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Pediatric liver disease

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https://www.readbyqxmd.com/read/28065514/review-of-childhood-obesity-from-epidemiology-etiology-and-comorbidities-to-clinical-assessment-and-treatment
#1
REVIEW
Seema Kumar, Aaron S Kelly
Childhood obesity has emerged as an important public health problem in the United States and other countries in the world. Currently 1 in 3 children in the United States is afflicted with overweight or obesity. The increasing prevalence of childhood obesity is associated with emergence of comorbidities previously considered to be "adult" diseases including type 2 diabetes mellitus, hypertension, nonalcoholic fatty liver disease, obstructive sleep apnea, and dyslipidemia. The most common cause of obesity in children is a positive energy balance due to caloric intake in excess of caloric expenditure combined with a genetic predisposition for weight gain...
January 5, 2017: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/28052604/optn-srtr-2015-annual-data-report-liver
#2
W R Kim, J R Lake, J M Smith, M A Skeans, D P Schladt, E B Edwards, A M Harper, J L Wainright, J J Snyder, A K Israni, B L Kasiske
Several notable developments in adult liver transplantation in the US occurred in 2015. The year saw the largest number of liver transplants to date, leading to reductions in median waiting time, in waitlist mortality for all model for end-stage liver disease categories, and in the number of candidates on the waiting list at the end of the year. Numbers of additions to the waiting list and of liver transplants performed in patients with hepatitis C virus infection decreased for the first time in recent years...
January 2017: American Journal of Transplantation
https://www.readbyqxmd.com/read/28052602/optn-srtr-2015-annual-data-report-intestine
#3
J M Smith, M A Skeans, S P Horslen, E B Edwards, A M Harper, J J Snyder, A K Israni, B L Kasiske
Intestine and intestine-liver transplant remains important in the treatment of intestinal failure, despite decreased morbidity associated with parenteral nutrition. In 2015, 196 new patients were added to the intestine transplant waiting list, with equal numbers waiting for intestine and intestine-liver transplant. Among prevalent patients on the list at the end of 2015, 63.3% were waiting for an intestine transplant and 36.7% were waiting for an intestine-liver transplant. The pretransplant mortality rate decreased dramatically over time for all age groups...
January 2017: American Journal of Transplantation
https://www.readbyqxmd.com/read/28052307/ultrashort-bowel-syndrome-outcome-in-children-treated-in-a-multidisciplinary-intestinal-rehabilitation-unit
#4
Mariela Dore, Paloma Triana Junco, Ane Andres Moreno, Vanesa Nuñez Cerezo, Martha Romo Muñoz, Alba Sánchez Galán, Alejandra Vilanova Sánchez, Gerardo Prieto, Esther Ramos, Francisco Hernandez, Leopoldo Martínez Martínez, Manuel Lopez Santamaria
Aim Short bowel syndrome (SBS) is the leading cause of intestinal failure (IF) in the pediatric population. Our aim was to review long-term outcome of ultrashort bowel syndrome (USBS) in an Intestinal Rehabilitation Unit (IRU). Patients and Methods Retrospective study of patients with USBS (defined as < 10 cm of remnant small bowel) treated between 2000 and 2015. Demographic data, clinical, and treatment variables including parenteral nutrition (PN), surgical techniques, and intestinal transplantation (IT) were analyzed...
January 4, 2017: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28044976/-kidsetransplant-a-serious-game-for-liver-transplanted-children
#5
Ana-Maria Calinescu, Valérie McLin, Stéphane Spahni, Thomas Boggini, Mirana Randriambelonoro, Roxane Jaquier-Grant, Michèle Steiner, Antoine Geissbuhler, Barbara E Wildhaber
Serious games are increasingly used in pediatric care, especially to educate young patients with chronic diseases, to improve their understanding of the disease and develop independence in disease management, as it can ultimately improve clinical results. This is also true for liver transplanted children and adolescents, who are not only challenged by their therapeutic burden, but who need to face transition to adult care, often without remembering their transplantation, which was performed, most of the time, very early in their life...
