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https://www.readbyqxmd.com/read/28802643/update-on-urinary-stones-in-children-current-and-future-concepts-in-surgical-treatment-and-shockwave-lithotripsy
#1
REVIEW
Mesrur Selcuk Silay, Jonathan S Ellison, Thomas Tailly, Paolo Caione
CONTEXT: Miniaturized instrumentation used for endoscopic treatment of urinary stone disease in children has been readily adopted in clinical practice. However, there is a need to optimize and individualize the surgical approach according to the patient's age, body habitus, and stone characteristics. Promising novel equipment and techniques will continue to advance the surgical care of these children. OBJECTIVE: To review the literature regarding surgical and shockwave lithotripsy (SWL) treatment of urinary stone disease in children and provide an overview on future treatment innovations...
August 9, 2017: European Urology Focus
https://www.readbyqxmd.com/read/28766875/species-differences-in-renal-development-and-associated-developmental-nephrotoxicity
#2
REVIEW
Kendall S Frazier
The developing kidney is sensitive to both morphological and functional disturbances during the gestational and postnatal phases of growth and differentiation. Exposure to drugs or chemicals during these critical windows of renal development can result in aplasia, dysplasia, polycystic kidney disease, hydronephrosis, or other features characteristic of nephrotoxicity, including tubule dilation, necrosis, or mineralization. Functional effects can occur without associated morphological abnormalities. Differences in the timing of nephrogenesis and morphologic renal development among species help to explain specific phenotypes of various gestational and postnatal teratogens and nephrotoxins...
August 2, 2017: Birth defects research
https://www.readbyqxmd.com/read/28724397/partial-uniparental-isodisomy-of-chromosome-16-unmasks-a-deleterious-biallelic-mutation-in-ift140-that-causes-mainzer-saldino-syndrome
#3
Benjamin M Helm, Jason R Willer, Azita Sadeghpour, Christelle Golzio, Eric Crouch, Samantha Schrier Vergano, Nicholas Katsanis, Erica E Davis
BACKGROUND: The ciliopathies represent an umbrella group of >50 clinical entities that share both clinical features and molecular etiology underscored by structural and functional defects of the primary cilium. Despite the advances in gene discovery, this group of entities continues to pose a diagnostic challenge, in part due to significant genetic and phenotypic heterogeneity and variability. We consulted a pediatric case from asymptomatic, non-consanguineous parents who presented as a suspected ciliopathy due to a constellation of retinal, renal, and skeletal findings...
July 19, 2017: Human Genomics
https://www.readbyqxmd.com/read/28689245/imaging-of-renal-medullary-carcinoma-in-children-and-young-adults-a-report-from-the-children-s-oncology-group
#4
Jesse K Sandberg, Elizabeth A Mullen, Mariana M Cajaiba, Ethan A Smith, Sabah Servaes, Elizabeth J Perlman, James I Geller, Peter F Ehrlich, Nicholas G Cost, Jeffrey S Dome, Conrad V Fernandez, Geetika Khanna
BACKGROUND: Renal medullary carcinoma is a rare renal malignancy of childhood. There are no large series describing the imaging appearance of renal medullary carcinoma in children. OBJECTIVE: To characterize the clinical and imaging features of pediatric renal medullary carcinoma at initial presentation. MATERIALS AND METHODS: We retrospectively analyzed images of 25 pediatric patients with renal medullary carcinoma enrolled in the Children's Oncology Group renal tumors classification, biology and banking study (AREN03B2) from March 2006 to August 2016...
July 8, 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28682025/-hypertension-in-chronic-kidney-disease
#5
Antonio De Pascalis, Alessandro Balducci
The progression of chronic kidney disease CKD is largely independent of the underlying kidney disorder once renal function has fallen below a critical level. Hypertension is an independent risk factor for disease progression in both adult and pediatric patients with kidney disorders. Optimal blood pressure control (130 /80mm Hg) represents a main goal of conservative therapy in patients with chronic kidney disease CKD but it is rarely achieved in clinical practice. Angiotensin-converting enzyme inhibitors and angiotensin receptor blockers are more effective than other drugs in slowing progression of proteinuric CKD...
