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https://www.readbyqxmd.com/read/28087478/renal-involvement-in-lysinuric-protein-intolerance-contribution-of-pathology-to-assessment-of-heterogeneity-of-renal-lesions
#1
Emmanuel Estève, Pauline Krug, Aurélie Hummel, Jean-Baptiste Arnoux, Olivia Boyer, Anais Brassier, Pascale de Lonlay, Vincent Vuiblet, Stéphanie Gobin, Rémi Salomon, Christine Piètrement, Jean-Paul Bonnefont, Aude Servais, Louise Galmiche
Lysinuric protein intolerance (LPI) is a rare autosomal recessive disease caused by mutations in the SLC7A7 gene encoding the light subunit of a cationic amino acid transporter. Symptoms mimic primary urea cycle defects but dysimmune symptoms are also described. Renal involvement in LPI was first described in the 1980's. In 2007, it appeared that it could concern as much as 75% of LPI patients and could lead to end stage renal disease. The most common feature is proximal tubular dysfunction and nephrocalcinosis but glomerular lesions are also reported...
January 10, 2017: Human Pathology
https://www.readbyqxmd.com/read/28087009/improving-kidney-disease-in-obese-adolescents-a-surgical-approach
#2
Ian R Macumber
Obesity has been associated with chronic kidney disease in adult studies, but little data exist in pediatrics. Nehus et al. offer the first study to show that bariatric surgery in severely obese adolescents is associated with improved renal outcomes. Despite limitations, this study provided compelling evidence that aggressive weight loss intervention can improve renal outcomes in young, severely obese subjects. Further investigation is required to determine mechanisms by which this may occur.
February 2017: Kidney International
https://www.readbyqxmd.com/read/28081237/co-occurrence-of-asthma-and-nephrolithiasis-in-children
#3
Ganesh K Kartha, Ina Li, Suzy Comhair, Serpil C Erzurum, Manoj Monga
It has been proposed that epithelial dysfunction and inflammation may predispose patients to kidney stone formation. Asthma is another chronic condition related to epithelial dysfunction and inflammation. We hypothesized that pediatric patients with asthma would have an increased prevalence of nephrolithiasis. Furthermore, we investigated if asthma patients with nephrolithiasis have clinical characteristics and urine profiles that point to mechanisms of stone formation. We evaluated 865 pediatric patients who had a diagnosis of nephrolithiasis...
2017: PloS One
https://www.readbyqxmd.com/read/28078809/three-year-analysis-of-repeated-laboratory-tests-for-the-markers-total-cholesterol-ferritin-vitamin-d-vitamin-b12-and-folate-in-a-large-research-and-teaching-hospital-in-italy
#4
Monica Lanzoni, Marco Fornili, Irene Felicetta, Rita Maiavacca, Elia Biganzoli, Silvana Castaldi
RATIONALE, AIMS AND OBJECTIVES: The increasing number of diagnostic tests requests all over the world is a problem that can partially be explained by inappropriate testing. Impact on the total costs of health systems becomes relevant when tests are performed in a large amount. In this paper, retesting of total cholesterol, ferritin, vitamin B12 , vitamin D, and folate is assessed. METHODS: The Quality Unit of Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico di Milano (Fondazione) decided to perform a first assessment of the appropriate use of the laboratory tests cholesterol, ferritin, vitamin B12 , vitamin D, and folate focusing on the retesting interval for the same patient in the time period January 1, 2012, to December 31, 2014, in every care setting...
January 12, 2017: Journal of Evaluation in Clinical Practice
https://www.readbyqxmd.com/read/28063688/crystalloid-fluid-choice-and-clinical-outcomes-in-pediatric-sepsis-a-matched-retrospective-cohort-study
#5
Scott L Weiss, Luke Keele, Fran Balamuth, Neika Vendetti, Rachael Ross, Julie C Fitzgerald, Jeffrey S Gerber
OBJECTIVE: To test the hypothesis that resuscitation with balanced fluids (lactated Ringer [LR]) is associated with improved outcomes compared with normal saline (NS) in pediatric sepsis. STUDY DESIGN: We performed matched analyses using data from 12 529 patients <18 years of age with severe sepsis/septic shock at 382 US hospitals between 2000 and 2013 to compare outcomes with vs without LR as part of initial resuscitation. Patients receiving LR were matched 1:1 to patients receiving only NS (NS group), including separate matches for any (LR-any group) or exclusive (LR-only group) LR use...
