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Pediatric liver transplantation

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https://www.readbyqxmd.com/read/29161210/index-use-of-smoflipid-in-a-tertiary-intestinal-rehabilitation-and-transplantation-program
#1
Kwai Lam, Thomas Schiano, Maria Isabel Fiel, Kishore Iyer
Long-term parenteral nutrition (PN) may be complicated by PN-associated liver disease (PNALD), and some studies suggest an association between the use of soy-based fat emulsions and PNALD development. Patients' liver function typically improves and PNALD resolves after reducing or stopping a soy-based fat emulsion, and thus lipid minimization has been the primary strategy for managing PNALD in many intestinal rehabilitation programs. However, fat emulsions often cannot be stopped entirely, leading some patients to develop PNALD even after lipid reduction strategies have been implemented...
November 1, 2017: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/29161056/de-novo-food-allergy-in-pediatric-liver-transplantation-recipients
#2
Ratchaneewan Sinitkul, Wiparat Manuyakorn, Wasu Kamchaisatian, Soamarat Vilaiyuk, Suwat Benjaponpitak, Chatmanee Lertudompholwanit, Suporn Treepongkaruna
BACKGROUND: Food allergy (FA) prevalence is increasing in pediatric liver transplantation (LT). However, the clinical course is still limited. OBJECTIVE: This retrospective cohort study aimed to identify the prevalence, risk factors, and the natural history of de novo FA in children post LT. METHODS: Medical records of pediatric LT recipients from Jan 2001 - Dec 2014 were reviewed. De novo FA was diagnosed by symptoms after exposure to culprit food occurring after LT, and improvement after diet elimination...
November 22, 2017: Asian Pacific Journal of Allergy and Immunology
https://www.readbyqxmd.com/read/29160012/near-infrared-spectroscopy-might-be-a-useful-tool-for-predicting-the-risk-of-vascular-complications-after-pediatric-liver-transplants-two-case-reports
#3
Juntaro Shiba, Masaaki Satoh, Koki Taira, Yasunori Niwa, Soichiro Inoue, Koichi Mizuta, Mamoru Takeuchi
In patients that have undergone liver transplants, a postoperative reduction in the blood flow of the liver graft represents a critical complication. We recently encountered an interesting phenomenon; that is, we found that the rSO2 level of the liver graft, as measured by NIRS, drops in patients that subsequently require an emergency liver biopsy. An 8-month-old female and an 8-month-old male underwent living donor liver transplants for biliary atresia. In both cases, a reduction in rSO2 was detected before an emergency liver biopsy was required...
November 21, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/29154411/effect-of-graft-size-matching-on-pediatric-living-donor-liver-transplantation-at-a-single-center
#4
Jun-Jie Li, Cai-Hua Zu, Shi-Peng Li, Wei Gao, Zhong-Yang Shen, Jin-Zhen Cai
We retrospectively analyzed 252 patients with end-stage liver disease who had undergone LDLT from January 2009 to September 2015. Of these, 25 had a GRWR of <2.0% (Group A), 204 had a GRWR of ≥2.0% or <4.0% (Group B), and 23 had a GRWR ≥4.0% (Group C). The three GRWR groups demonstrated similar characteristics, except for recipient age and recipient BMI. The overall one, two, and three-year graft survival rates were 95.1%, 93.5%, and 93.5%, respectively. However, among the three groups, graft survival rates at one year, two years and three years were significantly different (P=0...
November 20, 2017: Clinical Transplantation
https://www.readbyqxmd.com/read/29149103/human-hepatocyte-transplantation-for-liver-disease-current-status-and-future-perspectives
#5
REVIEW
Valeria Iansante, Ragai R Mitry, Céline Filippi, Emer Fitzpatrick, Anil Dhawan
Liver transplantation is the accepted treatment for patients with acute liver failure and liver-based metabolic disorders. However, donor organ shortage and lifelong need for immunosuppression are the main limitations to liver transplantation. In addition, loss of the native liver as target organ for future gene therapy for metabolic disorders limits the futuristic treatment options, resulting in the need for alternative therapeutic strategies. A potential alternative to liver transplantation is allogeneic hepatocyte transplantation...
