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Pediatric liver transplantation

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https://www.readbyqxmd.com/read/28937543/cholangiocarcinoma-among-children-and-adolescents-a-review-of-the-literature-and-seer-database-analysis
#1
Jennifer R Newsome, Rajkumar Venkatramani, Andras Heczey, Heather E Danysh, Douglas S Fishman, Tamir Miloh
OBJECTIVES: Cholangiocarcinoma (CCA) is a biliary malignancy found primarily in adults. The incidence of CCA in children is unknown. The aim of this study was to describe characteristics of CCA in children and adolescents. METHODS: Using the Surveillance, Epidemiology, and End Results Program (SEER 18) database, we identified incident cases of CCA diagnosed at <20 years of age during the period of 1973-2013. Additionally, we reviewed published case reports describing pediatric patients with CCA...
September 20, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28927979/enlarging-vascular-stents-after-pediatric-liver-transplantation
#2
Yi-Ting Yeh, Cheng-Yen Chen, Hsiou-Shan Tseng, Hsin-Kai Wang, Hsin-Lin Tsai, Niang-Cheng Lin, Chou-Fu Wei, Chinsu Liu
BACKGROUND: Endovascular intervention with stent placement to treat portal vein (PV) and hepatic vein (HV) stenosis after pediatric liver transplantation (LT) is still controversial in small children owing to the potential risk of functional stenosis after growth. The aim of this study is to evaluate the safety and efficacy of stent placement in this population. METHODS: Between 2004 and 2016, 6 children (all <3 years) received HV (n = 2) and PV (n = 4) stents placement among 46 pediatric LT patients at our institution...
September 5, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28921939/extreme-hepatic-resections-for-the-treatment-of-advanced-hepatoblastoma-are-planned-close-margins-an-acceptable-approach
#3
Adriana Fonseca, Abha Gupta, Furqan Shaikh, Raveena Ramphal, Vicky Ng, Ian McGilvray, J Ted Gerstle
BACKGROUND: Orthotopic liver transplantation (OLT) is considered the standard for children with hepatoblastoma (HB) in whom complete surgical resection is not possible. However, OLT is not always available or feasible. OBJECTIVE: To describe the outcome of children with HB who were initially deemed unresectable and underwent complex hepatectomy with planned close margins, and ultimately avoided OLT. METHODS: Demographic data, surgical and pathologic details, and survival information were collected from children treated for HB between January 2010 to December 2015...
September 17, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28921748/increased-risk-organ-transplantation-in-the-pediatric-population
#4
Sean M Wrenn, Peter W Callas, Trishul Kapoor, Alia F Aunchman, Adam N Paine, Jaime A Pineda, Carlos E Marroquin
IRD organs are classified by the Public Health Service to be at above-average risk for harboring human immunodeficiency virus, hepatitis C, and hepatitis B. Traditionally underutilized, there exists even greater reluctance for their use in pediatric patients. We performed a retrospective analysis via the United Network for Organ Sharing database of all pediatric renal and hepatic transplants performed from 2004 to 2008 in the United States. Primary outcomes were patient and graft survival. Proportional hazards regression was performed to control for potentially confounding factors...
September 17, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28918602/population-pharmacokinetics-and-optimal-sampling-strategy-for-model-based-precision-dosing-of-melphalan-in-patients-undergoing-hematopoietic-stem-cell-transplantation
#5
Kana Mizuno, Min Dong, Tsuyoshi Fukuda, Sharat Chandra, Parinda A Mehta, Scott McConnell, Elias J Anaissie, Alexander A Vinks
BACKGROUND: High-dose melphalan is an important component of conditioning regimens for patients undergoing hematopoietic stem cell transplantation. The current dosing strategy based on body surface area results in a high incidence of oral mucositis and gastrointestinal and liver toxicity. Pharmacokinetically guided dosing will individualize exposure and help minimize overexposure-related toxicity. OBJECTIVE: The purpose of this study was to develop a population pharmacokinetic model and optimal sampling strategy...