December 2016: Médecine Sciences: M/S
https://www.readbyqxmd.com/read/28040394/long-term-hematological-visceral-and-growth-outcomes-in-children-with-gaucher-disease-type-3-treated-with-imiglucerase-in-the-international-collaborative-gaucher-group-gaucher-registry
#6
Amal El-Beshlawy, Anna Tylki-Szymanska, Ashok Vellodi, Nadia Belmatoug, Gregory A Grabowski, Edwin H Kolodny, Julie L Batista, Gerald F Cox, Pramod K Mistry
In Gaucher disease (GD), deficiency of lysosomal acid β-glucosidase results in a broad phenotypic spectrum that is classified into three types based on the absence (type 1 [GD1]) or presence and severity of primary central nervous system involvement (type 2 [GD2], the fulminant neuronopathic form, and type 3 [GD3], the milder chronic neuronopathic form). Enzyme replacement therapy (ERT) with imiglucerase ameliorates and prevents hematological and visceral manifestations in GD1, but data in GD3 are limited to small, single-center series...
December 6, 2016: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28039895/genetic-profiling-of-children-with-advanced-cholestatic-liver-disease
#7
Mohammad Shagrani, Jessica Burkholder, Dieter Broering, Mohamed Abouelhoda, Tariq Faquih, Mohamed El-Kalioby, Shazia N Subhani, Ewa Goljan, Renad Albar, Dorota Monies, Nejat Mazhar, Basma S AlAbdulaziz, Khalid Al Abdelrahman, Nada Altassan, Fowzan S Alkuraya
Advanced cholestatic liver disease is a leading referral to pediatric liver transplant centers. Recent advances in the genetic classification of this group of disorders promise a highly personalized management although the genetic heterogeneity also poses a diagnostic challenge. Using a next-generation sequencing-based multi-gene panel, we performed retrospective analysis of 98 pediatric patients who presented with advanced cholestatic liver disease. A likely causal mutation was identified in the majority (61%), spanning many genes including ones that have only rarely been reported to cause cholestatic liver disease e...
December 30, 2016: Clinical Genetics
https://www.readbyqxmd.com/read/28032939/sequential-analysis-of-variable-markers-for-predicting-outcomes-in-pediatric-patients-with-acute-liver-failure
#8
Hajime Uchida, Seisuke Sakamoto, Akinari Fukuda, Kengo Sasaki, Takanobu Shigeta, Shunsuke Nosaka, Masaya Kubota, Atsuko Nakazawa, Satoshi Nakagawa, Mureo Kasahara
BACKGROUND & AIMS: Our aim was to analyze serial changes in the predictive variables and a scoring system retrospectively adapted to evaluate outcomes in pediatric patients with acute liver failure (ALF). METHODS: We retrospectively collected data on 65 patients with ALF. The 65 patients were divided into 2 groups according to the need for liver transplantation (LT) as follows: LT group (n = 54) and non-LT group (n = 11). The early determination scoring system of the indications for LT proposed by the Intractable Hepato-Biliary Diseases Study Group of Japan (JIHBDSG) was used in our study...
December 29, 2016: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/28031453/acute-liver-failure-meets-soph-syndrome-a-case-report-on-an-intermediate-phenotype
#9
Fanny Kortüm, Iris Marquardt, Malik Alawi, Georg Christoph Korenke, Stephanie Spranger, Peter Meinecke, Kerstin Kutsche
Acute liver failure (ALF) is a life-threatening condition in the absence of preexisting liver disease in children. The main clinical presentation comprises hepatic dysfunction, elevated liver biochemical values, and coagulopathy. The etiology of ALF remains unclear in most affected children; however, the recent identification of mutations in the neuroblastoma amplified sequence (NBAS) gene in autosomal recessively inherited ALF has shed light on the cause of a subgroup of fever-triggered pediatric ALF episodes...
January 2017: Pediatrics
https://www.readbyqxmd.com/read/28030482/pediatric-parenteral-nutrition-associated-liver-disease
#10
Jill C Israelite
Pediatric parenteral nutrition-associated liver disease (PNALD) is typically defined as a decrease in bile flow that is independent of a mechanical obstruction and of any other underlying liver disease. It is most often seen in pediatric patients receiving parenteral nutrition support. Up to 50% to 66% of children receiving long-term parenteral nutrition are reported to be diagnosed with PNALD. The goal of treatment for PNALD is advancement to full enteral nutrition and elimination of dependence on parenteral nutrition support...