March 2017: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/28679474/a-comparison-of-micro-perc-and-retrograde-intrarenal-surgery-results-in-pediatric-patients-with-renal-stones
#6
Haluk Sen, Ilker Seckiner, Omer Bayrak, Kazim Dogan, Sakip Erturhan
INTRODUCTION: With advancements in endoscopic surgery, open surgical techniques for urinary system stones have paved the way for the application of less invasive treatment modalities in patients with pediatric kidney stone disease. These treatment options include extracorporeal shock wave lithotripsy (ESWL), percutaneous nephrolithotomy (PCNL), and retrograde intrarenal surgery (RIRS). OBJECTIVE: We aimed to compare the efficacy and safety of RIRS and micro-PCNL techniques in the surgical treatment of kidney stones in pediatric patients...
June 19, 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28631039/graft-nephrectomy-in-children
#7
Benedict L Phillips, Chris J Callaghan
Kidney transplantation is recognised as the gold standard treatment of end-stage renal disease in most children, with excellent graft survival rates. When graft failure occurs, renal transplant recipients (RTRs) have the option of removal of the transplant (graft nephrectomy [GN]), or leaving the failed transplant in situ. The aims of this review are to discuss the indications for GN, surgical techniques, outcomes after GN (including risks of allosensitisation and the impact on subsequent transplants), and the possible role of routine GN in the asymptomatic RTR with a failed renal allograft...
June 19, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28621839/evaluation-of-hemodynamic-performance-of-a-combined-ecls-and-crrt-circuit-in-seven-positions-with-a-simulated-neonatal-patient
#8
Elizabeth Profeta, Kaitlyn Shank, Shigang Wang, Christian O'Connor, Allen R Kunselman, Karl Woitas, John L Myers, Akif Ündar
As it is common for patients treated with extracorporeal life support (ECLS) to subsequently require continuous renal replacement therapy (CRRT), and neonatal patients encounter limitations due to lack of access points, inclusion of CRRT in the ECLS circuit could provide advanced treatment for this population. The objective of this study was to evaluate an alternative neonatal ECLS circuit containing either a Maquet RotaFlow centrifugal pump or Maquet HL20 roller pump with one of seven configurations of CRRT using the Prismaflex 2000 System...
June 16, 2017: Artificial Organs
https://www.readbyqxmd.com/read/28620007/von-hippel-lindau-and-hereditary-pheochromocytoma-paraganglioma-syndromes-clinical-features-genetics-and-surveillance-recommendations-in-childhood
#9
REVIEW
Surya P Rednam, Ayelet Erez, Harriet Druker, Katherine A Janeway, Junne Kamihara, Wendy K Kohlmann, Katherine L Nathanson, Lisa J States, Gail E Tomlinson, Anita Villani, Stephan D Voss, Joshua D Schiffman, Jonathan D Wasserman
Von Hippel-Lindau disease (vHL) is a hereditary tumor predisposition syndrome that places affected individuals at risk for multiple tumors, which are predominantly benign and generally occur in the central nervous system or abdomen. Although the majority of tumors occur in adults, children and adolescents with the condition develop a significant proportion of vHL manifestations and are vulnerable to delayed tumor detection and their sequelae. Although multiple tumor screening paradigms are currently being utilized for patients with vHL, surveillance should be reassessed as the available relevant clinical information continues to expand...
June 15, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/28601936/acute-kidney-injury-emerging-pharmacotherapies-in-current-clinical-trials
#10
Stefanie Woolridge Benoit, Prasad Devarajan
Acute kidney injury (AKI) is a significant source of morbidity and mortality in pediatric patients, affecting more than one quarter of critically ill children. Despite significant need, there are no targeted therapies to reliably prevent or treat AKI. Recent advances in our understanding of renal injury and repair signaling pathways have enabled the development of several targeted pharmaceuticals. Here we review emerging pharmacotherapies for AKI that are currently in clinical trials. Categorized by their general mechanism of action, the therapies discussed include anti-inflammatory agents (recAP, AB103, ABT-719), antioxidants (iron chelators, heme arginate), vasodilators (levosimendan), apoptosis inhibitors (QPI-1002), and repair agents (THR-184, BB-3, mesenchymal stem cells)...
