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Pediatric liver disease

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https://www.readbyqxmd.com/read/28433098/wilson-disease-in-children
#1
Eve A Roberts, Piotr Socha
Wilson disease (WD) is an inherited disorder mainly of hepatocellular copper disposition, due to dysfunction of the Wilson ATPase, a P1B-ATPase encoded by the gene ATP7B. In children, as in older age brackets, clinical disease is highly diverse. Although hepatic disease is the common presentation in children/adolescents, neurologic, psychiatric, and hematologic clinical presentations do occur. Very young children may have clinically evident liver disease due to WD. Early diagnosis, preferably when the child/adolescent is asymptomatic, is most likely to result in near-normal longevity with generally good health so long as the patient tolerates effective medication, is adherent to the lifelong treatment regimen, and has consistent access to the medication...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28426902/hepatic-steatosis-after-pediatric-liver-transplant
#2
Emily R Perito, Tabitha Vase, Rageshree Ramachandran, Andrew Phelps, Kuang-Yu Jen, Robert H Lustig, Vickie A Feldstein, Philip Rosenthal
RATIONALE: Hepatic steatosis develops after liver transplant in 30% of adults, and non-alcoholic fatty liver disease (NAFLD) is the most common chronic liver disease in non-transplanted children. However, post-transplant steatosis has been minimally studied in pediatric liver transplant recipients. We explored the prevalence, persistence, and association with chronic liver damage of hepatic steatosis in these children. RESULTS: In this single-center study of pediatric patients transplanted 1988-2015 (n=318), 31% of those with any post-transplant biopsy (n=271) had ≥1 biopsy with steatosis...
April 20, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28426447/measles-cases-in-children-requiring-hospital-access-in-an-academic-pediatric-hospital-in-italy-2008-2013
#3
Marta Ciofi Degli Atti, Antonietta Filia, Antonino Bella, Annamaria Sisto, Maria Antonietta Barbieri, Antonino Reale, Massimiliano Raponi
BACKGROUND: The Lazio Region is one of the Italian regions where sustained measles transmission continues to occur. We investigated measles cases reported by the Emergency Department (ED) of the largest pediatric hospital in Italy, located in Lazio. METHODS: We reviewed clinical records of all measles cases from 0 to 18 years of age evaluated in the ED in 2008-2013. We compared demographic and clinical characteristics of patients admitted to the inpatient setting with those of patients discharged home, to assess possible determinants of hospital admission...
April 18, 2017: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28422310/adult-onset-cystic-fibrosis-liver-disease-diagnosis-and-characterization-of-an-underappreciated-entity
#4
Christopher Koh, Sasan Sakiani, Pallavi Surana, Xiongce Zhao, Jason Eccleston, David E Kleiner, David Herion, T Jake Liang, Jay H Hoofnagle, Milica Chernick, Theo Heller
BACKGROUND & AIMS: Cystic fibrosis liver disease (CFLD), a leading cause of death in cystic fibrosis (CF), is mostly described in pediatric populations. Adult-onset CFLD lacks sufficient characterization and diagnostic tools. METHODS: A cohort of CF patients without CFLD during childhood were followed for up to 38 years with serologic testing, imaging, and noninvasive fibrosis markers. Historical CFLD diagnostic criteria were compared with a newly proposed CFLD criteria...
April 19, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28420301/seizures-in-pediatric-patients-with-liver-transplant-and-efficacy-of-levetiracetam
#5
Betül Kılıç, Serdal Güngör, Müjgan Arslan, Mukadder Ayşe Selimoğlu, Sezai Yılmaz
The aim of this study was to evaluate the risk factors, clinical implications, and prognosis of new-onset seizures that occurred after pediatric liver transplantation, and to assess the efficacy of levetiracetam treatment. The clinical and laboratory data of liver transplanted 28 children who had seizures after liver transplantation and specifically of 18 children who received levetiracetam were analyzed retrospectively. Sixteen patients (88.9%) remained seizure-free and in 2 (11.1%), more than 50% reduction in seizures were detected with levetiracetam treatment...