January 2017: Journal of Infusion Nursing: the Official Publication of the Infusion Nurses Society
https://www.readbyqxmd.com/read/28027602/is-size-the-only-determinant-of-delayed-abdominal-closure-in-pediatric-liver-transplant
#11
Shirin Elizabeth Khorsandi, Arthur William Raven Day, Miriam Cortes, Akash Deep, Anil Dhawan, Hector Vilca-Melendez, Nigel Heaton
The aim was to determine the factors associated with the use of delayed abdominal closure in pediatric liver transplant and whether this affected outcome. From a prospectively maintained database, transplants performed in children (≤ 18 years) were identified (October 2010 - March 2015). Primary abdominal closure was defined as mass closure performed at time of transplant. Delayed abdominal closure was defined as mass closure not initially performed at the same time as transplant. 230 children underwent liver transplantation...
December 27, 2016: Liver Transplantation
https://www.readbyqxmd.com/read/28027587/analysis-of-surgical-interruption-of-the-enterohepatic-circulation-as-a-treatment-for-pediatric-cholestasis
#12
Kasper S Wang, Greg Tiao, Lee M Bass, Paula M Hertel, Douglas Mogul, Nanda Kerkar, Matthew Clifton, Colleen Azen, Laura Bull, Philip Rosenthal, Dylan Stewart, Riccardo Superina, Ronen Arnon, Molly Bozic, Mary L Brandt, Patrick A Dillon, Annie Fecteau, Kishore Iyer, Binita Kamath, Saul Karpen, Frederick Karrer, Kathleen M Loomes, Cara Mack, Peter Mattei, Alexander Miethke, Kyle Soltys, Yumirle P Turmelle, Karen West, Jessica Zagory, Cat Goodhue, Benjamin L Shneider
: To evaluate the efficacy of non-transplant surgery for pediatric cholestasis, 58 clinically diagnosed children, including 20 Alagille syndrome (ALGS), 16 Familial Intrahepatic Cholestasis-1 (FIC1), 18 Bile Salt Export Pump (BSEP) disease, & 4 others with low γ-glutamyl transpeptidase disease (levels <100 U/L), were identified across 14 Childhood Liver Disease Research Network (ChiLDReN) centers. Data were collected retrospectively from individuals who collectively had 39 partial external biliary diversions (PEBD), 11 ileal exclusions (IE) and 7 gallbladder-to-colon diversion (GBC)...
December 27, 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28006876/health-related-quality-of-life-after-pediatric-liver-transplantation-a-systematic-review
#13
REVIEW
Arpita Parmar, Shannon Marie Vandriel, Vicky Lee Ng
With improved survival rates after pediatric liver transplantation (LT), attention is targeting improving the health-related quality of life (HRQOL) as an outcome metric. We conducted a systematic review of the literature to examine HRQOL after pediatric LT, focusing on assessment tools, and factors associated with HRQOL.A literature search was conducted through PubMed, Web of Science, OVID, and Google Scholar for all studies matching the eligibility criteria between January 2004 and September 2016. Titles and abstracts were screened independently by two authors and consensus for included studies was achieved through discussion...
December 22, 2016: Liver Transplantation
https://www.readbyqxmd.com/read/27998312/rare-presentation-of-subcapsular-hepatic-steatosis-in-a-woman-with-uncontrolled-diabetes-without-peritoneal-dialysis-a-case-report
#14
Varun Chowdhary, Jennifer S Golia Pernicka, Richa Sharma
BACKGROUND: Subcapsular hepatic steatosis is a rare atypical pattern of fatty deposition of the liver reported in patients with diabetic nephropathy receiving peritoneal dialysis with intraperitoneal insulin. To date, there has been only one pediatric and zero adult cases of subcapsular hepatic steatosis with no history of continuous ambulatory peritoneal dialysis. We report the first published case of subcapsular hepatic steatosis in an adult diabetic patient without any history of peritoneal dialysis or evidence of chronic renal disease...
December 20, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27997531/redefining-short-bowel-syndrome-in-the-21st-century
#15
REVIEW
Valeria C Cohran, Joshua D Prozialeck, Conrad R Cole
In 1968, Wilmore and Dudrick reported an infant sustained by parenteral nutrition (PN) providing a potential for survival for children with significant intestinal resections. Increasing usage of TPN over time led to some patients developing Intestinal Failure Associated Liver Disease (IFALD), a leading cause of death and indication for liver/intestinal transplant. Over time, multi-disciplinary teams called Intestinal Rehabilitation Programs (IRPs) began providing meticulous and innovative management. Usage of alternative lipid emulsions and lipid minimization strategies have resulted in the decline of IFALD and an increase in long-term and transplant-free survival, even in the setting of ultrashort bowel (< 20 cm)...