June 10, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28417541/clinical-efficacy-of-cabozantinib-in-two-pediatric-patients-with-recurrent-renal-cell-carcinoma
#11
Mary Frances Wedekind, Mark Ranalli, Nilay Shah
Advanced-stage renal cell carcinoma (RCC) carries a dismal prognosis for pediatric patients with few studied therapeutic options. Cabozantinib is a small molecule tyrosine kinase inhibitor against the oncoprotein MET. It is currently approved by the U.S. Food and Drug administration for second-line treatment of RCC in adults. There is no published data on its use in children with RCC. We report here two pediatric patients with recurrent metastatic RCC whose tumors expressed MET and were treated with cabozantinib...
April 18, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28396162/phase-i-study-of-clofarabine-and-2-gy-total-body-irradiation-as-a-nonmyeloablative-preparative-regimen-for-hematopoietic-stem-cell-transplantation-in-pediatric-patients-with-hematologic-malignancies-a-therapeutic-advances-in-childhood-leukemia-consortium-study
#12
Sandeep Soni, Hisham Abdel-Azim, Meghann McManus, Eneida Nemecek, Richard Sposto, Ann Woolfrey, Haydar Frangoul
Clofarabine is a purine nucleoside analog with immunosuppressive and antileukemic activity and its inclusion in reduced-intensity regimens could potentially improve outcomes. We performed a prospective phase I study of clofarabine combined with 2 Gy total body irradiation (TBI) as a nonmyeloablative preparative regimen for allogeneic stem cell transplantation in pediatric patients who were considered at high risk of mortality from standard myeloablative regimens. The main goal of the study was to delineate the maximum feasible dose (MFD) of clofarabine in combination with 2 Gy TBI...
April 7, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28326824/osteopontin-its-potential-role-in-cancer-of-children-and-young-adults
#13
Gabrielle Karpinsky, Aleksandra Fatyga, Malgorzata Anna Krawczyk, Madeleine Chamera, Natalia Sande, Dagmara Szmyd, Ewa Izycka-Swieszewska, Ewa Bien
OBJECTIVE: Osteopontin (OPN) is aglyco-phosphoprotein, involved in tissue remodeling, inflammation and boneresorption. In various adult neoplasms OPN was shown to correlate with cancer progression, invasiveness and metastasis. AIM: to define the role of OPN in malignancies of children and young adults. MATERIAL AND METHODS: a structured PubMed and Google Scholar literature analysis based on reports published in English between I'1995 and XII'2015...
March 14, 2017: Biomarkers in Medicine
https://www.readbyqxmd.com/read/28284824/-report-on-chronic-dialysis-in-france-in-2016
#14
Société Francophone de Néphrologie Dialyse Et Transplantation
The report on dialysis in France in 2016 from the French Speaking Society of Nephrology Dialysis and Transplantation (SFNDT) provides an exhaustive and documented inventory on dialysis in France. It underlines the organizations that are important in 2016 to maintain a high quality dialysis. Several measures are proposed to maintain and improve the care of dialysis in France: (1) The regulation of dialysis treatment in France must be maintained; (2) a burden of care indicator is proposed to ensure that patients requiring the most care are treated in the centers...
March 8, 2017: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/28260440/vascular-complications-after-renal-transplant-a-single-center-experience
#15
Ebru H Ayvazoglu Soy, Aydincan Akdur, Mahir Kirnap, Fatih Boyvat, Gokhan Moray, Mehmet Haberal
OBJECTIVES: Despite surgical and medical advances, vascular complications are still among the major concerns after renal transplant, with a reported incidence of 3% to 15%. We evaluated the incidence and management of our transplant team 's vascular complications over 40 years. MATERIALS AND METHODS: From November 1975 to the present, we have performed a total of 2594 renal transplant procedures. Of these, 1997 grafts (76%) were obtained from living donors, and 597 grafts (24%) were obtained from deceased donors...
