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Nephrotic syndrom

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https://www.readbyqxmd.com/read/29054945/acute-onset-diplopia-in-a-case-of-nephrotic-syndrome
#1
Nripen Gaur, Pradeep Sharma, Brijesh Takkar, Jagjeet Singh
No abstract text is available yet for this article.
October 19, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29054532/a-randomized-clinical-trial-indicates-that-levamisole-increases-the-time-to-relapse-in-children-with-steroid-sensitive-idiopathic-nephrotic-syndrome
#2
Mariken P Gruppen, Antonia H Bouts, Marijke C Jansen-van der Weide, Maruschka P Merkus, Aleksandra Zurowska, Michal Maternik, Laura Massella, Francesco Emma, Patrick Niaudet, Elisabeth A M Cornelissen, Thierry Schurmans, Ann Raes, Johan van de Walle, Mieke van Dyck, Ashima Gulati, Arvind Bagga, Jean-Claude Davin
Levamisole has been considered the least toxic and least expensive steroid-sparing drug for preventing relapses of steroid-sensitive idiopathic nephrotic syndrome (SSINS). However, evidence for this is limited as previous randomized clinical trials were found to have methodological limitations. Therefore, we conducted an international multicenter, placebo-controlled, double-blind, randomized clinical trial to reassess its usefulness in prevention of relapses in children with SSINS. The efficacy and safety of one year of levamisole treatment in children with SSINS and frequent relapses were evaluated...
October 17, 2017: Kidney International
https://www.readbyqxmd.com/read/29051055/skeletal-impairment-in-pierson-syndrome-is-there-a-role-for-laminin%C3%AE-2-in-bone-physiology
#3
Camille Beaufils, Delphine Farlay, Irma Machuca-Gayet, Alice Fassier, Martin Zenker, Caroline Freychet, Edith Bonnelye, Aurélia Bertholet-Thomas, Bruno Ranchin, Justine Bacchetta
INTRODUCTION: Pierson syndrome is caused by a mutation of LAMB2, encoding for laminin β2. Clinical phenotype is variable but usually associates congenital nephrotic syndrome (CNS) and ocular abnormalities. Neuromuscular impairment has also been described. METHODS: We report on a 15-year old girl, suffering from Pierson Syndrome, who developed severe bone deformations during puberty. This patient initially displayed CNS and microcoria, leading to the clinical diagnosis of Pierson syndrome...
October 16, 2017: Bone
https://www.readbyqxmd.com/read/29050110/-interpretation-of-evidence-based-guideline-on-diagnosis-and-treatment-of-steroid-sensitive-relasping-steroid-dependent-nephrotic-syndrome-in-children-2016
#4
F Yang, X Y Jiang
No abstract text is available yet for this article.
October 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29050108/-evidence-based-guideline-on-diagnosis-and-treatment-of-steroid-sensitive-relapsing-steroid-dependent-nephrotic-syndrome-in-children-2016
#5
(no author information available yet)
No abstract text is available yet for this article.
October 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29049388/an-inducible-mouse-model-of-podocin-mutation-related-nephrotic-syndrome
#6
Mansoureh Tabatabaeifar, Tanja Wlodkowski, Ivana Simic, Helga Denc, Geraldine Mollet, Stefanie Weber, John Julius Moyers, Barbara Brühl, Michael Joseph Randles, Rachel Lennon, Corinne Antignac, Franz Schaefer
Mutations in the NPHS2 gene, encoding podocin, cause hereditary nephrotic syndrome. The most common podocin mutation, R138Q, is associated with early disease onset and rapid progression to end-stage renal disease. Knock-in mice carrying a R140Q mutation, the mouse analogue of human R138Q, show developmental arrest of podocytes and lethal renal failure at neonatal age. Here we created a conditional podocin knock-in model named NPHS2 R140Q/-, using a tamoxifen-inducible Cre recombinase, which permits to study the effects of the mutation in postnatal life...
2017: PloS One
https://www.readbyqxmd.com/read/29045960/pharmacokinetics-of-once-daily-prolonged-release-formulation-of-tacrolimus-in-children-with-primary-nephrotic-syndrome
#7
Y Han, S Q DU, H J Xiao, Y Zhou, J Ding, J J Ding, Y M Cui
OBJECTIVE: Tacrolimus prolonged-release(PR) formulation is a new once-daily formulation of the calcineurin inhibitor tacrolimus, which is currently used in adult liver or kidney transplant patients,and is also gradually widely used in children with nephrotic syndrome.The present study was undertaken to preliminarily investigate the pharmacokinetic characteristics of tacrolimus PR in pediatric nephrotic syndrome recipients. METHODS: This single-center open-label prospective study was performed in pediatric nephrotic syndrome recipients...
October 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/29045958/-clinical-characteristics-of-glucocorticoid-induced-eye-adverse-reactions-in-children-with-primary-nephrotic-syndrome
#8
Y Zhao, B G Su, H J Xiao, H W Zhang, X Y Liu, F Wang, J Ding
OBJECTIVE: To investigate the clinical features and side effects, with regard to glucocorticoid-induced ocular hypertension, glaucoma or cataract in children with primary nephrotic syndrome. METHODS: Clinical data were collected and analyzed from 71 cases of primary nephrotic syndrome with glucocorticoid-induced ocular hypertension, glaucoma or cataract from Jun. 2014 to Jun. 2016. These children were hospitalized in Peking University First Hospital. RESULTS: Totally 1 580 children with primary nephrotic syndrome were collected, glucocorticoid-induced complications in eyes were found in 71 cases, and the incidence was 4...
