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Pediatric AND liver AND transplant

Alvaro Torres, Seppo K Koskinen, Henrik Gjertsen, Björn Fischler
Our main goal with this study was to share our off-label experience with CEUS for identifying circulatory complications after liver transplantation in children. A total of 74 CEUS examinations performed on 34 pediatric patients who underwent a liver transplant were retrospectively included. About 53% of the examinations were performed on children 2 years old or younger. About 82% of the examinations were performed within 30 days from the transplant. About 62% of patients were transplanted due to a cholestatic disease, 11% due to a metabolic disease, 8% were re-transplanted due to graft failure, and 19% was due to other conditions...
December 7, 2018: Pediatric Transplantation
Piotr Czubkowski, Malgorzata Markiewicz-Kijewska, Kazimierz Janiszewski, Małgorzata Rurarz, Piotr Kaliciński, Dorota Jarzębicka, Jan Pertkiewicz, Diana Kamińska, Irena Jankowska, Mikołaj Teisseyre, Marek Szymczak, Joanna Pawłowska
BACKGROUND Biliary strictures (BS) are frequent after pediatric liver transplantation (LTx) and in spite of ongoing progress, they remain a significant cause of morbidity. In children, the majority of reconstruction is hepatico-jejunal anastomosis (HJA). The aim of this study was to analyze our experience in percutaneous transhepatic treatment of BS. MATERIAL AND METHODS Between 1998 and 2014, 589 (269 living donor) pediatric LTx were performed in our institution. We retrospectively reviewed clinical data of patients with HJA who developed BS and who underwent percutaneous transhepatic biliary drainage (PTBD)...
December 11, 2018: Annals of Transplantation: Quarterly of the Polish Transplantation Society
Jérôme Dumortier, Eduardo Couchonnal, Florence Lacaille, Christine Rivet, Dominique Debray, Olivier Boillot, Alain Lachaux, Oanez Ackermann, Emmanuel Gonzales, Barbara E Wildhaber, Emmanuel Jacquemin, Valérie McLin
BACKGROUND: During the past decade, mTOR inhibitors (mTORi), everolimus and sirolimus, have been increasingly used after adult liver transplantation (LT). The aim of the present study was to describe the use of mTORi in pediatric LT recipients. METHODS: All pediatric LT recipients who received mTORi before December 2017 from 4 European pediatric LT centers were included and analyzed. RESULTS: The present retrospective study included 30 patients; 21 were male (70%), median age was 9...
December 6, 2018: Clinics and Research in Hepatology and Gastroenterology
Mar Miserachs, Arpita Parmar, Agnieszka Bakula, Loreto Hierro, Lorenzo D'Antiga, Imeke Goldschmidt, Dominique Debray, Valérie A McLin, Valeria Casotti, Joanna Pawłowska, Carmen Camarena, Anthony R Otley, Ulrich Baumann, Vicky L Ng
OBJECTIVE: Pediatric recipients of liver transplantation (LT) often report lower Health-Related Quality of Life (HRQOL) than healthy controls when assessed on generic HRQOL measurement tools. The recent addition of the Pediatric Liver Transplant Quality of Life (PeLTQL), a novel disease-specific HRQOL instrument for pediatric LT recipients, into the clinical armamentarium of tools now routinely available to clinical care teams, provides the unique opportunity to identify disease-related challenges in children who have undergone this life-saving intervention...
December 6, 2018: Clinics and Research in Hepatology and Gastroenterology
Beau Kelly, James E Squires, Brian Feingold, David K Hooper, George V Mazariegos
PURPOSE OF REVIEW: Pediatric transplantation faces unique challenges in implementing dynamic quality improvement measures because of proportionally smaller volumes compared to adults, logistics of being integrated successfully within larger or complex hospital systems, lack of adult-affiliated transplant centers, varying focus in prioritization of relevant outcome metrics, and potential lack of sufficient resources. RECENT FINDINGS: To address these challenges, multiinstitutional collaborations have developed which have proven increasingly effective in driving awareness and quality improvement measures to supplement regulatory efforts in the pediatric population...
December 3, 2018: Current Opinion in Organ Transplantation
Emily R Perito, John Bucuvalas, Jennifer C Lai
Functional impairment is associated with mortality in adult liver transplant candidates. This has not been studied in pediatric liver transplant candidates. UNOS STAR files were used to investigate functional status, waitlist mortality, and post-transplant outcomes in children<18, listed 2006-16 for primary liver transplant. Functional status was categorized using the Lansky play-performance scale (LPPS), as normal/good (80-100%), moderately impaired (50-70%), or severely impaired (10-40%) by center assessment...