February 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28258497/treatment-of-pediatric-onset-lupus-nephritis-a-proposal-of-optimal-therapy
#16
REVIEW
Hiroshi Tanaka, Kensuke Joh, Tadaatsu Imaizumi
Lupus nephritis (LN) is one of the major clinical manifestations of systemic lupus erythematosus (SLE) which occurs frequently in the early stages of pediatric-onset cases. Since SLE is a chronic disease associated with frequent disease flares and effective and safe maintenance therapy is required for achieving a favorable outcome, optimal treatment for LN in pubertal patients is a great challenge that remains to be overcome. Although its etiology remains unclear, it has been reported that the innate and adaptive immune systems have been reported to play an important role in the pathogenesis of SLE...
March 3, 2017: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/28254584/outcomes-and-risk-factors-for-graft-loss-lessons-learned-from-1-056-pediatric-kidney-transplants-at-the-university-of-minnesota
#17
Srinath Chinnakotla, Priya Verghese, Blanche Chavers, Michelle N Rheault, Varvara Kirchner, Ty Dunn, Clifford Kashtan, Thomas Nevins, Michael Mauer, Timothy Pruett
BACKGROUND: Advances in immunosuppression, surgical techniques, and management of infections in children receiving kidney transplants have affected outcomes. STUDY DESIGN: We analyzed a prospectively maintained database of pediatric kidney transplantations. RESULTS: From June 1963 through October 2016, we performed 1,056 pediatric kidney transplantations. Of these, 129 were in children less than 2 years old. The most common indications for transplant were congenital anomalies (dysplastic kidneys), obstructive uropathy, and congenital nephrotic syndrome...
April 2017: Journal of the American College of Surgeons
https://www.readbyqxmd.com/read/28197030/superperc-a-new-technique-in-minimally-invasive-percutaneous-nephrolithotomy
#18
Kaushik Shah, Madhu Sudan Agrawal, Dilip Kumar Mishra
INTRODUCTION: Percutaneous nephrolithotomy (PCNL) has undergone significant changes in recent years in the quest for improving efficacy and reducing morbidity. Newer minimally-invasive modalities of PCNL such as mini-PCNL, ultra-mini PCNL, and micro-PCNL have evolved with advancement in optics and technology. However, with these newer advancements, migration of small fragments produced with laser lithotripsy remains a concern, which may result in incomplete stone clearance. We describe a new technique of PCNL termed "Superperc", that utilizes suction to remove all the fragments and maintain one-way flow...
January 2017: Indian Journal of Urology: IJU: Journal of the Urological Society of India
https://www.readbyqxmd.com/read/28126219/imaging-of-the-pediatric-urinary-system
#19
REVIEW
Ellen M Chung, Karl A Soderlund, Kimberly E Fagen
Recent advances in pediatric urinary tract imaging include development of alternative imaging methods without use of ionizing radiation; evolving understanding of the relationship of urinary tract infection, vesicoureteral reflux, and renal scarring, including the important role of dysfunctional voiding; development of a consensus nomenclature and risk-based classification for fetal and antenatal urinary tract dilation; advances in the understanding of sporadic and inherited renal cystic disease; and a proposed modification of the Bosniak criteria for distinguishing complex renal cysts from cystic renal tumors in children...
March 2017: Radiologic Clinics of North America
https://www.readbyqxmd.com/read/28094348/weight-of-abcb1-and-por-genes-on-oral-tacrolimus-exposure-in-cyp3a5-nonexpressor-pediatric-patients-with-stable-kidney-transplant
#20
G N Almeida-Paulo, I Dapía García, R Lubomirov, A M Borobia, N L Alonso-Sánchez, L Espinosa, A J Carcas-Sansuán
Tacrolimus (TAC) is highly effective for the prevention of acute organ rejection. However, its clinical use may be challenging due to its large interindividual pharmacokinetic variability, which can be partially explained by genetic variations in TAC-metabolizing enzymes and transporters. The aim of this study was to evaluate the influence of genetic and clinical factors on TAC pharmacokinetic variability in 21 stable pediatric renal transplant patients. This study was nested in a previous Prograf to Advagraf conversion clinical trial...
January 17, 2017: Pharmacogenomics Journal
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