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Pediatric, renal, advances

Priya S Verghese
Successful renal transplantation is the optimal treatment for chronic kidney failure, but this was not always so for children. Beginning with the first kidney transplants in the 1950s, children experienced poorer patient and graft survival rates than adult patients. But over the last 6 decades, an improved understanding of the immune system which has steered pediatric multi-center clinical / pharmacokinetic and mechanistic studies that have sculpted our immunosuppression with markedly better patient and graft survivals...
October 12, 2016: Pediatric Research
Priya Verghese, Kristen Gillingham, Arthur Matas, Srinath Chinnakotla, Blanche Chavers
The association of blood transfusions with GS after pediatric KTx is unclear. We retrospectively analyzed blood transfusions post-KTx and subsequent outcomes. Between 1984 and 2013, 482 children (<18 years of age) underwent KTx at our center. Recipient demographics, outcomes and transfusion data were collected. Cox regression with post-KTx blood transfusion as a time-dependent covariate was performed to model the impact of blood transfusion on outcomes. Of the 208 (44%) that were transfused, 39% had transfusion <1 month post-KTx; 48% >12 months...
October 6, 2016: Pediatric Transplantation
Lyndsay A Harshman, Diana Zepeda-Orozco
Chronic kidney disease (CKD) in children is an irreversible process that, in some cases, may lead to end-stage renal disease. The majority of children with CKD have a congenital disorder of the kidney or urological tract arising from birth. There is strong evidence for both a genetic and epigenetic component to progression of CKD. Utilization of gene-mapping strategies, ranging from genome-wide association studies to single-nucleotide polymorphism analysis, serves to identify potential genetic variants that may lend to disease variation...
March 2016: Journal of Pediatric Genetics
Laurel K Willig
Inherited renal disease is a leading cause of morbidity and mortality in pediatric nephrology. High throughput advancements in genomics have led to greater understanding of the biologic underpinnings of these diseases. However, the underlying genetic changes explain only part of the molecular biology that contributes to disease manifestation and progression. Other omics technologies will provide a more complete picture of these cellular processes. This review discusses these omics technologies in the context of pediatric renal disease...
March 2016: Journal of Pediatric Genetics
Nicolas Waespe, Machiel Van Den Akker, Robert J Klaassen, Lani Lieberman, Meredith S Irwin, Salah S Ali, Mohamed Abdelhaleem, Bozana Zlateska, Mira Liebman, Michaela Cada, Tal Schechter, Yigal Dror
Advanced myelodysplastic syndrome harbors a high risk of progression to acute myeloid leukemia and poor prognosis. In children, there is no established treatment to prevent or delay progression to leukemia prior to hematopoietic stem cell transplantation. Azacitidine is a hypomethylating agent, which was shown to slow progression to leukemia in adults with myelodysplastic syndrome. There is little data on the efficacy of azacitidine in children. We reviewed 22 pediatric patients with advanced myelodysplastic syndrome from a single center, diagnosed between January 2000 and December 2015...
August 18, 2016: Haematologica
Nowell M Fine, Sudhir S Kushwaha
The mammalian target of rapamycin (mTOR) inhibitors sirolimus and everolimus are increasingly utilized in cardiothoracic transplantation. Several recent clinical trials have demonstrated their efficacy in combination with reduced cyclosporine dosing in de novo heart transplant recipients, in particular with everolimus. A number of other studies have demonstrated their efficacy for improving renal function and reducing calcineurin inhibitor use, attenuating cardiac allograft vasculopathy progression and reducing cytomegalovirus infections in maintenance heart transplant populations...
August 5, 2016: Transplantation
Jennifer G Jetton, Ronnie Guillet, David J Askenazi, Lynn Dill, Judd Jacobs, Alison L Kent, David T Selewski, Carolyn L Abitbol, Fredrick J Kaskel, Maroun J Mhanna, Namasivayam Ambalavanan, Jennifer R Charlton
INTRODUCTION: Acute kidney injury (AKI) affects ~30% of hospitalized neonates. Critical to advancing our understanding of neonatal AKI is collaborative research among neonatologists and nephrologists. The Neonatal Kidney Collaborative (NKC) is an international, multidisciplinary group dedicated to investigating neonatal AKI. The AWAKEN study (Assessment of Worldwide Acute Kidney injury Epidemiology in Neonates) was designed to describe the epidemiology of neonatal AKI, validate the definition of neonatal AKI, identify primary risk factors for neonatal AKI, and investigate the contribution of fluid management to AKI events and short-term outcomes...
