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Pediatric AND liver AND transplant

T Ueno, S Hiwatashi, R Saka, H Yamanaka, Y Takama, Y Tazuke, K Bessho, S Kogaki, T Yonekura, H Okuyama
Pediatric living donor liver transplantation (LDLT) in patients with advanced portopulmonary hypertension (PoPH) is associated with poor prognoses. Recently, novel oral medications, including endothelin receptor antagonists (ERAs), phosphodiesterase 5 (PDE5) inhibitors, and oral prostacyclin (PGI2) have been used to treat PoPH. Pediatric patients with PoPH who underwent LDLT from 2006 to 2016 were enrolled. Oral pulmonary hypertension (PH) medication was administered to control pulmonary arterial pressure (PAP)...
March 15, 2018: Transplantation Proceedings
T Ueno, S Hiwatashi, R Saka, H Yamanaka, Y Takama, Y Tazuke, K Bessho, H Okuyama
Chronic rejection (CR) remains a challenging complication after liver transplantation. Everolimus, which is a mammalian target of rapamycin inhibitor, has an anti-fibrosis effect. We report here the effect of everolimus on CR. Case 1 was a 7-year-old girl who underwent living donor liver transplantation (LDLT) shortly after developing fulminant hepatitis at 10 months of age. Liver function tests (LFTs) did not improve after transplantation despite treatment with tacrolimus + mycophenolate mofetil (MMF). Antithymoglobulin (ATG) and steroid pulse therapy were also ineffective...
March 19, 2018: Transplantation Proceedings
Sara Gattis, Inci Yildirim, Andi L Shane, Staci Serluco, Courtney McCracken, Rochelle Liverman
Background: In solid organ transplant (SOT) recipients, influenza infection can lead to subsequent graft dysfunction and death. Vaccination is the most effective approach to preventing influenza infection; however, vaccination rates are low, and interventions to optimize vaccine coverage are needed. The purpose of this study was to evaluate if pharmacy-initiated screening and recommendations for influenza immunization improve the rate of vaccination in pediatric SOT recipients. Methods: We performed a retrospective pre-post chart review of all kidney, liver, and heart transplant recipients followed by Children's Healthcare of Atlanta/Emory University transplant services between September 1, 2011, and February 16, 2017...
October 11, 2018: Journal of the Pediatric Infectious Diseases Society
Thomas Gossios, Ioanna Zografou, Veta Simoulidou, Athina Pirpassopoulou, Konstantinos Christou, Asterios Karagiannis
BACKGROUND: Familial hypercholesterolemia (FH) is an autosomal-dominant genetic disease, associated with premature atherosclerotic cardiovascular disease (CVD), especially in its homozygous type (HoFH). OBJECTIVE: The aim of this review is to discuss the safety and efficacy of combination treatments (procedures and drugs) for HoFH. RESULTS: Historically, liver transplantation was used first; however, it is currently considered only as a last resort for some patients...
October 8, 2018: Current Pharmaceutical Design
Şükrü Güngör, Mukadder Ayşe Selimoğlu, Fatma İlknur Varol, Serdal Güngör
BACKGROUND: Wilson's disease (WD) may present with different manifestations: from an asymptomatic state to liver cirrhosis. Here, we aimed to evaluate clinical presentations and laboratory findings and prognoses among WD cases. DESIGN AND SETTING: Cross-sectional study based on patients' records from the university hospital, İnönü University, Malatya, Turkey. METHODS: The medical records of 64 children with WD were evaluated focusing on the clinical, laboratory and liver biopsy findings in different clinical presentations...
July 2018: São Paulo Medical Journal, Revista Paulista de Medicina
Pierluigi Toniutto, Davide Bitetto, Ezio Fornasiere, Elisa Fumolo
Liver transplantation (LT) has become the treatment of choice for a wide range of liver diseases in both adult and pediatric patients. Until recently, the largest proportion of LT in adults, were performed in patients with hepatitis C (HCV) related cirrhosis. The recent availability of safe and effective direct antiviral agents to cure HCV infection in almost all patients whatever the HCV genotype and severity of liver disease, will reduce the need for LT in this category of recipients. Thus, it is presumed that in the next 1 to 2 decades HCV related liver disease will diminish substantially, whereas non-alcoholic steato-hepatitis (NASH) will correspondingly escalate as an indication for LT...
