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Pediatric Nephrology: Journal of the International Pediatric Nephrology Association

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https://www.readbyqxmd.com/read/29046945/cystic-renal-mass-in-an-infant-with-significant-family-history-answers
#1
Elsa Wynd, Peter Borzi, John Burke
No abstract text is available yet for this article.
October 18, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29046944/complement-functional-tests-for-monitoring-eculizumab-treatment-in-patients-with-atypical-hemolytic-uremic-syndrome-an-update
#2
Gianluigi Ardissino, Francesca Tel, Martina Sgarbanti, Donata Cresseri, Antenore Giussani, Samantha Griffini, Elena Grovetto, Ilaria Possenti, Michela Perrone, Sara Testa, Fabio Paglialonga, Piergiorgio Messa, Massimo Cugno
BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy (TMA) characterized by platelet consumption, hemolysis, and organ damage. Eculizumab (ECU), a humanized antibody that blocks complement activity, has been successfully used in aHUS, but the best treatment schedule is not yet clear. METHODS: Here, we report our experience with ECU maintenance treatment and the interval between subsequent doses being extended based on global classical complement pathway (CCP) activity aimed at <30% for maintaining aHUS into remission...
October 18, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29034405/immunohistochemical-and-serological-characterization-of-membranous-nephropathy-in-children-and-adolescents
#3
Anne K Dettmar, Thorsten Wiech, Markus J Kemper, Armin Soave, Michael Rink, Jun Oh, Rolf A K Stahl, Elion Hoxha
BACKGROUND: Membranous nephropathy (MN) is a common cause of nephrotic syndrome in adults, but is less frequent in children. Antibodies against four antigens leading to MN have been described in children: phospholipase A2 receptor 1 (PLA2R1), thrombospondin type-1 domain-containing 7A (THSD7A), neutral endopeptidase (NEP), and cationic bovine serum albumin (BSA). METHODS: Twelve children with MN were included in this study. Sera of all patients were analyzed for antibodies against PLA2R1, THSD7A, NEP, and BSA...
October 15, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29032433/diffuse-mesangial-sclerosis-in-a-pdss2-mutation-induced-coenzyme-q10-deficiency
#4
Béla Iványi, Gábor Z Rácz, Péter Gál, Kitti Brinyiczki, István Bódi, Tibor Kalmár, Zoltán Maróti, Csaba Bereczki
BACKGROUND: A 7-month-old male infant was admitted because he was suffering from nephrotic syndrome, along with encephalomyopathy, hypertrophic cardiomyopathy, clinically suspected deafness and retinitis pigmentosa, and an elevated serum lactate level. METHODS: Coenzyme Q10 supplementation was started because of the clinical suspicion of primary CoQ10 deficiency. Despite intensive efforts, he passed away 4 weeks after admission. RESULTS: The results of genetic tests, available postmortem, explored two hitherto undescribed mutations in the PDSS2 gene...
October 14, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29022104/deriving-and-understanding-the-risk-of-post-transplant-recurrence-of-nephrotic-syndrome-in-the-light-of-current-molecular-and-genetic-advances
#5
REVIEW
Agnieszka Bierzynska, Moin A Saleem
After renal transplantation, recurrence of the original disease is the second most common cause of graft loss, after rejection. The most dramatic manifestation of this phenomenon is in patients with nephrotic syndrome (NS). NS is a descriptive term describing a clinical picture centred on proteinuria arising from damage to the glomerular filtration barrier (GFB). There are many different drivers of that damage, ranging from immune dysregulation to genetic disorders and chronic disease/infections. The main categories in childhood are "idiopathic" (presumed immune mediated) and genetic NS, with further stratification of the idiopathic group according to steroid responses...
October 11, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28993887/the-effect-of-everolimus-on-renal-angiomyolipoma-in-pediatric-patients-with-tuberous-sclerosis-being-treated-for-subependymal-giant-cell-astrocytoma
#6
John J Bissler, David N Franz, Michael D Frost, Elena Belousova, E Martina Bebin, Steven Sparagana, Noah Berkowitz, Antonia Ridolfi, J Christopher Kingswood
BACKGROUND: Patients with tuberous sclerosis complex (TSC) often have multiple TSC-associated hamartomas, particularly in the brain and kidney. METHODS: This was a post hoc analysis of pediatric patients being treated for subependymal giant cell astrocytomas (SEGAs) during the phase 3, randomized, double-blind, placebo-controlled EXIST-1 trial. Patients were initially randomly assigned to receive everolimus 4.5 mg/m(2)/day (target blood trough 5-15 mg/dl) or placebo and could continue in an open-label extension phase...