December 20, 2016: Pediatric Research
https://www.readbyqxmd.com/read/27986169/hepatic-steatosis-etiology-patterns-and-quantification
#16
Ilkay S Idilman, Ilknur Ozdeniz, Musturay Karcaaltincaba
Hepatic steatosis can occur because of nonalcoholic fatty liver disease (NAFLD), alcoholism, chemotherapy, and metabolic, toxic, and infectious causes. Pediatric hepatic steatosis is also becoming more frequent and can have distinctive features. The most common pattern is diffuse form; however, it can present in heterogenous, focal, multinodular, perilesional, perivascular, subcapsular, and lobar forms. Focal steatosis and fat sparing can occur because of the presence of veins of Sappey, pancreaticoduodenal vein, and aberrant right and left gastric veins, which drain into the liver as third inflow...
December 2016: Seminars in Ultrasound, CT, and MR
https://www.readbyqxmd.com/read/27982761/liver-stiffness-in-pediatric-patients-with-fatty-liver-disease-diagnostic-accuracy-and-reproducibility-of-shear-wave-elastography
#17
Matteo Garcovich, Silvio Veraldi, Enrico Di Stasio, Maria Assunta Zocco, Lidia Monti, Paolo Tomà, Maurizio Pompili, Antonio Gasbarrini, Valerio Nobili
Purpose To evaluate the diagnostic accuracy of shear-wave elastography (SWE) in identifying different degrees of fibrosis in a cohort of consecutive children and adolescents with nonalcoholic steatohepatitis (NASH). Materials and Methods Consecutive pediatric patients scheduled to undergo liver biopsy were studied with an ultrasonography-based SWE system. Elastography was performed in 68 of 69 patients with biopsy-proved NASH (37 boys and 31 girls; mean age, 12.6 years ± 2.48; age range, 8-17 years). The correlations among laboratory findings, liver stiffness, and fibrosis score were analyzed, and the area under the receiver operating characteristic curve (AUC) was used to assess the presence of any fibrosis (score ≥F1) or significant fibrosis (score ≥F2)...
December 13, 2016: Radiology
https://www.readbyqxmd.com/read/27976737/altered-systemic-bile-acid-homeostasis-contributes-to-liver-disease-in-pediatric-patients-with-intestinal-failure
#18
Yong-Tao Xiao, Yi Cao, Ke-Jun Zhou, Li-Na Lu, Wei Cai
Intestinal failure (IF)-associated liver disease (IFALD), as a major complication, contributes to significant morbidity in pediatric IF patients. However, the pathogenesis of IFALD is still uncertain. We here investigate the roles of bile acid (BA) dysmetabolism in the unclear pathogenesis of IFALD. It found that the histological evidence of pediatric IF patients exhibited liver injury, which was characterized by liver bile duct proliferation, inflammatory infiltration, hepatocyte apoptosis and different stages of fibrosis...
December 15, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27975113/pediatric-liver-transplantation-an-update-for-the-pediatrician
#19
Kristin Capone, Karine Amirikian, Ruba K Azzam
Pediatric liver transplantation is a state-of-the-art treatment for children with end-stage liver disease. Over the past few decades, the advent of new surgical techniques using split liver grafts and living donors has drastically increased the organ availability for pediatric patients, while advances in immunosuppression have improved overall outcomes. The pediatrician is a key player in the multidisciplinary team that cares for these children starting with the timely referral of children who require liver transplantation to the active participation in optimizing the child's overall health before and after transplantation...
December 1, 2016: Pediatric Annals
https://www.readbyqxmd.com/read/27975112/acute-liver-failure
#20
Catherine D Newland
Pediatric acute liver failure (ALF) is a complex and rapidly progressive syndrome that results from a variety of age-dependent etiologies. It is defined by the acute onset of liver disease with no evidence of chronic liver disease. There must be biochemical or clinical evidence of severe liver dysfunction as defined by an international normalized ratio (INR) ≥2. If hepatic encephalopathy is present, INR should be ≥1.5. Unfortunately, due to the rarity of ALF in pediatric patients, there is a paucity of diagnostic and management algorithms and each patient must have an individualized approach...
December 1, 2016: Pediatric Annals
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