers extracting the population of patients (1-21 years of age) admitted (inpatient or observation) with acute, upper or indeterminate GIB (1/2007-9/2015)...
March 7, 2017: World Journal of Gastroenterology: WJG
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