October 5, 2018: Minerva Gastroenterologica e Dietologica
Jacob S Ghannam, Michael R Cline, Anthony N Hage, Jeffrey Forris Beecham Chick, Rajiv N Srinivasa, Narasimham L Dasika, Ravi N Srinivasa, Joseph J Gemmete
BACKGROUND: Transjugular intrahepatic portosystemic shunt (TIPS) placement has been extensively studied in adults. The experience with TIPS placement in pediatric patients, however, is limited. OBJECTIVE: The purpose of this study was to report technical success and clinical outcomes in pediatric patients undergoing TIPS placement. MATERIALS AND METHODS: Twenty-one children - 12 (57%) boys and 9 (43%) girls, mean age 12.1 years (range, 2-17 years) - underwent TIPS placement from January 1997 to January 2017...
October 6, 2018: Pediatric Radiology
Lieu T Tran, George V Mazariegos, Daniela Damian, Peter J Davis
BACKGROUND: Liver transplantation in children is often associated with coagulopathy and significant blood loss. Available data are limited. In this observational retrospective study, we assessed transfusion practices in pediatric patients undergoing liver transplantation at a single institution over the course of 9 years. METHODS: Data were retrospectively collected from patient medical records at the Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center...
October 3, 2018: Anesthesia and Analgesia
Jin Ge, Evelyn K Hsu, John Bucuvalas, Jennifer C Lai
BACKGROUND AND AIMS: Each year, approximately 60 children, representing 12% of waitlist candidates, die awaiting liver transplantation. The current allocation algorithm for pediatric donor livers prioritizes local/regional adults over national children. We attempted to better understand the impact of the present algorithm on pediatric candidates. METHODS: We analyzed pediatric donor liver offers from 2010 to 2014. Donors and recipients were classified based on age...
October 3, 2018: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
Mureo Kasahara, Kengo Sasaki, Hajime Uchida, Yoshihiro Hirata, Masahiro Takeda, Akinari Fukuda, Seisuke Sakamoto
PV hypoplasia may increase the risk of posttransplant complications, especially when it extends to near the SMV and SpV junction. We described our experience of 10 pediatric cases of PV hypoplasia/thrombus in which the pullout technique was required for PV reconstruction. There were five male and five female patients. The median age was 9 months, and the median weight was 8.1 kg. The indications for the pullout technique were PV hypoplasia in seven patients and PV thrombus in 3. The inflow sites of the enlarged LGV were as follows: the main PV trunk (n = 2), the SMV and SpV junction (n = 4), and the SpV (n = 4)...
October 2, 2018: Pediatric Transplantation
Juliet A Emamaullee, Victor Nekrasov, Susan Gilmour, Norman Kneteman, George Yanni, Rohit Kohli, Daniel Thomas, Yuri Genyk
BACKGROUND: ADH is a rare and potentially fatal complication following LT. In this study, a systematic review was completed to identify risk factors which may contribute to ADH. METHODS: Transplant databases at three LT programs were reviewed. Four pediatric and zero adult cases were identified. Next, a systematic review was completed. Fourteen studies describing 41 patients with ADH were identified. Patient demographics, transplant characteristics, and features of ADH diagnosis were examined...
October 2, 2018: Pediatric Transplantation
Anthony P Y Liu, Janice J K Ip, Alex W K Leung, C W Luk, C H Li, Karin K H Ho, Regina Lo, Edwin K W Chan, Albert C Y Chan, Patrick H Y Chung, Alan K S Chiang
BACKGROUND AND AIM: We reviewed the results and pattern of failure of the consensus HB/HCC 1996 treatment protocol for pediatric hepatoblastoma (HB) in Hong Kong. The role of SIOPEL and Children's Hepatic tumors International Collaboration (CHIC) risk stratification was evaluated. METHODS: Patients enrolled on the protocol from 1996 to 2014 were included. PRETEXT staging, SIOPEL, and CHIC risk groups were retrospectively assigned. RESULTS: Sixty patients were enrolled with median age at diagnosis of 1...
September 30, 2018: Pediatric Blood & Cancer
Joohyun Kim, Michael A Zimmerman, Stacee M Lerret, John P Scott, Stylianos Voulgarelis, Roger A Fons, Bernadette E Vitola, Grzegorz W Telega, George M Hoffman, Richard J Berens, Johnny C Hong
INTRODUCTION: Biliary complications after pediatric orthotopic liver transplantation remain causes of significant patient morbidity. Staged operative approach in complex hepatobiliary surgery has improved postoperative outcomes but has not been evaluated in pediatric orthotopic liver transplantation. We sought to analyze the outcomes of staged biliary reconstruction after orthotopic liver transplantation in high acuity patients. METHODS: A retrospective analysis of 43 pediatric orthotopic liver transplantations at our center (January 2013 through December 2017)...
