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Pediatric heart transplantation

Samuel B Goldfarb, Bronwyn J Levvey, Leah B Edwards, Anne I Dipchand, Anna Y Kucheryavaya, Lars H Lund, Bruno Meiser, Joseph W Rossano, Roger D Yusen, Josef Stehlik
No abstract text is available yet for this article.
October 2016: Journal of Heart and Lung Transplantation
Joseph W Rossano, Anne I Dipchand, Leah B Edwards, Samuel Goldfarb, Anna Y Kucheryavaya, Bronwyn J Levvey Rn, Lars H Lund, Bruno Meiser, Roger D Yusen, Josef Stehlik
No abstract text is available yet for this article.
October 2016: Journal of Heart and Lung Transplantation
Allison Ducharme-Smith, Ben Z Katz, Amy E Bobrowski, Carl L Backer, Elfriede Pahl
BKV infection and nephropathy complicate pediatric HTx, but the incidence and time course of the disease are unknown. We assessed the incidence of BKV infection and its association with kidney dysfunction in pediatric HTx recipients. A single center prospective study compared pediatric (<18 years) HTx recipients, with and without BKV infection, who received an allograft between September 2013 and December 2014. Screening of urine for BKV was performed prior to transplant, and at week 1, and at months 3, 6, 9, 12, and 15 months post-transplantation...
October 20, 2016: Pediatric Transplantation
Tanya N Antonini, Stacey S Beer, Tamir Miloh, William J Dreyer, Susan E Caudle
OBJECTIVE: The purpose of this study was to review the current literature on neuropsychological functioning in two groups of children requiring organ transplants (liver or heart) and present recent clinical data collected through the liver and cardiac transplantation programs at a large pediatric academic medical center. METHOD: Data included in this study came from 18 patients who completed evaluations for heart transplant (n = 8) or liver transplant (n = 10) between the ages of 2 and 6 years (inclusive)...
October 11, 2016: Clinical Neuropsychologist
Paul M K Gordon, Aneal Khan, Umair Sajid, Nicholas Chang, Varun Suresh, Leo Dimnik, Ryan E Lamont, Jillian S Parboosingh, Steven R Martin, Richard T Pon, Jene Weatherhead, Shelly Wegener, Debra Isaac, Steven C Greenway
Cell-free DNA (cfDNA) has significant potential in the diagnosis and monitoring of clinical conditions. However, accurately and easily distinguishing the relative proportion of DNA molecules in a mixture derived from two different sources (i.e., donor and recipient tissues after transplantation) is challenging. In human cellular transplantation, there is currently no useable method to detect in vivo engraftment, and blood-based non-invasive tests for allograft rejection in solid organ transplantation are either non-specific or absent...
2016: Frontiers in Cardiovascular Medicine
Eva Hlavackova, Martin Liska, Hana Jicinska, Jiri Navratil, Jiri Litzman
The Fontan operation or total cavopulmonal connection (TCPC) is a palliative surgical correction of rare and complex inborn cardiac malformations that are characterized by univentricular circulation. Protein-losing enteropathy (PLE) develops in 4-13% of patients after the Fontan procedure. Fontan-related PLE leads to secondary combined immunodeficiency marked by hypogammaglobulinemia and predominantly CD4+ lymphocytopenia. Here, we present 3 case reports of patients with secondary immunodeficiency after the Fontan operation...
September 30, 2016: International Archives of Allergy and Immunology
Willemijn M H Zijlstra, Johannes M Douwes, Mark-Jan Ploegstra, Usha Krishnan, Marcus T R Roofthooft, Hans L Hillege, D Dunbar Ivy, Erika B Rosenzweig, Rolf M F Berger
Congenital heart disease (CHD) is a frequent cause of pediatric pulmonary arterial hypertension (PAH), with diverse etiology and outcome. We aimed to describe phenotypic heterogeneity in pediatric PAH associated with CHD (PAH-CHD), assess the applicability of the Nice CHD classification, and explore whether this classification accurately reflects patient/disease characteristics and survival. All children with CHD from a contemporary cohort of consecutive pediatric PAH patients followed in three major referral centers (Denver, New York, the Netherlands) were characterized and classified on the basis of the latest proposed clinical classification for PAH-CHD (World Symposium on Pulmonary Hypertension, Nice, 2013)...