January 4, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28060793/preterm-birth-and-ventilation-decrease-surface-density-of-glomerular-capillaries-in-lambs-regardless-of-postnatal-respiratory-support-mode
#6
Eveline Staub, Mar Janna Dahl, Calan Yost, Sydney Bowen, Toshio Aoki, Adam Blair, Zhengming Wang, Donald M Null, Bradley A Yoder, Kurt H Albertine
BACKGROUND: Prematurity is often complicated by respiratory support, including invasive mechanical ventilation (IMV) and non-invasive support (NIS). Compared to IMV, NIS reduces injury to the lung and brain. Prematurity may also disrupt glomerular architecture. Whether NIS differentially affects glomerular architecture is incompletely understood. We hypothesized that IMV would lead to greater disruption of glomerular architecture than NIS. METHODS: This is a secondary analysis of kidneys from moderately preterm lambs delivered at ~131d gestation (term ~150d) that had antenatal steroid exposure and surfactant treatment before resuscitation by IMV...
January 6, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28060690/-peritoneal-dialysis-in-the-first-two-years-of-life-experience-of-a-nephrology-and-renal-transplantation-pediatric-unit
#7
Sofia Deuchande, Tânia Mano, Cristina Novais, Rute Machado, Rosário Stone, Margarida Almeida
INTRODUCTION: Peritoneal dialysis is the dialytic method of choice in chronic end-stage renal disease in children. This study main purpose was to characterize the long-term survival of a pediatric population who began peritoneal dialysis within the first two years of life. MATERIAL AND METHODS: A descriptive and retrospective study was performed in a portuguese nephrology and renal transplantation pediatric unit, between January 1991 and August 2014. End-stage renal disease etiology, mortality, comorbidities and complications of peritoneal dialysis and end-stage renal disease, growth and psychomotor development were evaluated...
September 2016: Acta Médica Portuguesa
https://www.readbyqxmd.com/read/28048032/su-f-j-63-abdominal-diameter-changes-in-children-during-volumetric-modulated-arc-therapy-vmat-is-re-planning-needed
#8
F Guerreiro, G Janssens, E Seravalli, B Raaymakers
PURPOSE: To investigate the dosimetric impact of daily changes in patient's diameter, due to weight gain/loss and air in the bowel, based on CBCT information during radiotherapy treatment of pediatric abdominal tumors. METHODS: 10 pediatric patients with neuroblastoma (n=6) and Wilms' (n=4) tumors were included. Available CBCTs were affinely registered to the planning CT for daily set-up variations corrections. A density override approach assigning air-density to the random air pockets and water-density to the remaining anatomy was used to determine the CBCT and CT dose...
June 2016: Medical Physics
https://www.readbyqxmd.com/read/28047673/su-d-207a-06-pediatric-abdominal-organ-motion-quantified-via-a-novel-4d-mri-method
#9
J Uh, M J Krasin, J T Lucas, C Tinkle, T E Merchant, C Hua
PURPOSE: To develop a 4D MRI method for assessing respiration-induced abdominal organ motion in children receiving radiation therapy. METHODS: A 4D MRI using internal image-based respiratory surrogate has been developed and implemented on a clinical scanner (1.5T Siemens Avanto). Ten patients (younger group: N=6, 2-5 years, anesthetized; older group: N=4, 11-15 years) with neuroblastoma, Wilm's tumor rhabdomyosarcoma, or desmoplastic small round cell tumor received free breathing 4D MRI scans for treatment planning...
June 2016: Medical Physics
https://www.readbyqxmd.com/read/28042690/en-bloc-kidney-transplantation-from-infant-donors-younger-than-10%C3%A2-months-into-pediatric-recipients
#10
Hong-Yang Wang, Jun Li, Long-Shan Liu, Rong-Hai Deng, Qian Fu, Dicken Shiu-Chung Ko, Huan-Xi Zhang, Su-Xiong Deng, Chang-Xi Wang
Early graft loss and poor graft function limit the use of kidneys from infant donors. Six en bloc kidney transplantations were performed from infant donors younger than 10 months into pediatric recipients between November 2012 and September 2015 at our center. We retrospectively analyzed recipient and donor demographics, surgery procedures, complications, graft function and size, and patient and graft survival with a follow-up of 6-39 months (median 15.5 months). Donor age ranged from 1 to 10 months with weight ranging from 3...
