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Pediatric Liver

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https://www.readbyqxmd.com/read/27919847/using-on-site-liver-3-d-reconstruction-and-volumetric-calculations-in-split-liver-transplantation
#1
Trevor W Reichman, Brittany Fiorello, Ian Carmody, Humberto Bohorquez, Ari Cohen, John Seal, David Bruce, George E Loss
BACKGROUND: Split liver transplantation increases the number of grafts available for transplantation. Pre-recovery assessment of liver graft volume is essential for selecting suitable recipients. The purpose of this study was to determine the ability and feasibility of constructing a 3-D model to aid in surgical planning and to predict graft weight prior to an in situ division of the donor liver. METHODS: Over 11 months, 3-D volumetric reconstruction of 4 deceased donors was performed using Pathfinder Scout© liver volumetric software...
December 2016: Hepatobiliary & Pancreatic Diseases International: HBPD INT
https://www.readbyqxmd.com/read/27906803/naspghan-clinical-practice-guideline-for-the-diagnosis-and-treatment-of-nonalcoholic-fatty-liver-disease-in-children
#2
Miriam B Vos, Stephanie H Abrams, Sarah E Barlow, Sonia Caprio, Stephen R Daniels, Rohit Kohli, Marialena Mouzaki, Pushpa Sathya, Jeffrey B Schwimmer, Shikha S Sundaram, Stavra A Xanthakos
Nonalcoholic fatty liver disease (NAFLD) is a highly prevalent chronic liver disease that occurs in the setting of insulin resistance and increased adiposity. It has rapidly evolved into the most common liver disease seen in the pediatric population and is a management challenge for general pediatric practitioners, subspecialists and for health systems. In this guideline, the expert committee on NAFLD (ECON) reviewed and summarized the available literature, formulating recommendations to guide screening and clinical care of children with NAFLD...
November 30, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27906797/non-cirrhotic-portal-fibrosis-in-pediatric-population
#3
Vikrant Sood, Bikrant Bihari Lal, Rajeev Khanna, Dinesh Rawat, Chhagan Bihari Sharma, Seema Alam
BACKGROUND: Non-cirrhotic portal fibrosis (NCPF) has been classically described as a disease of young to middle age with limited literature regarding its occurrence, onset or clinical presentation in children. We hereby present a series of 19 patients diagnosed and managed as NCPF in pediatric age group. METHODS: A retrospective review of all the patients presenting to the pediatric hepatology department (age < 18 years) and diagnosed as NCPF was done and data was evaluated...
November 30, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27904251/pediatric-biliary-interventions-in-the-native-liver
#4
REVIEW
Lisa H Kang, Colin N Brown
Biliary disease in infants and children frequently presents diagnostic and therapeutic challenges. Pediatric interventional radiologists are often involved in the multidisciplinary teams who care for these patients. This article reviews several notable causes of biliary disease in children who have not undergone liver transplantation, describes the role of percutaneous interventional procedures in managing these conditions, and details applicable biliary interventional techniques.
December 2016: Seminars in Interventional Radiology
https://www.readbyqxmd.com/read/27900388/data-driven-modeling-for-precision-medicine-in-pediatric-acute-liver-failure
#5
Ruben Zamora, Yoram Vodovotz, Qi Mi, Derek Barclay, Jinling Yin, Simon Horslen, David Rudnick, Kathleen M Loomes, Robert H Squires
Absence of early outcome biomarkers for Pediatric Acute Liver Failure (PALF) hinders medical and liver transplant decisions. We sought to define dynamic interactions among circulating inflammatory mediators to gain insights into PALF outcome sub-groups. Serum samples from 101 participants in the PALF study, collected over the first 7 days following enrollment, were assayed for 27 inflammatory mediators. Outcomes (Spontaneous survivors [S, n=61], Non-survivors [NS, n=12], and liver transplant patients [LTx, n=28]) were assessed at 21 days post-enrollment...
