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https://www.readbyqxmd.com/read/30095578/incidence-and-clinical-features-of-autoimmune-hepatitis-in-the-province-of-santa-fe-argentina
#1
Alejandro Carlos Costaguta, Andrea González, Sandra Pochettino, Liliana Trotta, Rosalía Vicentín, Marta Wagener
OBJETIVES: To investigate the incidence and clinical features of autoimmune hepatitis (AIH) in children from the province of Santa Fe, Argentina, over ten years. METHODS: From the records of all of the pediatric hepatologists in the province of Santa Fe, Argentina, we reviewed the clinical charts of patients < 18 years who were diagnosed with AIH (simplified score >6 points) and followed between January 2003 and December 2013. Population data were extracted from the 2010 national census...
August 8, 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/30091842/burkholderia-multivorans-septicemia-in-a-pediatric-liver-transplant-patient
#2
Shaun S C Ho, Nancy Nashid, Valerie J Waters, John J LiPuma, James E A Zlosnik, Anthony Otley, Gino R Somers, Binita M Kamath, Yvonne C W Yau
'Cepacia syndrome' caused by Burkholderia cepacia complex and often found to be associated in patients with cystic fibrosis, carries a high mortality rate. It is rare for Burkholderia multivorans, a species within the Burkhoderia cepacia complex to cause 'cepacia syndrome' even among the cystic fibrosis patients. This is the first reported fatal case of 'cepacia syndrome' caused by Burkholderia multivorans occurring in a non-cystic fibrosis pediatric liver transplant recipient. We describe the unique characteristics of this pathogen among the non-cystic fibrosis population and the importance of early recognition and treatment...
August 9, 2018: American Journal of Transplantation
https://www.readbyqxmd.com/read/30087518/management-of-portal-hypertension-in-the-pediatric-population-a-primer-for-the-interventional-radiologist
#3
REVIEW
Victoria Young, Shankar Rajeswaran
Interventional radiology's role in the management of portal hypertension in the pediatric population differs from the management of adult portal hypertension. In the pediatric population, portal hypertension is frequently secondary to thrombosis and cavernous transformation of the extrahepatic portion of the portal vein. Transjugular intrahepatic portosystemic shunt can be utilized to manage portal hypertension in children with intrinsic liver disease that results in cirrhosis and portal hypertension, and is often used as a bridge to transplant...
August 2018: Seminars in Interventional Radiology
https://www.readbyqxmd.com/read/30083892/post-transplant-disease-recurrence-in-pediatric-psc
#4
REVIEW
Nisreen Soufi, Fateh Bazerbachi, Mark Deneau
PURPOSE OF REVIEW: Review the current knowledge about recurrent primary sclerosing cholangitis (rPSC) after transplant in children. RECENT FINDINGS: Recurrent PSC is a significant complication that afflicts 16% of children after liver transplantation for primary sclerosing cholangitis (PSC) at a median onset of 38 months post-transplant. Possible risk factors include younger age at PSC diagnosis or transplant, the presence of overlap syndrome or IBD, and post-transplant induction with thymoglobulin...
August 6, 2018: Current Gastroenterology Reports
https://www.readbyqxmd.com/read/30082490/strategies-for-recognizing-and-managing-immune-mediated-adverse-events-in-the-treatment-of-hodgkin-lymphoma-with-checkpoint-inhibitors
#5
Santosha Vardhana, Kara Cicero, Moises J Velez, Craig H Moskowitz
The programmed death-1 (PD-1) receptor checkpoint inhibitors nivolumab and pembrolizumab represent an important therapeutic advance in the treatment of relapsed or refractory classical Hodgkin lymphoma (cHL). Clinical trials have shown substantial therapeutic activity and an acceptable safety profile in heavily pretreated patients, resulting in U.S. Food and Drug Administration approval of nivolumab for the treatment of cHL that has relapsed or progressed after either autologous hematopoietic cell transplantation (auto-HCT) and brentuximab vedotin treatment or three or more lines of systemic therapy (including auto-HCT), and of pembrolizumab for adult or pediatric patients with refractory cHL or cHL that has relapsed after three or more prior therapies...