January 1, 2017: Journal of Child Neurology
https://www.readbyqxmd.com/read/28420050/carotid-intima-media-thickness-and-arterial-stiffness-in-pediatric-systemic-lupus-erythematosus
#6
N Su-Angka, A Khositseth, S Vilaiyuk, K Tangnararatchakit, W Prangwatanagul
Objectives The carotid intima-media thickness (CIMT) and carotid arterial stiffness index (CASI) act as the surrogate markers of atherosclerosis. We aim to assess CIMT and CASI in pediatric systemic lupus erythematosus (SLE). Methods Patients ≤ 20 years old fulfilling diagnostic criteria for SLE were enrolled. Patients with active smoking, coronary heart disease, cerebrovascular disease, arterial thrombosis, family history of hypercholesterolemia, chronic liver disease, or other chronic severe diseases were excluded...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28417472/evaluation-of-liver-kidney-and-spleen-elasticity-in-healthy-newborns-and-infants-using-shear-wave-elastography
#7
Figen Bakirtas Palabiyik, Ercan Inci, Rustu Turkay, Derya Bas
OBJECTIVES: Elasticity measurements of tissues can be valuable in the diagnosis and management of various diseases. The aim of this study was to determine the elasticity values for normal liver, kidney, and spleen of healthy newborns and infants using shear wave elastography (SWE) imaging. METHODS: A total of 50 healthy term newborns and infants (19 girls and 31 boys; mean age 20.1 days, range 1 to 70 days) were examined by an experienced pediatric radiologist using SWE...
April 18, 2017: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/28411361/liver-transplant-in-children-with-hepatoblastoma
#8
Mehmet Hanifi Okur, Yücel Yankol, Bayındır Cimşit, Nesimi Mecit, Gökhan Ertuğrul, Turan Kanmaz, Ceyhun Bozkurt, Koray Acarlı, Münci Kalayoğlu
OBJECTIVES: In this paper, the results of liver transplant due to hepatoblastoma in 10 pediatric patients at Istanbul Şişli Memorial Hospital Transplantation Center are presented. MATERIALS AND METHODS: We retrospectively evaluated medical records of pediatric patients diagnosed with hepatoblastoma and who underwent liver transplant at our clinic between January 2009 and March 2014. We examined age, weight, chemotherapy regimen, graft type for liver transplant, duration of hospital stay, complications, follow-up duration, and survival information...
April 14, 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28411097/-acute-liver-failure-related-to-inherited-metabolic-diseases-in-young-children
#9
Filipa Dias Costa, Rita Moinho, Sandra Ferreira, Paula Garcia, Luísa Diogo, Isabel Gonçalves, Carla Pinto
INTRODUCTION: Pediatric acute liver failure (ALF) due to inherited metabolic diseases (IMD) is a rare life-threatening condition with a poor prognosis. Early intervention may be lifesaving. OBJECTIVE: To describe clinical presentation, investigation and outcomes of ALF related to IMD in young children. MATERIAL AND METHODS: Retrospective review of the medical records of children aged up to 24 months, admitted to a tertiary pediatric and neonatal Intensive Care Unit during a 27-year period, fulfilling the ALF criteria, with documented metabolic etiology...
April 11, 2017: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
https://www.readbyqxmd.com/read/28396861/non-invasive-investigations-for-the-diagnosis-of-fontan-associated-liver-disease-in-pediatric-and-adult-fontan-patients
#10
Amyna Fidai, Frederic Dallaire, Nanette Alvarez, Yvonne Balon, Robin Clegg, Michael Connelly, Frank Dicke, Deborah Fruitman, Joyce Harder, Kimberley Myers, David J Patton, Tim Prieur, Erika Vorhies, Robert P Myers, Steven R Martin, Steven C Greenway
Fontan-associated liver disease (FALD) is a serious complication related to the chronically elevated venous pressure and low cardiac output of this abnormal circulation. However, diagnostic markers for this condition are limited. We hypothesized that specific tests for fibrosis developed for other chronic liver diseases would identify a higher prevalence of FALD than ultrasound and standard laboratory tests and that identified abnormalities would correlate with time post-Fontan. In this cross-sectional study, we assessed 19 children (average age 8...
2017: Frontiers in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28396164/dose-escalation-of-total-marrow-irradiation-in-high-risk-patients-undergoing-allogeneic-hematopoietic-stem-cell-transplantation
#11
Susanta Hui, Claudio Brunstein, Yutaka Takahashi, Todd DeFor, Shernan G Holtan, Veronika Bachanova, Christopher Wilke, Darren Zuro, Celalettin Ustun, Daniel Weisdorf, Kathryn Dusenbery, Michael R Verneris
Patients with refractory leukemia or minimal residual disease (MRD) at transplant have increased risk of relapse. Augmentation of irradiation, especially to sites of disease (i.e., bone marrow) is one potential strategy to overcome this risk. We studied the feasibility of radiation dose escalation in high risk patients using total marrow irradiation (TMI) in a phase I dose-escalation trial. Four pediatric and 8 adult patients received conditioning with cyclophosphamide and fludarabine in conjunction with image-guided radiation to the bone marrow at 15 Gy and 18 Gy (in 3 Gy/fractions), while maintaining the total body irradiation (TBI) dose to the vital organs (lungs, hearts, eyes, liver, kidneys) at <13...