November 23, 2016: Molecular Medicine
https://www.readbyqxmd.com/read/27895749/a-single-center-retrospective-study-of-pediatric-hepatoblastoma
#6
Yi Zhang, Weiling Zhang, Suoqin Tang, Liping Chen, You Yi, Pinwei Zhang, Aiping Liu, Tian Zhi, Dongsheng Huang
Hepatoblastoma is a malignant liver tumor generally diagnosed in infants and children <3 years old. The current retrospective study aimed to investigate the associations of tumor stage, pathological type, metastasis and chemotherapy with clinical outcomes. In the current study, a total of 102 patients with hepatoblastoma were enrolled between September 2006 and June 2014. Clinical records and follow-up information for each of patient were obtained to conduct a Kaplan-Meier survival analysis and log-rank test...
November 2016: Oncology Letters
https://www.readbyqxmd.com/read/27895113/age-dependent-absolute-abundance-of-hepatic-carboxylesterases-ces1-and-ces2-by-lc-ms-ms-proteomics-application-to-pbpk-modeling-of-oseltamivir-in-vivo-pharmacokinetics-in-infants
#7
Mikael Boberg, Marc Vrana, Aanchal Mehrotra, Robin E Pearce, Andrea Gaedigk, Deepak Kumar Bhatt, J Steven Leeder, Bhagwat Prasad
The age-dependent absolute protein abundance of carboxylesterase 1 and 2 (CES1 and CES2) in human liver was investigated and applied to predict infant pharmacokinetics (PK) of oseltamivir. The CES absolute protein abundance was determined by LC-MS/MS proteomics in human liver microsomal and cytosolic fractions prepared from tissue samples obtained from 136 pediatric and 35 adult donors. Two surrogate peptides per protein were selected for the quantification of CES1 and CES2 protein abundance. Purified CES1 and CES2 protein standards were used as calibrators, and the heavy labeled peptides were used as the internal standards...
November 28, 2016: Drug Metabolism and Disposition: the Biological Fate of Chemicals
https://www.readbyqxmd.com/read/27894421/nonoperative-management-of-blunt-solid-organ-injury-in-pediatric-surgery
#8
REVIEW
David M Notrica, Maria E Linnaus
In the last decade, higher rates of nonoperative management of liver, spleen, and kidney injuries have been achieved. An algorithmic approach may improve success on a national level. Factors for success include management strategy based on physiologic status of the child, early attempt at resuscitation using blood products, and appropriate use of adjuncts. Shorter hospitalizations are appropriate for children who have not bled significantly, and discharge instructions facilitate the safety of early discharge...
February 2017: Surgical Clinics of North America
https://www.readbyqxmd.com/read/27889929/twenty-eight-years-of-intestinal-transplantation-in-paris-experience-of-the-oldest-european-centre
#9
F Lacaille, S Irtan, L Dupic, C Talbotec, F Lesage, V Colomb, N Salvi, F Moulin, F Sauvat, Y Aigrain, Y Revillon, O Goulet, C Chardot
AIM: Our aim is to describe our achievements in pediatric intestinal transplantation (ITx) and define areas for improvement. METHODS: After a period (1987-90) of 9 isolated small bowel transplants (SBTx) where only one patient survived with her graft, 110 ITx were performed on 101 children from 1994 to 2014: 60 SBTx, 45 liver-small bowel, 4 multivisceral (3 with kidneys), 1 modified multivisceral. Indications were short bowel syndrome (36), motility disorders (30), congenital enteropathies (34) and others (1)...
November 27, 2016: Transplant International: Official Journal of the European Society for Organ Transplantation
https://www.readbyqxmd.com/read/27888532/biliary-strictures-complicating-liver-transplantation-in-pediatric-patients-experience-in-a-south-american-transplant-center
#10
A Holguin, S Rodriguez-Takeuchi, L Ospina, D Acosta, V Botero, L Thomas, J Villegas, G Echeverri, L Caicedo
OBJECTIVE: To describe the experience of percutaneous transhepatic cholangiography (PTC) with biliary dilatation and drainage after pediatric liver transplantation, and to determine the long-term outcome of this procedure. METHODS: Retrospective study from 2001 to 2013. Follow-up after treatment was also undertaken. A survival analysis was performed in patients in whom the procedure and eventual removal of the catheter were successful. RESULTS: In all, 196 children received liver transplants; 40 of them (20 boys and 20 girls; median age of 4 years) were treated using PTC due to biliary complications...