September 26, 2018: Surgery
Dmitri Bezinover, Molly F Deacutis, Priti G Dalal, Robert P Moore, Jonathan G Stine, Ming Wang, Ethan Reeder, Christopher S Hollenbeak, Fuat H Saner, Thomas R Riley, Piotr K Janicki
BACKGROUND: This retrospective UNOS database evaluation analyzes the prevalence of preoperative portal vein thromboses (PVT), and postoperative thromboses leading to graft failure in pediatric patients undergoing liver transplantation (LT). METHODS: The evaluation was performed in three age groups: I (0-5), II (6-11), III (12-18) years old. Factors predictive of pre- and postoperative thromboses were analyzed. RESULTS: Between 2000 and 2015, 8982 pediatric LT were performed in the US...
September 25, 2018: HPB: the Official Journal of the International Hepato Pancreato Biliary Association
Yen H Pham, Tamir Miloh
Liver transplantation (LT) for children has excellent short- and long-term patient and graft survival. LT is a lifesaving procedure in children with acute or chronic liver disease, hepatic tumors, and a few genetic metabolic diseases in which it can significantly improve quality of life. In this article, the authors discuss the unique aspects of pediatric LT, including the indications, patient selection and evaluation, allocation, transplant surgery and organ selection, posttransplant care, prognosis, adherence, and transition of care...
November 2018: Clinics in Liver Disease
James E Squires, Patrick McKiernan, Robert H Squires
Pediatric acute liver failure (PALF) is a dynamic, life-threatening condition of disparate etiology. Management of PALF is dependent on intensive collaborative clinical care and support. Proper recognition and treatment of common complications of liver failure are critical to optimizing outcomes. In parallel, investigations to identify underlying cause and the implementation of timely, appropriate treatment can be life-saving. Predicting patient outcome in the era of liver transplantation has been unfulfilling and better predictive models must be developed for proper stewardship of the limited resource of organ availability...
November 2018: Clinics in Liver Disease
N Junge, J Dingemann, C Petersen, M P Manns, N Richter, J Klempnauer, U Baumann, A Schneider
BACKGROUND: A growing number of patients with biliary atresia and congenital cholestatic syndromes are reaching adulthood. These patients often have a number of typical medical features, including specific characteristics of liver transplantation medicine. OBJECTIVE: What are the special features in the care of adults suffering from liver diseases with manifestation in childhood and adolescence, both before and after liver transplantation (LTX). How does the progression of individual diseases differ depending on age at manifestation? What are specific aspects following pediatric LTX? PATIENTS AND METHODS: Evaluation and discussion of existing guidelines and recommendations of the individual disciplines and professional societies as well as the current literature...
September 27, 2018: Der Internist
Rajeev Khanna, Sanjeev Kumar Verma
Pediatric hepatocellular carcinoma (HCC) is the second common malignant liver tumor in children after hepatoblastoma. It differs from the adult HCC in the etiological predisposition, biological behavior and lower frequency of cirrhosis. Perinatally acquired hepatitis-B virus, hepatorenal tyrosinemia, progressive familial intrahepatic cholestasis, glycogen storage disease, Alagille's syndrome and congenital portosystemic shunts are important predisposing factors. Majority of children (87%) are older than 5 years of age...
September 21, 2018: World Journal of Gastroenterology: WJG
Ying Liu, Li-Ying Sun, Zhi-Jun Zhu, Lin Wei, Wei Qu, Zhi-Gui Zeng
BACKGROUND Progressive familial intrahepatic cholestasis (PFIC) is an autosomal recessive inherited disease that disrupts the genes for bile formation. Liver transplantation (LT) is the only effective treatment for PFIC patients with end-stage liver disease. We describe our experience in terms of clinical characteristics, complications, and outcome of LT for PFIC. CASE REPORT The data of 5 pediatric PFIC patients recipients (3 PFIC1, 1 PFIC2, and 1 PFIC3) who received LT at our Liver Transplant Center from June 2013 to February 2017 were retrospectively analyzed...
September 25, 2018: Annals of Transplantation: Quarterly of the Polish Transplantation Society
Guglielmo Paolantonio, Andrea Pietrobattista, George Koshy Parapatt, Daniela Liccardo, Gian Luigi Natali, Manila Candusso, Marco Spada, Massimo Rollo, Paolo Tomà
Stenosis of the HJ is a common complication of pediatric split LT with high morbidity and possible evolution to secondary biliary cirrhosis and re-transplantation if not treated. Because the endoscopy is generally infeasible in the Roux-en-Y, percutaneous interventional radiology management is usually the safest and most effective approach to avoid surgical revision of a stenotic bilio-enteric anastomosis. We present the case of a child with acute onset of cholestasis 7 months after left lateral segment partial LT due to occlusion of the HJ...
September 23, 2018: Pediatric Transplantation
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