2016: Frontiers in Pediatrics
Jonathan C Routh, Earl Y Cheng, J Christopher Austin, Michelle A Baum, Patricio C Gargollo, Richard W Grady, Adrienne R Herron, Steven S Kim, Shelly J King, Chester J Koh, Pangaja Paramsothy, Lisa Raman, Michael S Schechter, Kathryn A Smith, Stacy T Tanaka, Judy K Thibadeau, William O Walker, M Chad Wallis, John S Wiener, David B Joseph
PURPOSE: Care of children with spina bifida has significantly advanced in the last half century, resulting in gains in longevity and quality of life for affected children and caregivers. Bladder dysfunction is the norm in patients with spina bifida and may result in infection, renal scarring and chronic kidney disease. However, the optimal urological management for spina bifida related bladder dysfunction is unknown. MATERIALS AND METHODS: In 2012 the Centers for Disease Control and Prevention convened a working group composed of pediatric urologists, nephrologists, epidemiologists, methodologists, community advocates and Centers for Disease Control and Prevention personnel to develop a protocol to optimize urological care of children with spina bifida from the newborn period through age 5 years...
July 27, 2016: Journal of Urology
Ilan I Maizlin, Michelle C Shroyer, Lena Perger, Mike K Chen, Elizabeth A Beierle, Colin A Martin, Scott A Anderson, Vincent E Mortellaro, David A Rogers, Robert T Russell
BACKGROUND: Recent advances in renal replacement therapy (RRT) have brought about a proliferation of dialysis in neonates (<30 d). This study aimed to assess morbidity and mortality after RRT initiation in this population. METHODS: Retrospective chart review of all patients between 2006 and 2014 requiring RRT initiated in the first 30 d of life was performed. RESULTS: A total of 49 patients were identified, of which 39 were boys and 10 were girls...
July 2016: Journal of Surgical Research
Lori A Zimmerman, Constantine G Saites, Sigrid Bairdain, Chueh Lien, David Zurakowski, Robert C Shamberger, Bradley C Linden, Athos Bousvaros
OBJECTIVES: Infliximab (IFX) has become a mainstay of therapy for children with Crohn disease (CD). Despite medical advances, many children with CD, however, still require operative interventions. The risk of complications following resection in children treated with IFX remains largely unknown. We compare surgical outcomes stratified by IFX therapy in a cohort of children with CD who require bowel resection. METHODS: We reviewed the postoperative complications in 123 children with CD who underwent bowel resection with primary anastomosis at our institution between 1977 and 2011...
September 2016: Journal of Pediatric Gastroenterology and Nutrition
Ja Hye Kim, Beom Hee Lee, Ja Hyang Cho, Eungu Kang, Jin-Ho Choi, Gu-Hwan Kim, Han-Wook Yoo
Fabry disease is a progressive lysosomal storage disease caused by alpha-galactosidase A deficiency. This condition is characterized by progressive accumulation of glycosphingolipids with functional impairment in various organs, including the kidney, heart and cerebrovascular system. Enzyme replacement therapy (ERT) is essential because it attenuates the disease progression. The present study investigated the long-term efficacy of ERT in 19 Korean Fabry patients (11 adult males, 4 symptomatic female carriers and 4 pediatric males) who had received ERT for 8...
June 23, 2016: Journal of Human Genetics
Hua Pan, Aram Gazarian, Jean-Michel Dubernard, Alexandre Belot, Marie-Cécile Michallet, Mauricette Michallet
Although several tolerance induction protocols have been successfully implemented in adult renal transplantation, no tolerance induction approach has, as yet, been defined for solid organ transplantations in young infants. Pediatric transplant recipients have a pressing demand for the elaboration of tolerance induction regimens. Indeed, since they display a longer survival time, they are exposed to a higher level of risks linked to long-term immunosuppression (IS) and to chronic rejection. Interestingly, central tolerance induction may be of great interest in newborns, because of their immunological immaturity and the important role of the thymus at this early stage in life...
2016: Frontiers in Immunology
Rebecca C Graves, Richard N Fine
Retransplantation accounts for approximately 15 % of the annual transplants performed in the USA, and in the recent International Collaborative Transplant Study report on pediatric patients 15.2 % of the 9209 patients included in the report were retransplant recipients. Although the significant advances in clinical management and newer immunosuppressive agents have had a significant impact on improving short-term allograft function, it is apparent that long-term allograft function remains suboptimal. Therefore, it is likely that the majority of pediatric renal allograft recipients will require one or more retransplants during their lifetime...