September 16, 2017: Clinical Pharmacokinetics
https://www.readbyqxmd.com/read/28914695/autoimmune-acute-liver-failure-and-seronegative-autoimmune-liver-disease-in-children-are-they-different-from-classical-disease
#6
Vikas Jain, Anshu Srivastava, Surender K Yachha, Niraj Kumari, Rachana Kathuria, Moinak S Sarma, Ujjal Poddar, Narendra Krishnani
OBJECTIVES: Presentation as autoimmune acute liver failure (AI-ALF) and seronegative autoimmune liver disease (SN-AILD) represents two uncommon variants of AILD. We compared the clinical profile and outcome of AI-ALF with autoimmune-non-acute liver failure (AI-non-ALF) and also SN-AILD with seropositive autoimmune liver disease (SP-AILD). MATERIALS AND METHODS: Children managed as AI-ALF and AI-non-ALF including SN-AILD and SP-AILD were enrolled and compared. AI-non-ALF was diagnosed by simplified diagnostic criteria and AI-ALF by Pediatric Acute Liver Failure Study Group criteria with positive autoantibody, exclusion of other etiologies, elevated immunoglobulin G and histology when available...
September 13, 2017: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28913888/viral-upper-respiratory-infection-at-pediatric-liver-transplant-is-associated-with-hepatic-artery-thrombosis
#7
Jordan A Kohler, Flor M Munoz, John A Goss, Tamir A Miloh
No abstract text is available yet for this article.
September 15, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28912999/tacrolimus-aggravated-tube-feeding-syndrome-with-acute-renal-failure-in-a-pediatric-liver-transplant-recipient
#8
R Kula, M Melter, J Kunkel, C Dörfler, S Alikadic, B Knoppke, R Zant
Acute renal failure can be caused by calcineurin inhibitors (CNIs), due to arteriolopathy and altered tubular function. Within this context, we present the case of a 14-month-old liver transplant recipient who suffered an acute polyuric renal failure during a short episode of hypercaloric feeding. In our case, CNI-induced distal RTA led to nephrocalcinosis and therefore to secondary nephrogenic diabetes insipidus. The diet with high renal solute load consequently resulted in an acute polyuric renal failure with severe hypernatremic dehydration...
2017: Case Reports in Transplantation
https://www.readbyqxmd.com/read/28902943/use-of-fibrinogen-and-thrombin-sponge-in-pediatric-split-liver-transplantation
#9
Fernando Pompeu Piza Vicentine, Adriano Miziara Gonzalez, Barbara Burza Beninni, Ramiro Anthero de Azevedo, Marcelo Moura Linhares, Alberto Goldenberg, Gaspar de Jesus Lopes, Jose Luiz Martins, Alcides Augusto Salzedas
Purpose: To analyze the use of this sponge in pediatric patients undergoing split-liver transplantation. Methods: Retrospective study, including 35 pediatric patients undergoing split-liver transplantation, divided into two groups according to the use of the sponge: 18 patients in Group A (no sponge) and 17 in Group B (with sponge). Results: The characteristics of recipients and donors were similar. We observed greater number of reoperation due to bleeding in the wound area in Group A (10 patients - 55...
August 2017: Acta Cirúrgica Brasileira
https://www.readbyqxmd.com/read/28889961/analysis-of-the-reversibility-of-biliary-cirrhosis-in-young-rats-submitted-to-biliary-obstruction
#10
Maria Julia de Aro Braz, Leonardo Ervolino Corbi, Ana Cristina Aoun Tannuri, Maria Cecília Mendonça Coelho, Josiane Oliveira Gonçalves, Suellen Serafini, Uenis Tannuri
BACKGROUND/PURPOSE: Biliary atresia and other liver biliary obstructions are relevant conditions in pediatric surgery due to their progression to biliary cirrhosis and indication for liver transplantation. It is known that the period during which biliary obstruction persists determines the development of cirrhosis and its reversibility after a biliary drainage procedure. However, no time or histological markers of biliary cirrhosis reversibility have been established. MATERIALS AND METHODS: One hundred and twenty-nine young Wistar rats underwent surgery for ligation of the common bile duct and were maintained until 8weeks...