December 1, 2018: American Journal of Transplantation
Norihiko Tsuboi, Miku Hiratsuka, Setsushi Kaneko, Nao Nishimura, Satoshi Nakagawa, Mureo Kasahara, Takeshi Kamikubo
OBJECTIVE: To evaluate the impact of early mobilization after pediatric liver transplantation in the PICU. DESIGN: A 70-month retrospective before-after study. SETTING: Medical and surgical PICU with 20 beds at a tertiary children's hospital. PATIENTS: Seventy-five patients 2-18 years old who underwent liver transplantation and could walk before surgery. INTERVENTION: We meticulously planned and implemented an early mobilization intervention, a multifaceted framework for early mobilization practice in the PICU focusing on a multidisciplinary team approach...
November 26, 2018: Pediatric Critical Care Medicine
Mai Sato, Tetsuji Kaneko, Masao Ogura, Koichi Kamei, Shuichi Ito, Akinari Fukuda, Seisuke Sakamoto, Mureo Kasahara, Kenji Ishikura
BACKGROUND: Although chronic kidney disease (CKD) is still a common complication, the prognosis of kidney function after LT is not well known. Moreover, kidney function after LT in children with renal involvement is unknown. METHODS: We retrospectively analyzed patients aged < 20 years who underwent LT between November 2005 and March 2015 at our institute. RESULTS: The cohort included 313 pediatric LT recipients (135 males). The median age at LT was 1...
November 21, 2018: Transplantation
Sinan Holdar, Badr Alsaleem, Ali Asery, Abdulrahman Al-Hussaini
Background/Aims: Data from Western countries indicate that biliary atresia (BA) is the leading cause of end-stage liver disease in children and the most common indication for liver transplantation (LT) in the pediatric population. There is no data on the epidemiology and outcome of BA in Saudi Arabia. The main objective of our study was to understand the clinical and epidemiological characteristics and outcome of BA in the Saudi population. Patients and Methods: We retrospectively reviewed the database of infantile cholestasis cases that presented to our center from 2008 to 2015 and identified BA cases...
November 27, 2018: Saudi Journal of Gastroenterology: Official Journal of the Saudi Gastroenterology Association
Magd A Kotb, Alaa F Hamza, Hesham Abd El Kader, Magda El Monayeri, Dalia S Mosallam, Nazira Ali, Christine William Shaker Basanti, Hafez Bazaraa, Hany Abdelrahman, Marwa M Nabhan, Hend Abd El Baky, Sally T Mostafa El Sorogy, Inas E M Kamel, Hoda Ismail, Yasmin Ramadan, Safaa M Abd El Rahman, Neveen A Soliman
Primary hyperoxalurias are rare inborn errors of metabolism with deficiency of hepatic enzymes that lead to excessive urinary oxalate excretion and overproduction of oxalate which is deposited in various organs. Hyperoxaluria results in serious morbid-ity, end stage kidney disease (ESKD), and mortality if left untreated. Combined liver kidney transplantation (CLKT) is recognized as a management of ESKD for children with hyperoxaluria type 1 (PH1). This study aimed to report outcome of CLKT in a pediatric cohort of PH1 patients, through retrospective analysis of data of 8 children (2 girls and 6 boys) who presented by PH1 to Wadi El Nil Pediatric Living Related Liver Transplant Unit during 2001-2017...
November 26, 2018: Pediatric Transplantation
Christine S Hwang, Malcolm Macconmara, Dev M Desai
Pediatric liver and kidney transplantation have become the standard and accepted treatment for children with end-stage renal and liver disease. Since the first successful kidney transplant in 1954 by Dr Joseph Murray and the first liver transplant by Dr Thomas Starzl, the scope of indications for visceral organ transplantation as well as the range of recipient and donor ages has expanded. The first pediatric liver and kidney transplants, simultaneous multivisceral transplants, living-donor and donation-after-cardiac-death organs have evolved rapidly into the standard of care for end-stage renal and liver failure in children...
February 2019: Surgical Clinics of North America
Eleonora De Martin, Amelia Hessheimer, Ryan Chadha, Gokhan Kabacam, Jeremy Raganayagam, Varvara Kirchner, Marit Kalisvaart, Irene Scalera, Mamatha Bhat, Alan Contreras, Prashant Bhangui
The 24 Joint Annual Congress of the International Liver Transplantation Society (ILTS) in association with European Liver and Intestine Transplant Association (ELITA) and Liver Intensive Care Group of Europe (LICAGE) was held in Lisbon, Portugal from May 23-26, 2018. More than 1200 participants from over 60 countries including surgeons, hepatologists, anesthesiologists and critical care intensivists, radiologists, pathologists, organ procurement personnel and research scientists, came together with the common aim of improving care and outcomes for liver transplant recipients...