November 26, 2016: Clinical Transplantation
https://www.readbyqxmd.com/read/27882686/ensuring-adequate-immunizations-among-pediatric-liver-transplant-recipients-a-team-approach
#11
EDITORIAL
Annabelle de St Maurice, Natasha Halasa
No abstract text is available yet for this article.
December 2016: Pediatric Transplantation
https://www.readbyqxmd.com/read/27882406/bowel-perforation-after-liver-transplantation-for-biliary-atresia-a-retrospective-study-of-care-in-the-transition-from-children-to-adulthood
#12
Yusuke Yanagi, Toshiharu Matsuura, Makoto Hayashida, Yoshiaki Takahashi, Koichiro Yoshimaru, Genshirou Esumi, Tomoaki Taguchi
PURPOSE: We evaluated the outcomes of liver transplantation (LT) in pediatric and adult patients with biliary atresia (BA). We focused on bowel perforation after LT (BPLT) as the most common surgical complication and analyzed its risk factors. METHODS: This was a retrospective analysis of 70 BA patients who underwent LT. The patients were divided into three groups according to the timing of LT: within the first year of age (Group A), between 1 and 12 years of age (Group B), and after 12 years of age (Group C)...
November 23, 2016: Pediatric Surgery International
https://www.readbyqxmd.com/read/27882234/dysgerminoma-developing-from-an-ectopic-ovary-in-a-patient-with-wagr-syndrome-a-case-report
#13
Rie Miura, Yoshihito Yokoyama, Tatsuhiko Shigeto, Masayuki Futagami, Hideki Mizunuma, Akira Kurose, Kazushi Tsuruga, Shinya Sasaki, Kiminori Terui, Etsuro Ito
WAGR syndrome is caused by an 11p13 deletion and includes Wilms' tumor, aniridia, genitourinary anomalies and mental retardation. We encountered a case of a dysgerminoma originating in an ectopic ovary in a woman with WAGR syndrome. Our patient was a 24-year-old nulliparous woman who was diagnosed with WAGR syndrome. The patient had undergone left nephrectomy for a Wilms' tumor and postoperative chemotherapy at the age of 7 months. She also had a history of glaucoma surgery in both eyes, and was followed up at the Department of Pediatrics for diabetes mellitus, hypertension, liver dysfunction and hyperuricemia...
November 2016: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/27882046/the-role-of-genetic-and-immune-factors-for-the-pathogenesis-of-primary-sclerosing-cholangitis-in-childhood
#14
REVIEW
Priscila Menezes Ferri, Ana Cristina Simões E Silva, Soraya Luiza Campos Silva, Diego Junior Queiroga de Aquino, Eleonora Druve Tavares Fagundes, Débora Marques de Miranda, Alexandre Rodrigues Ferreira
Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease characterized by chronic inflammation of the biliary tree resulting in liver fibrosis. PSC is more common in male less than 40 years of age. The diagnosis of PSC is based on clinical, laboratory, image, and histological findings. A biochemical profile of mild to severe chronic cholestasis can be observed. Endoscopic retrograde cholangiography is the golden standard method for diagnosis, but magnetic resonance cholangiography is currently also considered a first-line method of investigation...
2016: Gastroenterology Research and Practice
https://www.readbyqxmd.com/read/27880991/vascular-complications-in-living-donor-liver-transplantation-at-a-high-volume-centre-evolving-protocols-and-trends-observed-over-10-years
#15
A R Shiraz, M A Nayeem, S Agarwal, N Goyal, S Gupta
Vascular complications continue to have devastating effect; although their nature, incidence and outcome might have actually changed with increasing experience and proficiency in high volume centers. Aim was to analyze the trends observed in vascular complications with changing protocols in adult and pediatric- living donor liver transplantation (A-LDLT and P-LDLT) over 10 years in two time frames in terms of nature, incidence and outcome. It is a retrospective analysis of 391 (Group I= Jan. 2006- Dec.2010) and 741 (Group II= Jan...