April 5, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
David S Cooper, Rajit K Basu, Jack F Price, Stuart L Goldstein, Catherine D Krawczeski
The field of cardiac intensive care continues to advance in tandem with congenital heart surgery. The focus of intensive care unit care has now shifted to that of morbidity reduction and eventual elimination. Acute kidney injury (AKI) after cardiac surgery is associated with adverse outcomes, including prolonged intensive care and hospital stays, diminished quality of life, and increased long-term mortality. Acute kidney injury occurs frequently, complicating the care of both postoperative patients and those with heart failure...
March 2016: World Journal for Pediatric & Congenital Heart Surgery
Joshua Y Menendez, Bradford A Woodworth, James M Johnston
Hyperprolactinemia is a rare entity in the pediatric population. The most common causes of hyperprolactinemia include drug use, hypothyroidism and renal insufficiency, though rarely a pituitary or sellar mass is discovered. We present an immunocompetent pediatric patient who presented with gynecomastia and was found to have hyperprolactinemia. Imaging showed a sphenoid mass and referral was made for a pituitary tumor. The mass was not a pituitary tumor and he was formally diagnosed with allergic fungal sinusitis and treated surgically...
2016: Turkish Neurosurgery
Maristella Santi, Gregorio P Milani, Giacomo D Simonetti, Emilio F Fossali, Mario G Bianchetti, Sebastiano A G Lava
BACKGROUND: The metabolism of sodium, potassium, and chloride and the acid-base balance are sometimes altered in cystic fibrosis. Textbooks and reviews only marginally address the homeostasis of magnesium in cystic fibrosis. METHODS: We performed a search of the Medical Subject Headings terms (cystic fibrosis OR mucoviscidosis) AND (magnesium OR hypomagnes[a]emia) in the US National Library of Medicine and Excerpta Medica databases. RESULTS: We identified 25 reports dealing with magnesium and cystic fibrosis...
February 2016: Pediatric Pulmonology
Jin Wook Baek, Kyung-Il Jo, Jeong Jin Park, Pyoung Jeon, Keon Ha Kim
PURPOSE: Until recently, extracranial involvement of moyamoya disease (MMD) had not been fully elucidated. The purpose of this study was to determine the prevalence and clinical implications of renal artery stenosis in pediatric MMD patients. MATERIALS & METHODS: This retrospective study included 101 pediatric (<18 years old) MMD patients who visited our hospital from July 2008 to May 2013. All patients had legible abdominal or renal angiography. Baseline characteristics, including hypertension (HT) and angiographic findings, were retrospectively evaluated...
January 2016: European Journal of Paediatric Neurology: EJPN
Deipanjan Nandi, Kimberly Y Lin, Matthew J O'Connor, Okan U Elci, Jeffrey J Kim, Jamie A Decker, Jack F Price, Farhan Zafar, David L S Morales, Susan W Denfield, William J Dreyer, John L Jefferies, Joseph W Rossano
Scarce data exist regarding costs of pediatric heart failure-related hospitalizations (HFRH) or how costs have changed over time. Pediatric HFRH costs, due to advances in management, will have increased significantly over time. A retrospective analysis of Healthcare Cost and Utilization Project Kids' Inpatient Database was performed on all pediatric HFRH. Inflation-adjusted charges are used as a proxy for cost. There were a total of 33,189 HFRH captured from 2000 to 2009. Median charges per HFRH rose from $35,079 in 2000 to $72,087 in 2009 (p < 0...
March 2016: Pediatric Cardiology
Jérôme Harambat, Pepe Mfutu Ekulu
The increasing attention paid to chronic kidney disease (CKD) as a major cause of mortality and disability, as well as the advances in management of CKD in children, have created a growing demand for pediatric renal replacement therapy (RRT) worldwide. A study by Koch Nogueira and colleagues of children on the transplant waiting list showed large disparities in access to pediatric kidney transplantation between regions in Brazil. This finding raises a wider question about inequalities in access to CKD care in children...
March 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Ronak A Gor, Christopher J Long, Aseem R Shukla, Andrew J Kirsch, Marcos Perez-Brayfield, Arun K Srinivasan
OBJECTIVE: To review peri-procedural outcomes from a large, multi-institutional series of pediatric urology patients treated with laparaendoscopic single-site surgery (LESS) for major extirpative and reconstructive procedures. MATERIALS AND METHODS: Consecutive LESS cases between January 2011 and May 2014 from three free-standing pediatric referral centers were reviewed. Data include age, sex, operative time, blood loss, length of stay, and complications according to the modified Clavien-Dindo classification...
February 2016: Urology
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