January 1, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28041953/synthetic-approaches-towards-the-multi-target-drug-spironolactone-and-its-potent-analogues-derivatives
#11
REVIEW
Fayaz Ali Larik, Aamer Saeed, Danish Shahzad, Muhammad Faisal, Hesham El-Seedi, Haroon Mehfooz, Pervaiz Ali Channar
Spironolactone is a well-known multi-target drug and is specifically used for the treatment of high blood pressure and heart failure. It is also used for the treatment of edema, cirrhosis of the liver, malignant, pediatric, nephrosis and primary hyperaldosteronism. Spironolactone in association with thiazide diuretics treats hypertension and in association with furosemide treats bronchopulmonary dyspepsia. The therapeutic mechanism of action of spironolactone involves binding to intracellular mineralocorticoids receptors (MRs) in kidney epithelial cells, thereby inhibiting the binding of aldosterone...
December 29, 2016: Steroids
https://www.readbyqxmd.com/read/28039956/longitudinal-analysis-of-living-donor-kidney-transplant-rates-in-pediatric-candidates-in-the-united-states
#12
Douglas S Keith, Gayle Vranic, John Barcia, Victoria Norwood, Angie Nishio-Lucar
Among adults, living donor kidney transplant rates began declining in the United States after 2004 but whether a similar decline is occurring in the pediatric candidates has not been well studied. Share 35, a change in allocation rules implemented in October of 2005, may also have influenced rates of living donation. We sought to determine whether a decline in rates was occurring in pediatric candidates and whether the Share 35 program was the cause of the decline. All children listed for a kidney transplant or transplanted with a living donor without listing between 1996 and 2011 were identified in the United States (N=14 911) of which 6046 had received a living donor transplant during follow-up...
December 30, 2016: Pediatric Transplantation
https://www.readbyqxmd.com/read/28039534/hypomagnesemia-and-increased-risk-of-new-onset-diabetes-mellitus-after-transplantation-in-pediatric-renal-transplant-recipients
#13
Wesley Hayes, Sheila Boyle, Adrian Carroll, Detlef Bockenhauer, Stephen D Marks
BACKGROUND: New-onset diabetes after transplantation (NODAT) is a significant co-morbidity following kidney transplantation. Lower post-transplant serum magnesium levels have been found to be an independent risk factor for NODAT in adult kidney transplant recipients. METHODS: We undertook a retrospective analysis of risk factors for NODAT in pediatric renal transplant recipients at our institution with the aim of determining if hypomagnesemia confers a significant risk of developing NODAT in this patient population...
December 30, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28035470/the-clinical-and-laboratory-features-of-chinese-han-anti-factor-h-autoantibody-associated-hemolytic-uremic-syndrome
#14
Di Song, Xiao-Rong Liu, Zhi Chen, Hui-Jie Xiao, Jie Ding, Shu-Zhen Sun, Hong-Yan Liu, Wei-Yi Guo, Su-Xia Wang, Feng Yu, Ming-Hui Zhao
BACKGROUND: Anti-complement factor H (CFH) autoantibody-associated hemolytic uremic syndrome (HUS) is a severe sub-type of HUS. METHODS: We assessed the clinical and renal pathological features, circulating complement levels, and genetic background of Chinese pediatric patients with this sub-type of HUS. Thirty-three consecutive patients with acute kidney injury who tested positive for serum anti-CFH autoantibodies were enrolled in this study. RESULTS: All of the eight patients who underwent renal biopsies presented with changes typical of thrombotic microangiopathy, especially changes in chronic characteristics...
December 29, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28033104/micronuclei-and-other-nuclear-anomalies-in-buccal-epithelial-cells-of-children-with-chronic-kidney-disease
#15
Banu Aykanat, Gonca Cakmak Demircigil, Necla Buyan, Esra Baskin, Kaan Gulleroglu, Kibriya Fidan, Umut Selda Bayrakci, Aydin Dalgic, Hamdi Karakayali, Mehmet Haberal, Sema Burgaz
The objective of this study was to reveal the likely genomic instability in children with chronic kidney disease (CKD) using micronucleus (MN) assay on buccal epithelial cells (BEC). We investigated the frequencies of micronuclei and other nuclear anomalies, such as nuclear buds, binucleated cells, condensed chromatin, and karyorrhectic and pyknotic cells in BEC. Children with CKD were grouped as follows: children in the pre-dialysis (PreD) stage (N=17), children on regular haemodialysis (HD) (N=14), and children who have undergone transplantation (Tx) (N=17)...