November 14, 2017: Pediatric Research
https://www.readbyqxmd.com/read/29144580/higher-retransplantation-rate-following-extended-right-split-liver-transplantation-an-analysis-from-the-eurotransplant-liver-follow-up-registry
#6
J Andrassy, S Wolf, M Lauseker, M Angele, M D van Rosmalen, U Samuel, X Rogiers, J Werner, M Guba
Split liver transplantation (SLT) has been perceived as an important strategy to increase the supply of liver grafts by creating 2 transplants from 1 allograft. The Eurotransplant allocation system (ELAS) envisages that the ERLs after splitting (usually in the pediatric center) are almost exclusively shipped to a second center. Whether the ELAS policy impacts the graft and patient survival of ERLT in comparison to WLT recipients remains unclear. Data on all LTs performed between 2007 and 2013 were retrieved from the ET Liver follow-up Registry (n=5351)...
November 16, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/29141316/-a-clinical-analysis-of-micafungin-treatment-of-pulmonary-invasive-fungal-infection-in-pediatric-patients-with-acute-leukemia-or-post-hematopoietic-stem-cells-transplantation
#7
K Huang, K Y Qiu, L L Deng, J P Fang, Y Li, H X Guo, D H Zhou
Objective: To investigate the efficacy and safety of micafungin (MCF) for pulmonary invasive fungal disease (PIFD) in pediatric patients with acute leukemia or post hematopoietic stem cells transplantation. Method: Twenty-five neutropenic PIFD children with acute leukemia or post hematopoietic stem cells transplantation in Sun Yat-sen Memorial Hospital of Sun Yat-sen University were selected from January 2012 to June 2015, including 12 males and 13 females, age range 2-15 (average 6.2±2.0) years. There were 12 cases of acute leukemia (AL) after chemotherapy, 4 cases of acute leukemia (AL) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) and 9 cases of β-thalassemia major after allo-HSCT...
November 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29131462/outcomes-of-liver-transplantation-in-pediatric-recipients-with-cardiovascular-disease
#8
Márcio Miranda Brito, Joao Seda Neto, Eduardo A Fonseca, Renata Pugliese, Vera B Danesi, Helry L Candido, Adriana Porta, Cristian V B Borges, Gilda Porta, Paulo Chapchap, Irene Kazue Miura
LT exerts considerable stress on the heart perioperatively. Limited data exist on impact of cardiovascular diseases on LT children. This study evaluated the outcomes of children with CVD who underwent LT and compared with pretransplant findings. From 518 LT recipients, 82 (15.8%) had CVD. Sixty patients were classified as low-risk adjustment for congenital heart surgery 1 (RACHS 1 and 2). Five patients were classified as RACHS ≥3. The most common echocardiographic finding in the CVD patients (25/82) was ASD...
November 12, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/29118687/renoportal-anastomosis-in-left-lateral-lobe-living-donor-liver-transplantation-a-pediatric-case
#9
Hiroyuki Ogasawara, Chikashi Nakanishi, Shigehito Miyagi, Kazuaki Tokodai, Yasuyuki Hara, Wataru Nakanishi, Koji Miyazawa, Kenji Shimizu, Hiroyuki Kumata, Hitoshi Goto, Masafumi Goto, Michiaki Unno, Takashi Kamei
In adult liver transplantation, renoportal anastomosis (RPA) has been introduced as a useful technique for patients with grade 4 portal vein thrombosis and a splenorenal shunt. Here, we report a pediatric case in which RPA allowed a left lateral lobe living donor liver transplantation (LDLT) despite portal vein thrombosis and a large splenorenal shunt. At 36 days old, the patient underwent a Kasai operation for biliary atresia. At 17 months old, she underwent LDLT because of repetitive cholangitis. Pretransplant examinations revealed a large splenorenal shunt and portal vein thrombosis...