August 6, 2018: Oncologist
https://www.readbyqxmd.com/read/30076624/clinical-consequences-of-cardiomyopathy-in-children-with-biliary-atresia-requiring-liver-transplantation
#6
Noelle M Gorgis, Curtis Kennedy, Fong Lam, Kathleen Thompson, Jorge Coss-Bu, Ayse Akcan Arikan, Trung Nguyen, Kathleen Hosek, Tamir Miloh, Saul J Karpen, Daniel J Penny, John Goss, Moreshwar S Desai
Cirrhotic Cardiomyopathy (CCM), a comorbidity of end-stage cirrhotic liver disease, remains uncharacterized in children, largely due to a lack of an established pediatric definition. The aim of this retrospective cohort analysis is to derive objective 2-dimensional echocardiographic (2DE) criteria to define CCM associated with biliary atresia (BA), or BA-CCM, and correlate presence of BA-CCM with liver transplant (LT) outcomes in this population. Using receiver operating characteristic (ROC) curve analysis, optimal cutoff values for left ventricular (LV) geometric parameters that were highly sensitive and specific for the primary outcomes: a composite of serious adverse events (CSAE) and peri-transplant death were determined...
August 3, 2018: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/30066626/successful-treatment-of-idiopathic-thrombocytopenic-purpura-after-liver-transplant-a-case-report
#7
Amir Human Hoveidaei, Hoda Soufi, Seyed Mohsen Dehghani, Mohammad Hadi Imanieh
Idiopathic thrombocytopenic purpura, a common acquired bleeding disorder in pediatric patients, is an autoimmune disorder characterized by a low platelet count. Organ transplant can transfer such diseases, but the occurrence of idiopathic thrombocytopenic purpura after liver transplant is rare. Here, we report a 17-month-old girl who was diagnosed with idiopathic thrombocytopenic purpura and who had a history of liver transplant 11 months earlier. Treatment of cytomegalovirus and Epstein-Barr virus infection led to a successful outcome...
July 31, 2018: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/30060738/a-case-report-of-full-recovery-from-severe-cerebral-edema-secondary-to-acetaminophen-induced-hepatotoxicity-in-a-13-year-old-girl
#8
Emily B Austin, Hailey Hobbs, Brian A Crouse, Anna-Theresa Lobos
BACKGROUND: Acetaminophen is a common cause of acute liver failure in pediatrics. Cerebral edema is a significant complication of acute hepatic failure and is associated with increased mortality. CASE PRESENTATION: We present a case of a 13 -year old girl with severe cerebral edema secondary to acetaminophen toxicity and hepatic failure. Her poor neurological status precluded her from liver transplantation and withdrawal of life sustaining treatment was recommended...
July 30, 2018: BMC Pediatrics
https://www.readbyqxmd.com/read/30059274/successful-integration-of-contrast-enhanced-us-into-routine-abdominal-imaging
#9
Xiaoyang Liu, Hyun-Jung Jang, Korosh Khalili, Tae Kyoung Kim, Mostafa Atri
Contrast material-enhanced US is recognized increasingly as a useful tool in a wide variety of hepatic and nonhepatic applications. The modality recently was approved for limited use for liver indications in adult and pediatric patients in the United States. Contrast-enhanced US uses microbubbles of gas injected intravenously as a contrast agent to demonstrate blood flow and tissue perfusion. The growing worldwide application of contrast-enhanced US in multiple organ systems is due largely to its advantages, including high contrast resolution (sensitivity to the contrast agent), real-time imaging, lack of nephrotoxicity, the purely intravascular property of microbubble contrast agents that allows the use of disruption-replenishment techniques, and repeatability during the same examination...
July 30, 2018: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/30052569/quality-of-life-in-patients-with-progressive-familial-intrahepatic-cholestasis-no-difference-between-post-liver-transplantation-and-post-partial-external-biliary-diversion
#10
Sonja Wassman, Eva-Doreen Pfister, Joachim F Kuebler, Benno M Ure, Imeke Goldschmidt, Jens Dingemann, Ulrich Baumann, Nagoud Schukfeh
OBJECTIVES: In patients with progressive familial intrahepatic cholestasis (PFIC), partial external biliary diversion (PEBD), which is associated with a permanent stoma, is recommended as first-line therapy, whereas primary liver transplantation (LTx) is restricted to those with cirrhosis. Our aim was to quantify the health-related quality of life (HRQOL) in PFIC patients and to evaluate whether there is a difference in their HRQOL depending on the surgical approach. METHODS: A prospective HRQOL study on a consecutive series of PFIC was conducted using Pediatric Quality of Life Inventory 4...