August 24, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28889651/living-donor-liver-transplantation-for-biliary-atresia-an-analysis-of-2-085-cases-in-the-registry-of-the-japanese-liver-transplantation-society
#11
Mureo Kasahara, Koji Umeshita, Seisuke Sakamoto, Akinari Fukuda, Hiroyuki Furukawa, Shotaro Sakisaka, Eiji Kobayashi, Eiji Tanaka, Yukihiro Inomata, Seiji Kawasaki, Mitsuo Shimada, Norihiro Kokudo, Hiroto Egawa, Hideki Ohdan, Shinji Uemoto
Biliary atresia (BA) is the most common indication for liver transplantation (LT) in pediatric population. This study analyzed the comprehensive factors that might influence the outcomes of patients with BA who undergo living donor LT by evaluating the largest cohort with the longest follow-up in the world. Between November 1989 and December 2015, 2,085 BA patients underwent LDLT in Japan. There were 763 male and 1,322 female recipients with a mean age of 5.9 years and body weight of 18.6 kg. The 1-, 5-, 10-, 15-and 20-year graft survival rates for the BA patients undergoing LDLT were 90...
September 10, 2017: American Journal of Transplantation
https://www.readbyqxmd.com/read/28884036/severe-hepatopulmonary-syndrome-in-a-child-with-caroli-syndrome
#12
W De Jesus-Rojas, K McBeth, A Yadav, J M Stark, R A Mosquera, C Jon
Hepatopulmonary Syndrome (HPS) is a potential complication of chronic liver disease and is more commonly seen in the adult population. Caroli Syndrome is a rare inherited disorder characterized by intrahepatic ductal dilation and liver fibrosis that leads to portal hypertension. In children with liver disease, HPS should be considered in the differential diagnosis of prolonged, otherwise unexplained, hypoxemia. The presence of HPS can improve patient priority on the liver transplantation wait list, despite their Pediatric End-Stage Liver Disease (PELD) score...
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/28867276/pediatric-liver-transplantation-an-asymmetrical-war-for-access-to-livers
#13
EDITORIAL
Mohamed Rela, Mettu Srinivas Reddy
No abstract text is available yet for this article.
September 1, 2017: Gastroenterology
https://www.readbyqxmd.com/read/28859015/characteristics-and-risk-factors-of-late-onset-bloodstream-infection-beyond-six-months-after-liver-transplantation-in-children
#14
Munehiro Furuichi, Akinari Fukuda, Seisuke Sakamoto, Mureo Kasahara, Isao Miyairi
BACKGROUND: Bloodstream infection (BSI) is a major cause of morbidity and mortality after pediatric liver transplantation (LT). However, most studies have focused on BSI occurring within a few months after LT. In this study, we evaluated the characteristics of BSI occurring beyond six months after pediatric LT. METHODS: We conducted a retrospective cohort study at a pediatric LT center in Japan from November 2005 to March 2016. We evaluated the causative organisms and site of late-onset BSI in children ≤18 years of age...
August 28, 2017: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28854959/hemophagocytic-lymphohistiocytosis-presenting-in-a-pediatric-patient-with-near-total-colonic-and-small-bowel-aganglionosis-a-case-report
#15
Brittany Badal, Michael J Wilsey, Sara Karjoo
BACKGROUND: Total colonic and small bowel aganglionosis is a rare condition typically requiring intestinal transplant for long-term survival. There have not been any previously reported cases of near total intestinal aganglionosis complicated by concerns for hemophagocytic lymphohistiocytosis and need for both multivisceral organ transplant and hematopoietic stem cell transplant. CASE PRESENTATION: Our patient is a 35-month-old Egyptian boy who presented with bilious emesis and failure to pass meconium shortly after birth...