October 9, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28993886/thrombotic-microangiopathy-following-haematopoietic-stem-cell-transplant
#7
Eleanor G Seaby, Rodney D Gilbert
Thrombotic microangiopathy is a potentially lethal complication of haematopoietic stem cell (bone marrow) transplantation. The pathophysiology is incompletely understood, although endothelial damage appears to be central. Platelet activation, neutrophil extracellular traps and complement activation appear to play key roles. Diagnosis may be difficult and universally accepted diagnostic criteria are not available. Treatment remains controversial. In some cases, withdrawal of calcineurin inhibitors is adequate...
October 9, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29038887/mutations-in-inf2-may-be-associated-with-renal-histology-other-than-focal-segmental-glomerulosclerosis
#8
Anja K Büscher, Nora Celebi, Peter F Hoyer, Hanns-Georg Klein, Stefanie Weber, Julia Hoefele
BACKGROUND: In 2010, INF2 mutations were associated with autosomal-dominant focal segmental glomerulosclerosis (FSGS), clinically presenting with moderate proteinuria in adolescence. However, in the meantime, cases with more severe clinical courses have been described, including progression to end-stage renal disease (ESRD) during childhood. INF2 mutations in patients with isolated FSGS are clustered in exons 2 to 4, encoding the diaphanous inhibitory domain, involved in the regulation of the podocyte actin cytoskeleton...
October 6, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28983789/acute-kidney-injury-in-necrotizing-enterocolitis-predicts-mortality
#9
Cory N Criss, David T Selewski, Bipin Sunkara, Joshua S Gish, Lily Hsieh, Jennifer S Mcleod, Jason O Robertson, Niki Matusko, Samir K Gadepalli
BACKGROUND: Morbidity and mortality with necrotizing enterocolitis (NEC) remains a significant challenge. Acute kidney injury (AKI) has been shown to worsen survival in critically ill neonates. To our knowledge, this study is the first to evaluate the prevalence of AKI and its impact on outcomes in neonatal NEC. METHODS: We carried out a single-center retrospective chart review of all neonates treated for NEC between 2003 and 2015 (N = 181). AKI is defined as a rise in serum creatinine (SCr) from a previous trough according to neonatal modified KDIGO criteria (stage 1 = SCr rise 0...
October 5, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28983708/screening-children-for-hypertension-the-case-against
#10
Nicole Ide, Matthew Thompson
No abstract text is available yet for this article.
October 5, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28983704/presentation-of-pediatric-henoch-sch%C3%A3-nlein-purpura-nephritis-changes-with-age-and-renal-histology-depends-on-biopsy-timing
#11
Imke Hennies, Charlotte Gimpel, Jutta Gellermann, Kristina Möller, Brigitte Mayer, Katalin Dittrich, Anja K Büscher, Matthias Hansen, Wiebke Aulbert, Elke Wühl, Richard Nissel, Gessa Schalk, Lutz T Weber, Michael Pohl, Simone Wygoda, Rolf Beetz, Günter Klaus, Henry Fehrenbach, Sabine König, Hagen Staude, Ortraud Beringer, Martin Bald, Ulrike Walden, Christian von Schnakenburg, Gunhard Bertram, Michael Wallot, Karsten Häffner, Thorsten Wiech, Peter F Hoyer, Martin Pohl
BACKGROUND: This study correlates the clinical presentation of Henoch-Schönlein purpura nephritis (HSPN) with findings on initial renal biopsy. METHODS: Data from 202 pediatric patients enrolled in the HSPN registry of the German Society of Pediatric Nephrology reported by 26 centers between 2008 and 2014 were analyzed. All biopsy reports were re-evaluated for the presence of cellular crescents or chronic pathological lesions (fibrous crescents, glomerular sclerosis, tubular atrophy >5%, and interstitial fibrosis >5%)...
October 5, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28983694/incomplete-vaccination-coverage-in-european-children-with-end-stage-kidney-disease-prior-to-renal-transplantation
#12
Britta Höcker, Martin Aguilar, Paul Schnitzler, Lars Pape, Luca Dello Strologo, Nicholas J A Webb, Martin Bald, Gurkan Genc, Heiko Billing, Jens König, Anja Büscher, Markus J Kemper, Stephen D Marks, Martin Pohl, Marianne Wigger, Rezan Topaloglu, Susanne Rieger, Kai Krupka, Thomas Bruckner, Alexander Fichtner, Burkhard Tönshoff
BACKGROUND: Because infections constitute a major cause of morbidity and mortality in paediatric renal allograft recipients, avoidance of preventable systemic infections by vaccination before transplantation is of utmost importance. However, data on the completeness of vaccinations and factors associated with incomplete vaccination coverage are scarce. METHODS: Within the framework of the Cooperative European Paediatric Renal Transplant Initiative (CERTAIN), we therefore performed a multi-centre, multi-national, retrospective study investigating the vaccination coverage before transplantation of 254 European children with end-stage renal disease (mean age 10...