July 26, 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/30051556/cytomegalovirus-infection-in-pediatric-patients-with-hepatoblastoma-after-liver-transplantation
#11
Seishiro Nodomi, Katsutsugu Umeda, Itaru Kato, Satoshi Saida, Hidefumi Hiramatsu, Eri Ogawa, Atsushi Yoshizawa, Shinya Okamoto, Hideaki Okajima, Shinji Uemoto, Souichi Adachi
No studies have examined CMV infection in pediatric patients with HB receiving LT. Here, we retrospectively analyzed the incidence of and risk factors for CMV infection in 24 pediatric patients with HB who underwent LT between 1997 and 2015. CMV infection was monitored by measuring expression of pp65 CMV antigen for up to 4 months post-LT. CMV infection, defined as detection of at least one pp65-positive leukocyte, was detected in nine (37.5%) patients who did not develop CMV disease. Nine (47.4%) of nineteen patients who received post-LT chemotherapy experienced CMV infection; however, no CMV infection was observed in the five patients who did not receive post-LT chemotherapy (P = 0...
July 26, 2018: Pediatric Transplantation
https://www.readbyqxmd.com/read/30050257/biliary-atresia-changing-trends-in-management-outlook-of-a-pediatric-liver-transplant-surgeon
#12
EDITORIAL
T Renu Kumar
No abstract text is available yet for this article.
July 2018: Journal of Indian Association of Pediatric Surgeons
https://www.readbyqxmd.com/read/30048393/elevated-risk-of-cancer-following-solid-organ-transplant-in-childhood-a-population-based-cohort-study
#13
Abhijat Kitchlu, Stephanie Dixon, Jade S Dirk, Rahul Chanchlani, Jovanka Vasilevska-Ristovska, Karlota Borges, Anne I Dipchand, Vicky L Ng, Diane Hebert, Melinda Solomon, J Michael Paterson, Sumit Gupta, S Joseph Kim, Paul C Nathan, Rulan S Parekh
BACKGROUND: Cancer risk is elevated among adult transplant recipients, but there is limited data regarding long-term cancer risk and mortality in pediatric recipients. METHODS: We conducted a population-based retrospective cohort study in Ontario, Canada. We included pediatric recipients of solid organ transplants at the Hospital for Sick Children, Toronto from 1991 to 2014, and compared rates of new cancers and cancer-specific mortality to nontransplanted Ontario children born in the same year...
July 25, 2018: Transplantation
https://www.readbyqxmd.com/read/30043420/endoscopic-treatment-in-biliary-strictures-after-pediatric-liver-transplantation
#14
Piotr Czubkowski, Jan Pertkiewicz, Małgorzata Markiewicz-Kijewska, Piotr Kaliciński, Małgorzata Rurarz, Irena Jankowska, Joanna Pawłowska
BACKGROUND AND AIM: The aim of the study was to assess efficacy and safety of endoscopic treatment in BS after pediatric LTx. METHODS: We retrospectively reviewed data of patients with DDA who developed BS and underwent ERCP. RESULTS: Of 189 transplanted patients with DDA, strictures developed in 30 (16%). In this subgroup, the median age at LTx was 14.7 (1.5-17.6) and follow-up period was 3.9 (1.3-11.3). ABS were in 76% and NABS in combination with ABS in 24% of patients...
July 24, 2018: Pediatric Transplantation
https://www.readbyqxmd.com/read/30041524/liver-transplantation-status-in-iran-a-multi-center-report-on-the-main-transplant-indicators-and-survival-rates
#15
S Ali Malek-Hosseini, Ali Jafarian, Saman Nikeghbalian, Hossein Poustchi, Kamran B Lankarani, Mohsen Nasiri Toosi, Heshmatollah Salahi, Mohsen Dehghani, Ahad Eshraghian, Maryam Sharafkhah, Sareh Eghtesad, Nazgol Motamed-Gorji, Kourosh Kazemi, Javad Salimi, Majid Moini, Alireza Shamsaeefar, Maryam Moini, Masoud Dehghani, Mohammad B Khosravi, Atabak Najafi, Hossein Sattari, Bita Geramizadeh, Mohammad Shafiee, Mohammad N Toutouni, Behnam Sanei, Seyed Mohammadreza Nejatollahi, Alireza Taghavi, Ali Bahador, Mohsen Aliakbarian, Bijan Eghtesad, Reza Malekzadeh
BACKGROUND: Iran's experience with liver transplantation (LT) began more than two decades ago. The purpose of this article is to present the status of LT in Iran, review specific characteristics of the programs, their outcomes, and their growth to become one of the largest LT programs in the world. METHODS: A questionnaire, asking for data on the number of transplants performed and specifics of the recipients and type of donors with focus on indications and outcomes was sent to LT programs...