September 2017: Case Reports in Gastroenterology
https://www.readbyqxmd.com/read/29116687/erythropoietic-protoporphyria-in-an-adult-with-sequential-liver-and-hematopoietic-stem-cell-transplantation-a-case-report
#10
Annika L Windon, Rashmi Tondon, Nathan Singh, Samir Abu-Gazala, David L Porter, J Eric Russell, Colleen Cook, Elaine Lander, Georgeine Smith, Kim M Olthoff, Abraham Shaked, Maarouf Hoteit, Emma E Furth, Marina Serper
Erythropoieitic protoporphyria (EPP) is a rare inherited disorder of the heme biosynthesis pathway resulting in the accumulation of protoporphyrins in the blood, erythrocytes, and other tissues. Because of a gene mutation in the FECH gene, ferrochelatase, the enzyme involved in the final step of heme synthesis, is deficient in these patients. While the major symptom of this disorder is photosensitivity, rarely, it can cause progressive liver disease requiring liver transplantation (LT). However, LT is not curative and only bone marrow transplantation (BMT) can correct the underlying enzymatic defect...
November 8, 2017: American Journal of Transplantation
https://www.readbyqxmd.com/read/29110830/long-term-outcomes-of-pediatric-intestinal-failure
#11
Brenna S Fullerton, Charles R Hong, Tom Jaksic
Management of pediatric intestinal failure has evolved in recent decades, with improved survival since the advent of specialized multidisciplinary intestinal failure centers. Though sepsis and intestinal failure associated liver disease still contribute to mortality, we now have growing data on the long-term outcomes for this population. While intestinal adaptation and parenteral nutrition weaning is most rapid during the first year on parenteral support, achievement of enteral autonomy is possible even after many years as energy and protein requirements decline dramatically with age...
October 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/29108345/mri-based-evaluation-of-multiorgan-iron-overload-is-a-predictor-of-adverse-outcomes-in-pediatric-patients-undergoing-allogeneic-hematopoietic-stem-cell-transplantation
#12
Natalia Maximova, Massimo Gregori, Giulia Boz, Roberto Simeone, Davide Zanon, Giulia Schillani, Floriana Zennaro
The medical records of 44 pediatric patients who underwent allogeneic transplantation from 2011 to 2015 were retrospectively reviewed. Magnetic resonance imaging was used to measure iron concentrations in the liver, spleen, pancreas and bone. These patients were divided into two groups, 18 with non-elevated (< 100 μmol/g; Group 1) liver iron concentration before transplantation and 26 with elevated (> 100 μmol/g; Group 2) concentration . We compared transplant-related outcomes in the two groups. Iron overload was a negative prognostic risk factor for sinusoidal obstruction syndrome (OR = 17), osteoporosis (OR = 6...
October 3, 2017: Oncotarget
https://www.readbyqxmd.com/read/29108119/duct-to-duct-biliary-reconstruction-in-pediatric-split-liver-transplantation
#13
Cahit Yilmaz, Can A Karaca, Rasim Ferecov, Zaza Iakobadze, Kamil Kilic, Adnan Tosun, Murat Kilic
No abstract text is available yet for this article.
November 6, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/29105236/liver-abnormalities-and-post-transplant-survival-in-pediatric-fontan-patients
#14
Sophie C Hofferberth, Tajinder P Singh, Heather Bastardi, Elizabeth D Blume, Francis Fynn-Thompson
The impact of liver parenchymal abnormalities on survival post-heart transplant remains unknown in pediatric Fontan patients. We assessed pediatric Fontan patients who underwent heart transplant and had documented pretransplant hepatic ultrasound (U/S) studies. Liver U/S findings were classified as normal (Group 1), mildly abnormal (Group 2, hepatomegaly/vascular congestion), or severely abnormal (Group 3, heterogeneous echotexture/nodularity). Among 30 study patients, 8 were classified as Group 1, 14 as Group 2, while 8 met Group 3 criteria...
November 3, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/29098318/-wilson-s-disease-what-has-been-confirmed-in-diagnostic-and-therapy
#15
REVIEW
E-D Pfister
Wilson's disease (WD) is a rare autosomal recessive disorder characterized by abnormal copper accumulation. Presenting a broad variety of phenotypes and, thus, being a chameleon within the group of metabolic diseases, the manifold clinical symptoms of WD can include hepatologic, neurologic, and psychiatric manifestations. Early onset presentations in infancy and late-onset manifestations in adults older than 70 years of age have been described. If the typical laboratory blood test values are missing, the diagnosis of WD may be difficult and often involves a combination of different parameters...