November 23, 2016: Liver Transplantation
https://www.readbyqxmd.com/read/27880037/suppressed-erythropoietin-expression-in-a-nitrofen-induced-congenital-diaphragmatic-hernia
#16
Hajime Takayasu, Koki Hagiwara, Kouji Masumoto
BACKGROUND: Erythropoietin (EPO), an essential stimulator of erythropoiesis produced by the fetal liver, is important both in vascular remodeling and modulation of the endothelial response in the pulmonary vasculature. In addition, EPO guides alveolar development, along with retinoic acid (RA). EPO is a direct target of RA, and the retinoid pathway is altered in the nitrofen-induced congenital diaphragmatic hernia (CDH) model. In the present study, we tested the hypothesis that the synthesis of EPO is suppressed in a rat model of CDH...
November 23, 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27878593/re-evaluation-of-liver-transaminase-cutoff-for-ct-after-pediatric-blunt-abdominal-trauma
#17
Jessica A Zagory, Avafia Dossa, Jamie Golden, Aaron R Jensen, Catherine J Goodhue, Jeffrey S Upperman, Christopher P Gayer
PURPOSE: Current guidelines for computed tomography (CT) after blunt trauma were developed to capture all intra-abdominal injuries (IAI). We hypothesize that current AST/ALT guidelines are too low leading to unnecessary CT scans for children after blunt abdominal trauma (BAT). METHODS: Patients who received CT of the abdomen after blunt trauma at our Level I Pediatric Trauma Center were stratified into a high risk (HR) (liver/spleen/kidney grade ≥III, hollow viscous, or pancreatic injuries) and low risk (LR) (liver/kidney/spleen injuries grade ≤II, or no IAI) groups...
November 23, 2016: Pediatric Surgery International
https://www.readbyqxmd.com/read/27878435/novel-phenotypes-and-loci-identified-through-clinical-genomics-approaches-to-pediatric-cataract
#18
Nisha Patel, Deepti Anand, Dorota Monies, Sateesh Maddirevula, Arif O Khan, Talal Algoufi, Mohammed Alowain, Eissa Faqeih, Muneera Alshammari, Ahmed Qudair, Hadeel Alsharif, Fatimah Aljubran, Hessa S Alsaif, Niema Ibrahim, Firdous M Abdulwahab, Mais Hashem, Haifa Alsedairy, Mohammed A Aldahmesh, Salil A Lachke, Fowzan S Alkuraya
Pediatric cataract is highly heterogeneous clinically and etiologically. While mostly isolated, cataract can be part of many multisystem disorders, further complicating the diagnostic process. In this study, we applied genomic tools in the form of a multi-gene panel as well as whole-exome sequencing on unselected cohort of pediatric cataract (166 patients from 74 families). Mutations in previously reported cataract genes were identified in 58% for a total of 43 mutations, including 15 that are novel. GEMIN4 was independently mutated in families with a syndrome of cataract, global developmental delay with or without renal involvement...
November 22, 2016: Human Genetics
https://www.readbyqxmd.com/read/27874250/immunosuppression-in-pediatric-liver-transplant-recipients-unique-aspects
#19
REVIEW
Miloh Tamir, Barton Andrea, Wheeler Justin, Pham Yen, Hewitt Winston, Keegan Tara, Sanchez Christine, Bulut Pinar, Goss John
Pediatric liver transplantation has experienced improved outcomes over the last 50 years. This can be attributed in part to establishing optimal use of immunosuppressive agents to achieve a balance between minimizing the risks of allograft rejection and infection. The management of immunosuppression in children is generally more complex and can be challenging when compared to the use of these agents in adult liver transplant patients. Physiologic differences in children alter the pharmacokinetics of immunosuppressive agents, which affects absorption, distribution, metabolism and drug excretion...
November 22, 2016: Liver Transplantation
https://www.readbyqxmd.com/read/27872411/osteoporosis-in-pediatric-liver-transplantation
#20
Walid Faraj, Ghina El Nounou, Abdallah Abou Al Naaj, Nancy Nakhoul, Ali Haydar, Mohammad Khalife
Liver transplantation provides an important, often life-saving treatment for end-stage liver disease. Osteoporosis post-liver transplantation has been described in adults; however, this has not been described in the pediatric population to date. We present a case of a 13-year-old female patient who underwent an orthotopic liver transplant for cryptogenic liver cirrhosis. Her immunosuppressants were tacrolimus and prednisone. Four months posttransplant, she started complaining of bilateral lower limb pain and limping while walking, progressing to a point where she was almost immobile...
December 2016: Progress in Transplantation
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