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Pediatric Nephrology: Journal of the International Pediatric Nephrology Association

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https://www.readbyqxmd.com/read/29931555/uric-acid-and-progression-of-chronic-kidney-disease
#1
Donald J Weaver
The association between serum uric acid levels and human disease has garnered intense interest over the last decade including chronic kidney disease. Animal studies have provided evidence for a potential mechanistic role of uric acid in promoting progression of chronic kidney disease. Epidemiologic studies have also suggested an association between elevated serum uric acid levels and worsening renal function in the general population as well as in patients with chronic kidney disease. However, there is currently insufficient evidence to recommend the use of uric acid-lowering therapy to delay progression of chronic kidney disease in this patient population...
June 21, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29872964/the-interplay-between-bone-and-vessels-in-pediatric-ckd-lessons-from-a-single-center-study
#2
Evgenia Preka, Bruno Ranchin, Anke Doyon, Melody Vierge, Tiphanie Ginhoux, Behrouz Kassai, Justine Bacchetta
OBJECTIVE: Mineral and bone disorders associated to chronic kidney disease (CKD-MBD) are a daily challenge for pediatric nephrologists, with a significant risk of long-term bone and vascular comorbidities. METHODS: This single-center study is a prospective transversal evaluation of pediatric CKD patients of our center, part of the European 4C study. In addition to clinical and biochemical data, vascular and bone evaluation was performed: 24-h blood pressure assessment, carotid intima-media thickness (cIMT), pulse wave velocity (PWV), and high-resolution peripheral quantitative computed tomography (HR-pQCT) at the ultra-distal tibia...
June 5, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29872963/waist-to-height-ratio-body-mass-index-and-cardiovascular-risk-profile-in-children-with-chronic-kidney-disease
#3
Kristen Sgambat, Jennifer Roem, Mark Mitsnefes, Anthony A Portale, Susan Furth, Bradley Warady, Asha Moudgil
BACKGROUND: Cardiovascular (CV) risk is high in children with chronic kidney disease (CKD), and further compounded in those who are overweight. Children with CKD have a unique body habitus not accurately assessed by body mass index (BMI). Waist-to-height ratio (WHr), a better predictor of CV risk in populations with short stature, has not been investigated in children with CKD. METHODS: Analysis of 1723 visits of 593 participants enrolled in the Chronic Kidney Disease in Children (CKiD) study was conducted...
June 5, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29869723/incidence-risk-factors-and-outcome-of-neonatal-acute-kidney-injury-a-prospective-cohort-study
#4
Mohamed A Shalaby, Zinab A Sawan, Esraa Nawawi, Saad Alsaedi, Heidi Al-Wassia, Jameela A Kari
BACKGROUND: Acute kidney injury (AKI) is common in neonates admitted to the neonatal intensive care unit (NICU). AKI is associated with increased morbidity and mortality and a greater long-term risk of chronic kidney disease. OBJECTIVES: To study the incidence and outcome of neonatal AKI in a single Saudi Arabian center, level 2\3 NICU. METHODS: This single-center prospective cohort study included all infants who received level II or III NICU care during 2015 (January to December)...
June 5, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29869118/genetic-diagnosis-of-steroid-resistant-nephrotic-syndrome-in-a-longitudinal-collection-of-czech-and-slovak-patients-a-high-proportion-of-causative-variants-in-nup93
#5
Martin Bezdíčka, Šárka Štolbová, Tomáš Seeman, Ondřej Cinek, Michal Malina, Naděžda Šimánková, Štěpánka Průhová, Jakub Zieg
BACKGROUND: Steroid-resistant nephrotic syndrome (SRNS) has a heterogeneous spectrum of monogenic causes that substantially differ among populations. The aim of this study was to analyse the genetic aetiology of SRNS in Czech and Slovak paediatric patients. METHODS: We analysed clinical data from 74 patients (38 boys) with congenital (15%), infant (14%), and childhood-onset (71%) SRNS collected from the Czech Republic and Slovakia from 2000 to 2017 (inclusive). The DNA samples were first analysed by Sanger sequencing (genes NPHS2, NPHS1, and WT1) and then by next generation sequencing (NGS) using a targeted panel of 48 genes previously associated with SRNS...