July 1, 2018: Archives of Iranian Medicine
https://www.readbyqxmd.com/read/30035306/persistence-of-sarcopenia-after-pediatric-liver-transplantation-is-associated-with-poorer-growth-and-recurrent-hospital-admissions
#16
Diana R Mager, Amber Hager, Poh Hwa Ooi, Kerry Siminoski, Susan M Gilmour, Jason Y K Yap
INTRODUCTION: Sarcopenia is prevalent in adults pre-liver transplantation (LTx) and post-LTx contributing to adverse outcomes. Little is known regarding the prevalence of sarcopenia in pediatric LTx recipients. This novel study examined sarcopenia prevalence and associations with post-LTx growth and healthcare utilization in pediatric LTx recipients. METHODS: We prospectively assessed body composition at annual clinical appointments in children (0.5-17 years; n = 58) by Dual-energy-X-ray absorptiometry (absolute/regional/percent fat mass [FM], fat-free mass [FFM], skeletal muscle mass [SMM])...
July 23, 2018: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/30028076/dsa-s-meaningful-impact-on-liver-transplantation
#17
EDITORIAL
Ariel W Aday, Jacqueline G O'Leary
The new Banff criteria for rejection recognize the role of Donor Specific Antibodies in acute and chronic immunologic complications post-LT. Although rare, acute AMR is clearly involved in liver allograft damage and loss. Emerging data have also confirmed in both pediatric and adult LT that chronic AMR manifests typically as fibrosis progression in patients with normal or near normal liver chemistries and is more common than previously appreciated. This article is protected by copyright. All rights reserved...
July 20, 2018: Liver Transplantation
https://www.readbyqxmd.com/read/30021057/predictive-factors-for-survival-in-children-liver-transplanted-for-wilson-disease-a-cohort-study-using-eltr-data
#18
Eva-Doreen Pfister, André Karch, Wojciech G Polak, Vincent Karam, René Adam, Christophe Duvoux, Darius Mirza, John O Grady, Jürgen Klempnauer, Raymond Reding, Piotr Kalicinski, Ahmet Coker, Pavel Trunecka, Ibrahim Astarcioglu, J C Duclos-Vallée, Johann Pratschke, Andreas Paul, Irinel Popescu, Stefan Schneeberger, Oliver Boillot, Björn Nashan, Rafael T Mikolajczyk, Ulrich Baumann
Liver transplantation (LTx) is a rescue therapy for life threatening complications of Wilson disease (WD). However, data on the outcome of WD patients post LTx are scarce. The aim of our study was to analyse a large pediatric WD cohort with the aim of investigating the long-term outcome of pediatric WD patients after LTx, and to identify predictive factors for patient and transplant survival. This is a retrospective cohort study using data of all children (<18 years) liver transplanted for WD enrolled in the European Liver Transplant Registry (ELTR) from 1968 until December 2013...
July 18, 2018: Liver Transplantation
https://www.readbyqxmd.com/read/30021047/getting-better-with-age-and-time-a-look-back-at-the-european-experience-for-liver-transplantation-for-pediatric-patients-with-wilson-s-disease
#19
EDITORIAL
Michael L Schilsky
Since the seminal experience of Starzl et al (1), liver transplant (LT) has been performed for Wilson's disease patients with failing livers and more controversially for neurologic disease. The majority of patients transplanted for Wilson's disease in a registry from the United States (2) and in a report from France (3) were adults with end-stage liver disease and its complications. These series each included pediatric patients where there was a marked increase in patients with acute liver failure due to Wilson's disease...
July 18, 2018: Liver Transplantation
https://www.readbyqxmd.com/read/30019834/long-term-outcomes-of-transmesenteric-portal-vein-recanalization-for-the-treatment-of-chronic-portal-vein-thrombosis-after-pediatric-liver-transplantation
#20
A C B S Cavalcante, C E Zurstrassen, F C Carnevale, R P S Pugliese, E A Fonseca, A M Moreira, Jpk Matushita, H L L Cândido, M A R Benavides, I K Miura, V L B Danesi, A P M Hirschfeld, C B V Borges, G Porta, P ChapChap, J Seda-Neto
Portal vein thrombosis (PVT) may occur at any time following liver transplantation. We describe our experience with portal vein recanalization in cases of thrombosis after liver transplantation. Twenty-eight children (5%) out of 566 liver transplant recipients underwent portal vein recanalization using a transmesenteric approach. All children received left hepatic segments, developed PVT and had symptoms or signs of portal hypertension. Portal vein recanalization was performed via the transmesenteric route in all cases...
July 18, 2018: American Journal of Transplantation
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