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Pediatric, renal, advances

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https://www.readbyqxmd.com/read/28985199/renal-lineage-cells-as-a-source-for-renal-regeneration
#1
REVIEW
Oren Pleniceanu, Dorit Omer, Orit Harari-Steinberg, Benjamin Dekel
The mammalian kidney is a highly complex organ, composed of various cell types within a unique structural framework. Nonetheless, in recent years, giant leaps in our understanding of nephrogenesis and the origin of new cells in the adult kidney, have resulted in novel routes to regenerate damaged nephrons. While several strategies can be envisioned to achieve this aim, one common theme is the reliance on renal lineage cells, since extra-renal cells, such as bone-marrow derived cells, have been shown to be devoid of renal differentiation capacity...
October 6, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28914984/hypercalcemic-disorders-in-children
#2
REVIEW
Victoria J Stokes, Morten F Nielsen, Fadil M Hannan, Rajesh V Thakker
Hypercalcemia is defined as a serum calcium concentration that is greater than 2 standard deviations above the normal mean, which in children may vary with age and sex, reflecting changes in the normal physiology at each developmental stage. Hypercalcemic disorders in children may present with hypotonia, poor feeding, vomiting, constipation, abdominal pain, lethargy, polyuria, dehydration, failure to thrive and seizures. In severe cases renal failure, pancreatitis and reduced consciousness may also occur and older children and adolescents may present with psychiatric symptoms...
September 15, 2017: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/28877075/microsatellite-instability-a-predictive-biomarker-for-cancer-immunotherapy
#3
Liisa Chang, Minna Chang, Hanna M Chang, Fuju Chang
Immunotherapy has shown promising results in various types of cancers. Checkpoint inhibitor drugs developed for cancer immunotherapy have been approved by the US Food and Drug Administration (FDA) for patients with advanced melanoma, non-small cell lung cancer, renal cell carcinoma, bladder cancers, and refractory Hodgkin lymphoma. In the latest announcement, the FDA has granted accelerated approval to pembrolizumab for pediatric and adult patients with microsatellite instability-high (MSI-H) or mismatch repair-deficient solid tumors...
September 4, 2017: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/28844315/massively-parallel-sequencing-and-targeted-exomes-in-familial-kidney-disease-can-diagnose-underlying-genetic-disorders
#4
Andrew J Mallett, Hugh J McCarthy, Gladys Ho, Katherine Holman, Elizabeth Farnsworth, Chirag Patel, Jeffery T Fletcher, Amali Mallawaarachchi, Catherine Quinlan, Bruce Bennetts, Stephen I Alexander
Inherited kidney disease encompasses a broad range of disorders, with both multiple genes contributing to specific phenotypes and single gene defects having multiple clinical presentations. Advances in sequencing capacity may allow a genetic diagnosis for familial renal disease, by testing the increasing number of known causative genes. However, there has been limited translation of research findings of causative genes into clinical settings. Here, we report the results of a national accredited diagnostic genetic service for familial renal disease...
August 23, 2017: Kidney International
https://www.readbyqxmd.com/read/28840306/role-of-magnetic-resonance-urography-in-pediatric-renal-fusion-anomalies
#5
REVIEW
Sherwin S Chan, Aikaterini Ntoulia, Dmitry Khrichenko, Susan J Back, Gregory E Tasian, Jonathan R Dillman, Kassa Darge
Renal fusion is on a spectrum of congenital abnormalities that occur due to disruption of the migration process of the embryonic kidneys from the pelvis to the retroperitoneal renal fossae. Clinically, renal fusion anomalies are often found incidentally and associated with increased risk for complications, such as urinary tract obstruction, infection and urolithiasis. These anomalies are most commonly imaged using ultrasound for anatomical definition and less frequently using renal scintigraphy to quantify differential renal function and assess urinary tract drainage...
August 24, 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28802643/update-on-urinary-stones-in-children-current-and-future-concepts-in-surgical-treatment-and-shockwave-lithotripsy
#6
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
#7
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...
October 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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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...
November 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
#17
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
#18
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
#19
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
#20
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
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