June 4, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29869117/validating-the-use-of-bioimpedance-spectroscopy-for-assessment-of-fluid-status-in-children
#6
Indranil Dasgupta, David Keane, Elizabeth Lindley, Ihab Shaheen, Kay Tyerman, Franz Schaefer, Elke Wühl, Manfred J Müller, Anja Bosy-Westphal, Hans Fors, Jovanna Dahlgren, Paul Chamney, Peter Wabel, Ulrich Moissl
BACKGROUND: Bioimpedance spectroscopy (BIS) with a whole-body model to distinguish excess fluid from major body tissue hydration can provide objective assessment of fluid status. BIS is integrated into the Body Composition Monitor (BCM) and is validated in adults, but not children. This study aimed to (1) assess agreement between BCM-measured total body water (TBW) and a gold standard technique in healthy children, (2) compare TBW_BCM with TBW from Urea Kinetic Modelling (UKM) in haemodialysis children and (3) investigate systematic deviation from zero in measured excess fluid in healthy children across paediatric age range...
June 4, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29869116/treating-the-idiopathic-nephrotic-syndrome-are-steroids-the-answer
#7
Georges Deschênes, Claire Dossier, Julien Hogan
The use of steroids in idiopathic nephrotic syndrome is the major discovery of the twentieth century in the field of pediatric nephrology. At onset of the twenty-first century, steroids remain the first line of treatment at first flare. All the protocols to treat the first flare are similar by a common sequence including a first phase of daily prednisolone/prednisone at a dose of 60 mg/m2 /day for at least 4 weeks followed by an alternate-day regimen for several weeks. It appears that a cumulated dose of 2240 mg/m2 given in 8 weeks at the first flare without tapering sequence is not inferior to increased dose and duration in terms of prevalence of frequent relapsers and the subsequent cumulated dose of steroids at 24 months of follow-up...
June 4, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29869115/imaging-studies-in-pediatric-fibromuscular-dysplasia-fmd-a-single-center-experience
#8
Robert Louis, Daniella Levy-Erez, Anne Marie Cahill, Kevin E Meyers
BACKGROUND: Fibromuscular dysplasia (FMD) is a non-inflammatory vascular disease that in children unlike in adults shows no sex predilection. FMD is often underdiagnosed, and its pathophysiology is unclear. Delayed diagnosis may lead to refractory hypertension and decreases the chance of successful treatment. Doppler ultrasound (US), magnetic resonance angiography (MRA), computed tomography angiography (CTA), and catheter-based angiography (angiography) are currently used to help make a clinicoradiological diagnosis of FMD...
June 4, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29860579/mitochondrial-mechanisms-and-therapeutics-in-ischaemia-reperfusion-injury
#9
REVIEW
Jack L Martin, Anja V Gruszczyk, Timothy E Beach, Michael P Murphy, Kourosh Saeb-Parsy
Acute kidney injury (AKI) remains a major problem in critically unwell children and young adults. Ischaemia reperfusion (IR) injury is a major contributor to the development of AKI in a significant proportion of these cases and mitochondria are increasingly recognised as being central to this process through generation of a burst of reactive oxygen species early in reperfusion. Mitochondria have additionally been shown to have key roles in downstream processes including activation of the immune response, immunomodulation, and apoptosis and necrosis...
June 2, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29808264/neonatal-hypertension-cases-causes-and-clinical-approach
#10
Michelle C Starr, Joseph T Flynn
Neonatal hypertension is increasingly recognized as dramatic improvements in neonatal intensive care, advancements in our understanding of neonatal physiology, and implementation of new therapies have led to improved survival of premature infants. A variety of factors appear to be important in determining blood pressure in neonates, including gestational age, birth weight, and postmenstrual age. Normative data on neonatal blood pressure values remain limited. The cause of hypertension in an affected neonate is often identified with careful diagnostic evaluation, with the most common causes being umbilical catheter-associated thrombosis, renal parenchymal disease, and chronic lung disease...