November 2, 2017: Der Internist
https://www.readbyqxmd.com/read/29082657/segmental-branching-pattern-of-the-left-portal-vein-anatomical-characteristics-and-clinical-implications
#16
Haythem Najah, Houssem Ammar, Rahul Gupt, Moncef Hamdoun, Abdelwaheb Morjane, Mongi Mighri, Sadok Sassi
INTRODUCTION: The existing knowledge on anatomy of segmental branches of left portal vein (LPV) is limited. This study aims to describe the surgical anatomy and variations of LPV and its segmental branching pattern. MATERIALS AND METHODS: Forty fresh cadaveric liver dissections were performed. The dissection of LPV was carried out from its emergence at the level of the portal vein bifurcation to its segmental branches penetrating the left hemiliver. LPV characteristics, the number and situation of its segmental branches were recorded...
October 30, 2017: Clinical Anatomy
https://www.readbyqxmd.com/read/29080241/next-generation-sequencing-in-pediatric-hepatology-and-liver-transplantation
#17
REVIEW
Emanuele Nicastro, Lorenzo D'Antiga
Next Generation Sequencing (NGS) has revolutionized the analysis of human genetic variations, offering a highly cost-effective way to diagnose monogenic diseases (MDs). Since nearly half of children with chronic liver disorders have a genetic cause and approximately 20% of pediatric liver transplants are performed in children with MDs, NGS offers the opportunity to significantly improve the diagnostic yield in this field. Among the NGS strategies, the use of targeted gene panels has proven useful to rapidly and reliably confirm a clinical suspicion, whereas the whole exome sequencing (WES) with variants filtering has been adopted to assist the diagnostic work up in unclear clinical scenarios...
October 28, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/29077642/long-term-follow-up-after-pediatric-liver-transplantation-predictors-of-growth
#18
Nathalie Loeb, Jillian S Owens, Michele Strom, Farsad Farassati, Krista Van Roestel, Kathryn Chambers, Penni Kean, Vicky L Ng, Yaron Avitzur, Megan Carricato, Paul Wales, Glenda Courtney Martin
OBJECTIVES: To describe long-term growth post-pediatric liver transplantation and to conduct bivariate and multivariate analysis of factors that may predict post-transplantation growth in children who received a liver transplant from January 1999 to December 2008 at the Hospital for Sick Children. METHODS: A retrospective cohort study was conducted with follow-up of up-to 10 years post-transplantation. Mean height and weight z-scores and annual differences in mean z-scores were plotted against time after transplantation...
October 27, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29076289/orthotopic-liver-transplantation-for-sensenbrenner-syndrome
#19
Ruth Linda Ackah, Dor Yoeli, Michael Kueht, Nhu Thao Nguyen Galván, Ronald Timothy Cotton, Abbas Rana, Christine Ann O'Mahony, John Alan Goss
Sensenbrenner syndrome, or cranioectodermal dysplasia, is a rare heterogeneic autosomal recessive disorder, affecting ~1 of 1 000 000 live births. The syndrome usually manifests within the first year of life and can present with progressive liver and renal involvement. For all Sensenbrenner patients, renal and liver diseases are the main contributors of morbidity and mortality. In this report, we present the case of a 7-year-old boy with congenital liver disease progressing to liver failure secondary to Sensenbrenner syndrome...
October 26, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/29071779/risk-factors-and-clinical-outcomes-of-pediatric-liver-transplant-recipients-with-post-transplant-lymphoproliferative-disease-in-a-multi-ethnic-asian-cohort
#20
James Guoxian Huang, Mervin Ye Qing Tan, Seng-Hock Quak, Marion Margaret Aw
BACKGROUND: We aimed to evaluate clinical characteristics, risk factors, and disease outcomes for liver transplant recipients (LTR) with post-transplant lymphoproliferative disease (PTLD) at our center. METHODS: Retrospective review of data of all pediatric LTR (1991-2015) was conducted. RESULTS: The overall incidence of PTLD was 16.4% (18/110), the majority (13/18) were early lesions, while 3/18 were polymorphic/monomorphic PTLD. The risk factors significant on univariate analysis were as follows: mean age (years) at transplant (1...
October 26, 2017: Transplant Infectious Disease: An Official Journal of the Transplantation Society
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