October 5, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28975420/diagnosis-and-management-of-bladder-bowel-dysfunction-in-children-with-urinary-tract-infections-a-position-statement-from-the-international-children-s-continence-society
#13
Stephen Yang, Michael E Chua, Stuart Bauer, Anne Wright, Per Brandström, Piet Hoebeke, Søren Rittig, Mario De Gennaro, Elizabeth Jackson, Eliane Fonseca, Anka Nieuwhof-Leppink, Paul Austin
BACKGROUND: We present a consensus view from the International Children's Continence Society (ICCS) on the evaluation and management of bladder bowel dysfunction (BBD) in children with urinary tract infection (UTI). The statement aims to highlight the importance of BBD in the development and recurrence of childhood UTI and its management to reduce its associated morbidity and sequelae. METHODS: A systematic literature search was done on PubMed, Embase, and Scopus databases until August 15, 2016...
October 3, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28971263/is-it-time-for-a-multi-specialty-approach-to-cardio-renal-dysfunction-in-children-with-cyanotic-congenital-heart-disease
#14
Tanya Holt, Guido Filler
No abstract text is available yet for this article.
October 2, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28971258/prevalence-and-associated-factors-of-renal-dysfunction-and-proteinuria-in-cyanotic-congenital-heart-disease
#15
Nattaphorn Hongsawong, Prapimdaw Khamdee, Suchaya Silvilairat, Wattana Chartapisak
BACKGROUND: Cyanotic nephropathy (CN), seen in 30-50% of patients with congenital cyanotic heart disease (CCHD), affects both tubular and glomerular function, resulting in proteinuria and azotemia. Microalbuminuria is an early marker for glomerular damage and an independent predictor of progressive renal disease. METHODS: A cross-sectional study was conducted. A total of 116 patients aged 1 month to 15 years with CCHD at Chiang Mai University Hospital between 2015 and 2016 were assessed and 94 patients were enrolled...
October 2, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28971255/to-screen-or-not-to-screen-for-high-blood-pressure
#16
EDITORIAL
Kjell Tullus
No abstract text is available yet for this article.
October 2, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28965242/uteroglobin-gene-polymorphism-g38a-may-be-a-risk-factor-in-childhood-idiopathic-nephrotic-syndrome
#17
Beltinge Demircioglu Kılıc, Mithat Buyukcelik, Sibel Oguzkan Balcı, Sacide Pehlivan, Seval Kul, Nilgun Col, Ayse Balat
BACKGROUND: Uteroglobin (UG) is a multifunctional protein with anti-inflammatory properties. The aim of this study was to first evaluate the role of UG gene G38A polymorphism in childhood idiopathic nephrotic syndrome (INS), and determine whether this variation may be related to the occurrence of INS or a steroid response. METHODS: One hundred and thirty-six children diagnosed with INS in Gaziantep University, Department of Pediatric Nephrology, and 70 healthy volunteers were included...
September 30, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28948294/reviews-submited-from-september-1st-2016-to-august-31st-2017
#18
(no author information available yet)
No abstract text is available yet for this article.
September 25, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28939943/accumulation-of-uraemic-toxins-is-reflected-only-partially-by-estimated-gfr-in-paediatric-patients-with-chronic-kidney-disease
#19
Evelien Snauwaert, Wim Van Biesen, Ann Raes, Els Holvoet, Griet Glorieux, Koen Van Hoeck, Maria Van Dyck, Nathalie Godefroid, Raymond Vanholder, Sanne Roels, Johan Vande Walle, Sunny Eloot
BACKGROUND: Chronic kidney disease (CKD) in childhood is characterised by the accumulation of uraemic toxins resulting in a multisystem disorder that has a negative impact on quality of life. Childhood CKD is predominantly defined by a decrease in glomerular filtration rate, estimated (eGFR) by a single serum measurement of endogenous biomarkers, e.g. creatinine. The objective of this study was to evaluate how accurately eGFR predicts the concentration of uraemic toxins in a paediatric CKD cohort...
September 22, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28939921/pathophysiology-and-treatment-of-cardiovascular-disease-in-pediatric-chronic-kidney-disease
#20
REVIEW
Nadine Khouzam, Katherine Wesseling-Perry
Life expectancy in patients with all stages of chronic kidney disease (CKD) falls far short of that in the general population. Cardiovascular disease is the leading cause of mortality in pediatric patients with CKD. In contrast to the intimal atherosclerotic lesions that characterize cardiovascular disease in the general population, vascular endothelial dysfunction, medial arterial calcification, and cardiac dysfunction contribute to cardiovascular pathological conditions in CKD. The pathogenesis of these lesions, the origins of which can be identified in the absence of traditional cardiovascular risk factors, is incompletely understood...
September 22, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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