May 28, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29808263/renal-allograft-survival-rates-in-kidneys-initially-declined-for-paediatric-transplantation
#11
Matko Marlais, Laura Pankhurst, Kate Martin, Lisa Mumford, E Jane Tizard, Stephen D Marks
BACKGROUND: The outcome of organs which have been declined for paediatric recipients is not known. This study aimed to determine the outcome of kidneys initially declined for paediatric recipients and establish renal allograft survival in kidneys that were eventually transplanted. METHODS: Data were obtained from the UK Transplant Registry for all donation after brain death (DBD) kidneys offered and declined to paediatric recipients (< 18 years) in the UK from 2009 to 2014...
May 28, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29789935/potential-use-of-stem-cells-as-a-therapy-for-cystinosis
#12
REVIEW
Celine J Rocca, Stephanie Cherqui
Cystinosis is an autosomal recessive metabolic disease that belongs to the family of lysosomal storage disorders (LSDs). Initial symptoms of cystinosis correspond to the renal Fanconi syndrome. Patients then develop chronic kidney disease and multi-organ failure due to accumulation of cystine in all tissue compartments. LSDs are commonly characterized by a defective activity of lysosomal enzymes. Hematopoietic stem and progenitor cell (HSPC) transplantation is a treatment option for several LSDs based on the premise that their progeny will integrate in the affected tissues and secrete the functional enzyme, which will be recaptured by the surrounding deficient cells and restore physiological activity...
May 22, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29789934/induction-prednisone-dosing-for-childhood-nephrotic-syndrome-how-low-should-we-go
#13
Matthew Sibley, Abishek Roshan, Alanoud Alshami, Marisa Catapang, Jasper J Jöbsis, Trevor Kwok, Nonnie Polderman, Jennifer Sibley, Douglas G Matsell, Cherry Mammen
BACKGROUND: Historically, children with nephrotic syndrome (NS) across British Columbia (BC), Canada have been cared for without formal standardization of induction prednisone dosing. We hypothesized that local historical practice variation in induction dosing was wide and that children treated with lower doses had worse relapsing outcomes. METHODS: This retrospective cohort study included 92 NS patients from BC Children's Hospital (1990-2010). We excluded secondary causes of NS, age < 1 year at diagnosis, steroid resistance, and incomplete induction due to early relapse...
May 22, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29774464/eculizumab-in-stec-hus-need-for-a-proper-randomized-controlled-trial
#14
Sebastian Loos, Jun Oh, Markus J Kemper
Hemolytic uremic syndrome caused by Shiga toxin-producing E. coli (STEC-HUS) is often associated with a severe morbidity including neurological involvement and a mortality of 1-5%. Although STEC-HUS is often self-limited, improvement of treatment strategies is needed for cases with complications and, among others, plasma exchange/plasmapheresis and use of antibiotics have been advocated. With the availability of the complement blocker eculizumab, now a standard treatment of atypical HUS, several series have addressed its use in STEC-HUS, with variable response; randomized controlled trials are lacking...
May 17, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29774463/early-cardiovascular-manifestations-in-children-and-adolescents-with-autosomal-dominant-polycystic-kidney-disease-a-single-center-study
#15
Vasiliki Karava, Cherine Benzouid, Julien Hogan, Claire Dossier, André Pierre Denjean, Georges Deschênes
BACKGROUND: This study aims to describe the cardiovascular manifestations in children with autosomal dominant polycystic kidney disease (ADPKD) and detect their relation with kidney disease and type of gene mutation. METHODS: Twenty-one patients (7 to 19 years old) were included. Cardiovascular evaluation involved blood pressure (BP), indexed left ventricular mass (LVMI), pulse wave velocity (PWV), and carotid intima media thickness (cIMT) measurement. Patients were classified according to percentile reference values of these parameters in healthy children...