April 7, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28393434/multivisceral-transplantation-for-abdominal-tumors-in-children-a-single-center-experience-and-review-of-the-literature
#12
Eliza Lee, Nicole Hodgkinson, Rima Fawaz, Khashayar Vakili, Heung Bae Kim
Standard management of intra-abdominal pediatric solid tumors requires complete resection. However, tumors with multiple organ and vascular involvement present a unique surgical challenge. We conducted a retrospective chart review of four patients, aged 2-14 years, undergoing MVT for intra-abdominal tumors with significant involvement of the visceral arteries and/or portomesenteric venous system at our institution. Indications for MVT included hepatocellular carcinoma, inflammatory myofibroblastic tumor, and two cases of hepatoblastoma...
April 9, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28392706/pancreatic-solitary-fibrous-tumor-in-a-toddler-managed-by-pancreaticoduodenectomy-a-case-report-and-review-of-the-literature
#13
Qingfeng Sheng, Weijue Xu, Jiangbin Liu, Baiyong Shen, Xiaxing Deng, Yibo Wu, Wei Wu, Shenghua Yu, Xueli Wang, Zhibao Lv
Solitary fibrous tumor (SFT) of the pancreas is rare, with 15 adult cases reported in the English literature. We described a 14-month-old boy who presented with obstructive jaundice. Dominantly elevated serum CA19-9 was detected. Imaging studies revealed a well-circumscribed, solid mass in the pancreatic head. A pancreaticoduodenectomy (child procedure) was performed using Shen's anastomosis technique. After resection of the tumor, liver function and serum tumor markers normalized and clinical signs receded...
2017: OncoTargets and Therapy
https://www.readbyqxmd.com/read/28391883/childhood-adult-onset-lysosomal-acid-lipase-deficiency-a-serious-metabolic-and-vascular-phenotype-beyond-liver-disease-four-new-pediatric-cases
#14
Pierre Poinsot, Sophie Collardeau Frachon, Lioara Restier, André Sérusclat, Mathilde Di Filippo, Sybil Charrière, Philippe Moulin, Alain Lachaux, Noel Peretti
BACKGROUND: The childhood/adult-onset lysosomal acid lipase deficiency (LALD; late-onset LALD) is a rare genetic disease. Children present severe fatty liver disease with early cirrhosis. Before enzyme replacement therapy, statins were the standard treatment to improve the severe dyslipidemia. However, late-onset LALD should be considered as a systemic metabolic disease: chronic hyper-low-density lipoprotein and hypo-high-density lipoprotein cholesterolemia induces early atherosclerosis in addition to the liver morbidity...
January 2017: Journal of Clinical Lipidology
https://www.readbyqxmd.com/read/28390159/the-natural-history-of-primary-sclerosing-cholangitis-in-781-children-a-multicenter-international-collaboration
#15
Mark R Deneau, Wael El-Matary, Pamela L Valentino, Reham Abdou, Khaled Alqoaer, Mansi Amin, Achiya Z Amir, Marcus Auth, Fateh Bazerbachi, Annemarie Broderick, Albert Chan, Jillian Cotter, Sylvia Doan, Mounif El-Youssef, Federica Ferrari, Katryn N Furuya, Madeleine Gottrand, Frederic Gottrand, Nitika Gupta, Matjaz Homan, M K Jensen, Binita M Kamath, Kyung Mo Kim, Kaija-Leena Kolho, Anastasia Konidari, Bart Koot, Raffaele Iorio, Oren Ledder, Cara Mack, Mercedes Martinez, Tamir Miloh, Parvathi Mohan, Niamh O'Cathain, Alexandra Papadopoulou, Amanda Ricciuto, Lawrence Saubermann, Pushpa Sathya, Eyal Shteyer, Vratislav Smolka, Atushi Tanaka, Raghu Varier, Veena Venkat, Bernadette Vitola, Miriam B Vos, Marek Woynarowski, Jason Yap
BACKGROUND: There are limited data on the natural history of primary sclerosing cholangitis (PSC) in children. We aimed to describe the disease characteristics and long-term outcomes of pediatric PSC. METHODS: We retrospectively collected all pediatric PSC cases from 36 participating institutions and conducted a survival analysis from the date of PSC diagnosis to dates of diagnosis of portal hypertensive or biliary complications, cholangiocarcinoma, liver transplantation, or death...