August 31, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28846072/mice-expressing-krasg12d-in-hematopoietic-multipotent-progenitor-cells-develop-neonatal-myeloid-leukemia
#16
Stefan P Tarnawsky, Rebecca J Chan, Mervin C Yoder
Juvenile myelomonocytic leukemia (JMML) is a pediatric myeloproliferative neoplasm that bears distinct characteristics associated with abnormal fetal development. JMML has been extensively modeled in mice expressing the oncogenic KrasG12D mutation. However, these models have struggled to recapitulate the defining features of JMML due to in utero lethality, nonhematopoietic expression, and the pervasive emergence of T cell acute lymphoblastic leukemia. Here, we have developed a model of JMML using mice that express KrasG12D in multipotent progenitor cells (Flt3Cre+ KrasG12D mice)...
August 28, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28842757/successful-long-term-outcome-of-pediatric-liver-kidney-transplantation-a-single-center-study
#17
Jesús Quintero Bernabeu, Javier Juamperez, Marina Muñoz, Olalla Rodriguez, Ramon Vilalta, José A Molino, Marino Asensio, Itxarone Bilbao, Gema Ariceta, Carlos Rodrigo, Ramón Charco
INTRODUCTION: Liver-kidney transplantation is a rare procedure in children, with just ten to 30 cases performed annually worldwide. The main indications are autosomal recessive polycystic liver-kidney disease and primary hyperoxaluria. This study aimed to report outcomes of liver-kidney transplantation in a cohort of pediatric patients. METHODS: We retrospectively analyzed all pediatric liver-kidney transplantations performed in our center between September 2000 and August 2015...
August 25, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28839442/gut-barrier-failure-biomarkers-are-associated-with-poor-disease-outcome-in-patients-with-primary-sclerosing-cholangitis
#18
Tamas Tornai, Eszter Palyu, Zsuzsanna Vitalis, Istvan Tornai, David Tornai, Peter Antal-Szalmas, Gary L Norman, Zakera Shums, Gabor Veres, Antal Dezsofi, Gabriella Par, Alajos Par, Peter Orosz, Ferenc Szalay, Peter Laszlo Lakatos, Maria Papp
AIM: To assess the prevalence of a panel of serologic markers that reflect gut barrier dysfunction in a mixed cohort of pediatric and adult primary sclerosing cholangitis (PSC) patients. METHODS: Sera of 67 PSC patients [median age (range): 32 (5-79) years, concomitant IBD: 67% and cirrhosis: 20%] were assayed for the presence of antibodies against to F-actin (AAA IgA/IgG) and gliadin (AGA IgA/IgG)] and for serum level of intestinal fatty acid-binding protein (I-FABP) by ELISA...
August 7, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28838453/cholestasis-after-pediatric-liver-transplantation-recurrence-of-a-progressive-familial-intrahepatic-cholestasis-phenotype-as-a-rare-differential-diagnosis-a-case-report
#19
B Prusinskas, S Kathemann, D Pilic, B Hegen, P Küster, V Keitel, D Häussinger, R Büscher, H A Baba, P F Hoyer, E Lainka
INTRODUCTION: Nonobstructive cholestasis after pediatric liver transplantation is a common diagnostic and therapeutic dilemma. We describe a girl with neonatal cholestasis because of progressive familial intrahepatic cholestasis 2 (PFIC-2) and presence of a homozygous splice site mutation in the ABCB11 gene. Liver transplantation was performed because of end-stage liver disease at the age of 6. Cholestasis with normal gamma-glutamyl transferase (GGT) developed 8 years after liver transplantation...
September 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28838451/hepatic-artery-reconstruction-using-3-in-1-segmental-resection-in-pediatric-living-donor-liver-transplantation-a-case-report-and-literature-review
#20
Y Luo, D Zhao, M Zhang, T Zhou, B-J Qiu, J-J Zhang, Q Xia
We report a transplant of the left lateral liver segments with 3 arteries for a pediatric recipient from a living donor. A 6-month-old female infant was diagnosed with liver cirrhosis secondary to biliary atresia and scheduled for living donor liver transplantation (LDLT; mother as donor). Left lateral hepatectomy was performed at the donor site. The dissection of the left hepatic artery (HA), which was divided immediately after its origin, showed 3 branches for segments II, III, and IV. The arteries for segment II, segment III, and segment IV were anastomosed to the recipient HA...
September 2017: Transplantation Proceedings
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