November 19, 2018: Transplantation
Martin I Montenovo, Kiran Bambha, Jorge Reyes, Andre Dick, James Perkins, Patrick Healey
BACKGROUND: The utilization of living donor grafts resulted in an increased availability of liver for pediatric recipients, and accordingly, this was associated with a significantly decreased waiting time before liver transplantation as well as reduced pre-transplant mortality. We hypothesized that the use of living donors in pediatric LT may lead to improved graft and patient survival, when compared to LT using deceased donors. METHODS: Retrospective cohort analysis of pediatric recipients (aged <18 years) registered in the UNOS database who received a primary liver transplant between February 2002 and December 2016...
November 18, 2018: Pediatric Transplantation
Christine S Hwang, Swee-Ling Levea, Justin R Parekh, Yun Liang, Dev M Desai, Malcolm MacConmara
INTRODUCTION: There is a mismatch that exists in donor liver organ supply and demand. DCD livers represents a potential source to increase the number of liver grafts available for use in pediatric recipients; however, there has been hesitancy to use such organs. We evaluated patient and allograft outcomes in pediatric liver transplant recipients of DCD livers. METHODS: The UNOS database was queried to examine outcomes in all liver transplant recipients from 1993 to 2017...
November 16, 2018: Pediatric Transplantation
Mirjana Balen Topić, Tomislava Skuhala, Boško Desnica, Klaudija Višković, Martin Drinković
Because of mostly asymptomatic cyst growth and often-neglected nonspecific low-grade symptoms, many cases of cystic echinococcosis (CE) caused by Echinococcus granulosus in the pediatric population are diagnosed at school age, in an advanced and even complicated stage. In 2003, after 5 months of intermittent dull upper-right abdominal pain and nausea, a 13-year-old boy was diagnosed with massive liver CE, with ∼20 round-shaped double-walled medium-sized infective cysts, which permeated the whole liver. Because of their wide distribution across the liver tissue and the risky superficial position of some cysts, liver transplantation emerged as the optimal therapeutic option...
November 14, 2018: Pediatrics
Marek Krawczyk
Experimental research on liver transplantation was introduced in Poland by Waldemar Olszewski with his team. It was in 1972. Not until 15 years later, in 1987, did Stanisław Zieliński in Szczecin and Marian Pardela in Katowice make an attempt of transplanting liver in humans. In 1989, the attempt was made by Jacek Pawlak and Marek Krawczyk in Warsaw. The first successful liver transplantation in Poland was performed by Piotr Kaliciński at the Children's Memorial Health Institute, Warsaw. Also, in early 1990s the attempts were made by Jerzy Polański in Warsaw and Piotr Szyber in Wrocław...
June 30, 2018: Polski Przeglad Chirurgiczny
Leslie Mataya, Namrata Patel, Ruba K Azzam
Autoimmune liver disease remains difficult to diagnose, and distinguishing the various causes is difficult as well. In children, it can present with wide variation, including autoimmune hepatitis (AIH), primary sclerosing cholangitis (PSC), and the "overlap syndrome" of AIH/PSC, also known as autoimmune sclerosing cholangitis. These liver disorders are thought to be immune-mediated, but their etiology remains unclear. They are not secondary to inherited or acquired diseases and they are not associated with any drugs, so they can only be diagnosed if these other diseases or conditions are excluded...
November 1, 2018: Pediatric Annals
Inessa Normatov, Shiran Kaplan, Ruba K Azzam
More than one-half of children with chronic liver disease suffer from malnutrition, which leads not only to a poor quality of life and even possibly catastrophic complications, but also to poor outcomes after a liver transplantation. These children have increased metabolic demands but often decreased intake with malabsorption and altered nutrient utilization, all of which make it difficult to keep up with nutritional demands. Assessment of a patient's nutritional status should be timely, and it should be performed routinely and proactively...
November 1, 2018: Pediatric Annals
Kristin Capone, Ruba K Azzam
Wilson's disease, also known as hepatolenticular degeneration, is an autosomal recessive genetic disorder due to a mutation of the ATP7B gene resulting in impaired hepatic copper excretion and copper accumulation in various tissues. It is associated with the classic triad of cirrhosis, neurological manifestations, and the ocular finding of Kayser-Fleischer rings; however, the clinical presentation can vary greatly from incidental findings of abnormal liver enzymes to acute liver failure necessitating liver transplant...
November 1, 2018: Pediatric Annals
Sydne Muratore, Siobhan Flanagan, David Hunter, Robert Acton
PURPOSE: Extrahepatic portal vein obstruction (EHPVO) is the most frequent cause of portal hypertension in children. Some patients are not amenable to meso-Rex bypass and alternative surgeries don't restore physiologic flow. We aim to demonstrate the feasibility and safety of minilaparotomy for recanalization of chronic EHPVO. METHODS: This 2013-2015 single-center, retrospective review included pediatric patients with chronic EHPVO who underwent minilaparotomy, mesenteric vein access, and attempted recanalization of the occluded portal vein...
November 8, 2018: Journal of Pediatric Gastroenterology and Nutrition
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