April 8, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28377772/treatment-of-pediatric-acute-graft-versus-host-disease-lessons-from-primary-immunodeficiency
#16
REVIEW
Aisling M Flinn, Andrew R Gennery
Allogeneic hematopoietic stem cell transplant (HSCT) is used to treat increasing numbers of malignant and non-malignant disorders. Despite significant advances in improved human leukocyte antigens-typing techniques, less toxic conditioning regimens and better supportive care, resulting in improved clinical outcomes, acute graft-versus-host disease (aGvHD) continues to be a major obstacle and, although it principally involves the skin, gastrointestinal tract, and liver, the thymus is also a primary target. An important aim following HSCT is to achieve complete and durable immunoreconstitution with a diverse T-cell receptor (TCR) repertoire to recognize a broad range of pathogens providing adequate long-term adaptive T-lymphocyte immunity, essential to reduce the risk of infection, disease relapse, and secondary malignancies...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28362660/role-of-nutrition-and-feeding-for-the-chronically-ill-pediatric-liver-patient-awaiting-liver-transplant
#17
Carly D G Leon, Stacee M Lerret
This article reviews the essential role of nutrition in optimizing care for pediatric patients with liver disease awaiting liver transplant. A review of growth and overall principles of feeding for pediatric patients, from infancy through childhood and into adolescence, is provided including the role of macro- and micronutrients, nutrient distribution, and nutrition supplementation. The importance of a thorough nutrition assessment is reviewed, including focus areas the nurse can address with patients and families such as diet histories, growth, and dietary modifications...
March 2017: Gastroenterology Nursing: the Official Journal of the Society of Gastroenterology Nurses and Associates
https://www.readbyqxmd.com/read/28355206/hepatic-mdr3-expression-impacts-lipid-homeostasis-and-susceptibility-to-inflammatory-bile-duct-obstruction-in-neonates
#18
Alexandra N Carey, Wujuan Zhang, Kenneth D R Setchell, Julia E Simmons, Tiffany Shi, Celine S Lages, Mary Mullen, Kaitlin Carroll, Rebekah Karns, Kazuhiko Bessho, Rachel Sheridan, Xueheng Zhao, Susanne N Weber, Alexander G Miethke
BACKGROUND: Heterozygous mutations in the gene ABCB4, encoding the phospholipid floppase MDR3 (Mdr2 in mice), are associated with various chronic liver diseases. Here, we hypothesize that reduced ABCB4 expression predisposes to extrahepatic biliary atresia (EHBA). METHODS: Livers from neonatal wildtype (wt) and heterozygous Mdr2-deficient mice were subjected to mass-spectrometry based lipidomics and RNAseq studies. Following postnatal infection with rhesus rotavirus (RRV), liver immune responses and EHBA phenotype were assessed...
March 29, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28355202/microrna-155-modulates-bile-duct-inflammation-via-targeting-suppressor-of-cytokine-signaling-1-in-biliary-atresia
#19
Rui Zhao, Rui Dong, Yifan Yang, Yuqing Wang, Jin Ma, Jiang Wang, Hao Li, Shan Zheng
BACKGROUND: Biliary atresia (BA) is an etiologically perplexing disease, manifested by neonatal cholestasis, repeated cholangitis, and progressive biliary fibrosis. MiR-155 has been implicated to modulate the immune response, which contributes to biliary injury. However, its potential role in the pathogenesis of BA has not been addressed so far. METHODS: The microRNA changes from BA patients and controls were identified via microarray. The immunomodulatory function of miR-155 was investigated via cell transfection and reporter assay system...
March 29, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28343660/a-pilot-study-of-ultrasound-elastography-as-a-non-invasive-method-to-monitor-liver-disease-in-children-with-short-bowel-syndrome
#20
Daniel Lodwick, Molly Dienhart, Jennifer N Cooper, Bonita Fung, Joseph Lopez, Sally Smith, Patrick Warren, Jane Balint, Peter C Minneci
BACKGROUND: Our objective was to evaluate the accuracy of acoustic radiation force impulse (ARFI) elastography in determining extent of liver fibrosis in pediatric patients with short bowel syndrome (SBS). METHODS: This prospective cohort study included pediatric patients with SBS who underwent ultrasound with ARFI measurements of shear wave speed (SWS) and liver biopsy within 30days of each other between 12/2014-9/2015. The mean and median SWS were compared to the stage of fibrosis...
March 16, 2017: Journal of Pediatric Surgery
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