September 2016: Pulmonary Circulation
Atira H Kaplan, Yuxi Chen, Matthew N Bartels
No abstract text is available yet for this article.
September 2016: PM & R: the Journal of Injury, Function, and Rehabilitation
Cyd K Eaton, Jennifer L Lee, Kristin A Loiselle, Bonney Reed-Knight, Laura L Mee, Ana M Gutierrez-Colina, Ronald L Blount
The goal of this study was to compare pretransplant patient HRQOL, parent psychological functioning, and the impact of the patient's ongoing illness on the family between organ types (ie, kidney, liver, heart) and age-groups (ie, children, AYAs). The sample included 80 pediatric patients with end-stage organ disease who were evaluated for transplantation and their parents. Parents completed self- and proxy reports at patients' pretransplant evaluations. Results indicated that patients evaluated for heart transplants consistently had lower HRQOL and their parents had greater psychological distress compared to the kidney and liver groups...
September 27, 2016: Pediatric Transplantation
Roosevelt Bryant, Farhan Zafar, Chesney Castleberry, John L Jefferies, Angela Lorts, Clifford Chin, David L S Morales
The Berlin Heart EXCOR pediatric ventricular assist device is approved by the FDA for bridge to cardiac transplantation (BTT) in children. As the clinical outcomes of the EXCOR continue to be evaluated in the U.S., data on post transplant survival are needed. The UNOS database was used to identify patients < 18 years old undergoing orthotopic heart transplantation (OHT) from 6/2004 to 6/2014. Patients undergoing BTT with the EXCOR were identified. A matched cohort of (358) patients undergoing OHT without pre-transplant mechanical circulatory support (no-MCS) was also identified as controls...
September 20, 2016: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
Caroline M Sierra, Robert Tan, Jim Eguchi, Leonard Bailey, Richard E Chinnock
Pediatric heart transplant patients at our institution are immunosuppressed with a CNI and another immune-modulating agent without utilizing corticosteroids. Patients whose renal function worsened and who did not respond to CNI minimization had their CNI discontinued. The clinical history of 35 pediatric heart transplant patients with significant renal insufficiency whose CNI was discontinued was retrospectively analyzed. Data including serum creatinine and weight were collected before, at time of, and every 3-6 months after CNI discontinuation...
September 23, 2016: Pediatric Transplantation
Patrick M Sullivan, Agustin E Rubio, Troy A Johnston, Thomas K Jones
OBJECTIVES: To describe long-term risk of mortality, aortic insufficiency (AI), and re-intervention following balloon aortic valvuloplasty (BAV) in pediatric patients and to identify risk factors for re-intervention. BACKGROUND: Few studies report long-term outcomes following BAV in infants and children. METHODS: Kaplan-Meier estimates and proportional hazards regression were used in a retrospective study of 154 patients undergoing BAV from 1993 to 2013...
September 21, 2016: Catheterization and Cardiovascular Interventions
Ivan Knezevic
No abstract text is available yet for this article.
September 17, 2016: Transplant International: Official Journal of the European Society for Organ Transplantation
Justin Parizo, Ashish Sarraju, Joshua W Knowles
Both HeFH and HoFH require dietary and lifestyle modification. Pharmacotherapy of adult HeFH patients is largely driven by the American Heart Association (AHA) algorithm. A high-potency statin is started initially with a goal low-density lipoprotein cholesterol (LDL-C) reduction of >50 %. The LDL-C target is adjusted to <100 or <70 mg/dL in subjects with coronary artery disease (CAD) with ezetimibe being second line. If necessary, a third adjunctive therapy, such as a PSCK9 inhibitor (not yet approved in children) or bile acid-binding resin, can be added...