May 17, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29774462/combining-gfr-estimates-from-cystatin-c-and-creatinine-what-is-the-optimal-mix
#16
Emil den Bakker, Reinoud Gemke, Joanna A E van Wijk, Isabelle Hubeek, Birgit Stoffel-Wagner, Arend Bökenkamp
BACKGROUND: Combining estimated glomerular filtration rate (eGFR) equations based on creatinine and cystatin C has been shown to improve the accuracy of GFR estimation. This study aims to optimize this strategy for height-independent GFR estimation in children. METHODS: Retrospective study of 408 inulin clearance tests with simultaneous International Federation of Clinical Chemistry-calibrated measurements of creatinine, cystatin C, and urea in children (mean age 12...
May 17, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29766272/blood-pressure-is-normal-but-is-the-heart
#17
Serkan Fazlı Çelik, Cemşit Karakurt, Yılmaz Tabel, Taner Elmas, Saim Yoloğlu
BACKGROUND: There is no detailed strain analysis of cardiac functions in treated hypertensive pediatric patients. The aim of this study was to evaluate the cardio-protective effects of different drug classes in treated pediatric hypertensive patients. METHODS: Sixty non-obese-treated hypertensive patients with preserved left ventricular (LV) systolic function and 45 age-, sex-, and body mass index-matched healthy subjects underwent clinical evaluation, including 24-h ambulatory blood pressure monitoring, standard echocardiographic examination, tissue Doppler imaging, and two-dimensional Speckle Tracking Echocardiography...
May 15, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29750317/mycophenolate-mofetil-for-sustained-remission-in-nephrotic-syndrome
#18
Uwe Querfeld, Lutz T Weber
The clinical application of mycophenolate mofetil (MMF) has significantly widened beyond the prophylaxis of acute and chronic rejections in solid organ transplantation. MMF has been recognized as an excellent treatment option in many immunologic glomerulopathies. For children with frequently relapsing nephrotic syndrome (FRNS) or steroid-dependent nephrotic syndrome (SDNS) experiencing steroid toxicity, MMF has been recommended as a steroid-sparing drug. Uncontrolled studies in patients with FRNS and SDSN have shown that many patients can achieve sustained remission of proteinuria with MMF monotherapy...
May 11, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29728803/acquired-thrombotic-thrombocytopenic-purpura-with-isolated-cfhr3-1-deletion-rapid-remission-following-complement-blockade
#19
Martin Bitzan, Rawan M Hammad, Arnaud Bonnefoy, Watfa Shahwan Al Dhaheri, Catherine Vézina, Georges-Étienne Rivard
BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is caused by the abundance of uncleaved ultralarge von Willebrand factor multimers (ULvWF) due to acquired (autoantibody-mediated) or congenital vWF protease ADAMTS13 deficiency. Current treatment recommendations include plasma exchange therapy and immunosuppression for the acquired form and (fresh) frozen plasma for congenital TTP. CASE-DIAGNOSIS/TREATMENT: A previously healthy, 3-year-old boy presented with acute microangiopathic hemolytic anemia, thrombocytopenia, erythrocyturia and mild proteinuria, but normal renal function, and elevated circulating sC5b-9 levels indicating complement activation...
May 4, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29725772/benefits-and-risks-of-protocol-biopsies-in-pediatric-renal-transplantation
#20
Roberto Gordillo, Raj Munshi, Eric J Monroe, Giridhar M Shivaram, Jodi M Smith
Protocol biopsies are defined as sampling of allograft tissue at predetermined times regardless of function. This procedure can be justified due to the lack of non-invasive methods to reliably diagnose rejection (acute or subclinical). Changes in creatinine are not seen with subclinical rejection or early acute rejection and do not always correlate with efficacy of treatment. Parents and providers are still hesitant to pursue protocol biopsy due to the potential complications and lack of definitive evidence of a benefit from doing this procedure...
May 3, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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