December 1, 2016: Arhiv za Higijenu Rada i Toksikologiju
https://www.readbyqxmd.com/read/28030425/features-of-hepatitis-in-hepatitis-associated-aplastic-anemia-clinical-and-histopathologic-study
#16
Kalyani R Patel, Alison Bertuch, Ghadir S Sasa, Ryan W Himes, Hao Wu
OBJECTIVES: Hepatitis-associated aplastic anemia (HAA) is a rare variant of aplastic anemia in which patients present with severe pancytopenia after an episode of acute hepatitis. The marrow failure is often rapid, severe, and usually fatal if untreated. The preceding hepatitis is largely under-studied. METHODS: Retrospective study of the clinical and histopathologic features of hepatitis in pediatric patients who subsequently developed aplastic anemia and comparison with consecutive cases of acute liver failure and random cases of autoimmune hepatitis during the same time frame...
January 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28029746/respiratory-manifestations-in-38-patients-with-alstr%C3%A3-m-syndrome
#17
Caroline Boerwinkle, Jan D Marshall, Joy Bryant, William A Gahl, Kenneth N Olivier, Meral Gunay-Aygun
OBJECTIVES: Alström syndrome (AS) is a rare, multi-system condition characterized by retinal degeneration, sensorineural hearing loss, obesity, insulin-resistant diabetes, hypertriglyceridemia, cardiomyopathy, hepatorenal disease, and recurrent respiratory infections. It belongs to a group of genetic disorders known as primary ciliopathies, which includes autosomal dominant and recessive polycystic kidney diseases, as well as Joubert and Bardet-Biedl syndromes. Prior studies have suggested phenotypic overlap between primary ciliopathies affecting the non-motile, sensory cilia, and primary ciliary dyskinesia (PCD), a motile ciliopathy characterized by respiratory tract disease...
December 28, 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28027279/the-influence-of-the-route-of-antibiotic-administration-methicillin-susceptibility-vancomycin-duration-and-serum-trough-concentration-on-outcomes-of-pediatric-staphylococcus-aureus-bacteremic-osteoarticular-infection
#18
J Chase McNeil, Sheldon L Kaplan, Jesus G Vallejo
BACKGROUND: Bacteremia is often one factor used in deciding the need for prolonged intravenous antimicrobial therapy in osteoarticular infections (OAI). We examined treatment practices and outcomes of bacteremic S. aureus osteoarticular infections (BOAI) evaluated at Texas Children's Hospital (TCH). METHODS: Cases of acute hematogenous OAI in children with positive blood cultures for S. aureus at TCH from 2011-2014 were reviewed. Orthopedic complications included chronic osteomyelitis, growth arrest, pathologic fracture, avascular necrosis and chronic dislocation...
December 23, 2016: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28018150/frequency-of-and-risk-factors-for-acute-kidney-injury-associated-with-vancomycin-use-in-the-pediatric-intensive-care-unit
#19
Sarah Bonazza, Lauren C Bresee, Timothy Kraft, B Catherine Ross, Deonne Dersch-Mills
BACKGROUND: Published information evaluating frequency of and risk factors for vancomycin-induced acute kidney injury (AKI) in the pediatric intensive care unit (PICU) population is conflicting. OBJECTIVES: The primary objective was to describe the proportion of our PICU patients who developed AKI with intravenous (IV) vancomycin. The secondary objective was to describe the associated potential risk factors. METHODS: Pediatric patients (0-18 years) who received their first IV vancomycin dose in the PICU were evaluated in this retrospective chart review...
November 2016: Journal of Pediatric Pharmacology and Therapeutics: JPPT: the Official Journal of PPAG
https://www.readbyqxmd.com/read/28017613/developmental-regulation-of-mitochondrial-apoptosis-by-c-myc-governs-age-and-tissue-specific-sensitivity-to-cancer-therapeutics
#20
Kristopher A Sarosiek, Cameron Fraser, Nathiya Muthalagu, Patrick D Bhola, Weiting Chang, Samuel K McBrayer, Adam Cantlon, Sudeshna Fisch, Gail Golomb-Mello, Jeremy A Ryan, Jing Deng, Brian Jian, Chris Corbett, Marti Goldenberg, Joseph R Madsen, Ronglih Liao, Dominic Walsh, John Sedivy, Daniel J Murphy, Daniel Ruben Carrasco, Shenandoah Robinson, Javid Moslehi, Anthony Letai
It is not understood why healthy tissues can exhibit varying levels of sensitivity to the same toxic stimuli. Using BH3 profiling, we find that mitochondria of many adult somatic tissues, including brain, heart, and kidneys, are profoundly refractory to pro-apoptotic signaling, leading to cellular resistance to cytotoxic chemotherapies and ionizing radiation. In contrast, mitochondria from these tissues in young mice and humans are primed for apoptosis, predisposing them to undergo cell death in response to genotoxic damage...
January 9, 2017: Cancer Cell
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