November 2016: Current Treatment Options in Cardiovascular Medicine
Hythem M Nawaytou, Putri Yubbu, Andrea E Montero, Deipanjan Nandi, Matthew J O'Connor, Robert E Shaddy, Anirban Banerjee
BACKGROUND: Left ventricular (LV) dysfunction after orthotopic heart transplantation (OHT) is multifactorial and can be an indicator of graft rejection or coronary artery vasculopathy. Analysis of rotational mechanics may help in the early diagnosis of ventricular dysfunction. Studies describing the left ventricular rotational strain in children after OHT are lacking. It is important to establish the baseline rotational mechanics in pediatric OHT to pursue further studies in this population...
September 2016: Circulation. Cardiovascular Imaging
Eyal Reinstein, Ana Gutierrez-Fernandez, Shay Tzur, Concetta Bormans, Shai Marcu, Einav Tayeb-Fligelman, Chana Vinkler, Annick Raas-Rothschild, Dana Irge, Meytal Landau, Mordechai Shohat, Xose S Puente, Doron M Behar, Carlos Lopez-Otın
In the vast majority of pediatric patients with dilated cardiomyopathy, the specific etiology is unknown. Studies on families with dilated cardiomyopathy have exemplified the role of genetic factors in cardiomyopathy etiology. In this study, we applied whole-exome sequencing to members of a non-consanguineous family affected by a previously unreported congenital dilated cardiomyopathy syndrome necessitating early-onset heart transplant. Exome analysis identified compound heterozygous variants in the FLNC gene...
September 7, 2016: European Journal of Human Genetics: EJHG
J Vockley, J Charrow, J Ganesh, M Eswara, G A Diaz, E McCracken, R Conway, G M Enns, J Starr, R Wang, J E Abdenur, J Sanchez-de-Toledo, D L Marsden
Long-chain fatty acid oxidation disorders (LC-FAOD) can cause cardiac hypertrophy and cardiomyopathy, often presenting in infancy, typically leading to death or heart transplant despite ongoing treatment. Previous data on triheptanoin treatment of cardiomyopathy in LC-FAOD suggested a clinical benefit on heart function during acute failure. An additional series of LC-FAOD patients with critical emergencies associated with cardiomyopathy was treated with triheptanoin under emergency treatment or compassionate use protocols...
August 27, 2016: Molecular Genetics and Metabolism
David B Nicholas, Bernadette Dodd, Simon Urschel, Amber Young, Lori J West
Given the arduous course of heart transplantation and follow-up care, recipients and their families face complex challenges and stressors warranting supportive interventions. This study explored the impact of a family camp as an intervention of education and social support for pediatric transplant recipients and their families. A total of 49 individuals participated in this evaluation, including eight children and nine youth with heart transplants, five siblings, 19 parents, and 13 health care providers. Participants ranked and described the 3-day family camp experience...
October 2016: Social Work in Health Care
Denise Bradley, Sebastian Moreira, Vishak Subramoney, Clifford Chin, Jane Ives, Ka Wang
BACKGROUND: Valganciclovir (VGCV) effectively prevents cytomegalovirus disease in adult and pediatric solid organ transplant (SOT) recipients. A dosing algorithm for VGCV for pediatric patients, based on body surface area and renal function, provides a personalized dose using age-appropriate formulations. The suitability of this dosing algorithm has not been assessed specifically in infants and neonates 4 months of age and younger receiving a SOT. METHODS: This multicenter prospective study evaluated the pharmacokinetics (PK) and safety of VGCV oral solution in 17 heart transplant recipients 4 months of age and younger who received two doses of VGCV on consecutive days using the pediatric dosing algorithm...
August 30, 2016: Pediatric Infectious Disease Journal
Jianmin Wang, Zuo Luan, Hua Jiang, Jianpei Fang, Maoquan Qin, Vincent Lee, Jing Chen
We investigated the efficacy of allogeneic hematopoietic stem cell transplantation (alloHSCT) in pediatric patients with mucopolysaccharidosis (MPS). A retrospective analysis of transplantation data from 34 cases of MPS from the China Children Transplant Group, treated between December 2004 and September 2015, was conducted. Among the 34 cases, 12 cases were type I, 12 were type II, 4 were type IV, 4 were type VI, and 2 were of an unknown type. The median age at transplantation was 3.75 years (range, 1 to 7 years); the median follow-up time was 14 months (range, 2 to 119 months)...
November 2016: Biology of Blood and Marrow Transplantation
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