October 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/29042083/aprotinin-prevents-proteolytic-epithelial-sodium-channel-enac-activation-and-volume-retention-in-nephrotic-syndrome
#9
Bernhard N Bohnert, Martina Menacher, Andrea Janessa, Matthias Wörn, Anja Schork, Sophie Daiminger, Hubert Kalbacher, Hans-Ulrich Häring, Christoph Daniel, Kerstin Amann, Florian Sure, Marko Bertog, Silke Haerteis, Christoph Korbmacher, Ferruh Artunc
Volume retention in nephrotic syndrome has been linked to activation of the epithelial sodium channel (ENaC) by proteolysis of its γ-subunit following urinary excretion of serine proteases such as plasmin. Here we tested whether pharmacological inhibition of urinary serine protease activity might protect from ENaC activation and volume retention in nephrotic syndrome. Urine from both nephrotic mice (induced by doxorubicin injection) and nephrotic patients exhibited high aprotinin-sensitive serine protease activity...
October 14, 2017: Kidney International
https://www.readbyqxmd.com/read/29034405/immunohistochemical-and-serological-characterization-of-membranous-nephropathy-in-children-and-adolescents
#10
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/29032953/abolishment-of-proximal-tubule-albumin-endocytosis-does-not-affect-plasma-albumin-during%C3%A2-nephrotic-syndrome-in-mice
#11
Kathrin Weyer, Pia K Andersen, Kasper Schmidt, Geraldine Mollet, Corinne Antignac, Henrik Birn, Rikke Nielsen, Erik I Christensen
The megalin/cubilin receptor complex is required for proximal tubular endocytosis and degradation of filtered albumin. An additional high-capacity retrieval pathway of intact albumin for the recovery of large amounts of filtered albumin has been proposed, possibly involving cooperation between megalin/cubilin and the neonatal Fc receptor. To clarify the potential role of such a pathway, we examined the effects of megalin/cubilin gene inactivation on tubular albumin uptake and plasma albumin levels in nephrotic, podocin knockout mice...
October 13, 2017: Kidney International
https://www.readbyqxmd.com/read/29032433/diffuse-mesangial-sclerosis-in-a-pdss2-mutation-induced-coenzyme-q10-deficiency
#12
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/29031489/-pediatric%C3%A2-nephrology-what%C3%A2-should-an%C3%A2-adult%C3%A2-nephrologist-know-about%C3%A2-these%C3%A2-diseases
#13
Sandrine Lemoine, Pierre Cochat, Aurélia Bertholet-Thomas, Charlène Levi, Catherine Bonnefoy, Anne-Laure Sellier-Leclerc, Justine Bacchetta
In nephrology, some diseases begin specifically during childhood; however, they must be known by adult nephrologists so that to ensure continuity and homogeneity for their management. The aim of this review is therefore to propose a brief overview of the main pediatric diseases, for which a specific knowledge is warranted, and notably pediatric idiopathic nephrotic syndrome, cystinosis, primary hyperoxaluria and hereditaries tubulopathies.
October 11, 2017: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/29031361/treatment-of-disorders-of-sodium-balance-in-chronic-kidney-disease
#14
REVIEW
David H Ellison
Extracellular fluid volume expansion is nearly universal in patients with CKD. Such volume expansion has features similar to the syndrome of heart failure with preserved ejection fraction, which not only leads to symptoms but can also lead to further organ damage. Unique treatment challenges are present in this patient population, including low glomerular filtration, which limits sodium chloride filtration, intrinsic tubule predisposition to sodium chloride retention, and proteinuria. In addition, pharmacokinetic considerations alter the disposition of diuretics in patients with CKD and nephrotic syndrome...
September 2017: Advances in Chronic Kidney Disease
https://www.readbyqxmd.com/read/29031360/sodium-homeostasis-in-chronic-kidney-disease
#15
REVIEW
Vivek Soi, Jerry Yee
The pathologic consequences of sodium retention in the CKD population can lead to hypertension, edema, and progressive disease. Sodium excess is responsible for increases in oxidative stress, which alters kidney vasculature. As progression of CKD occurs, hyperfiltration by remaining nephrons compensates for an overall decrease in the filtered load of sodium. In the later stages of CKD, compensatory mechanisms are overcome and volume overload ensues. Nephrotic syndrome as it relates to sodium handling involves a different pathophysiology despite a common phenotype...
September 2017: Advances in Chronic Kidney Disease
https://www.readbyqxmd.com/read/29030465/cluster-analysis-identifies-distinct-pathogenetic-patterns-in-c3-glomerulopathies-immune-complex-mediated-membranoproliferative-gn
#16
Paraskevas Iatropoulos, Erica Daina, Manuela Curreri, Rossella Piras, Elisabetta Valoti, Caterina Mele, Elena Bresin, Sara Gamba, Marta Alberti, Matteo Breno, Annalisa Perna, Serena Bettoni, Ettore Sabadini, Luisa Murer, Marina Vivarelli, Marina Noris, Giuseppe Remuzzi
Membranoproliferative GN (MPGN) was recently reclassified as alternative pathway complement-mediated C3 glomerulopathy (C3G) and immune complex-mediated membranoproliferative GN (IC-MPGN). However, genetic and acquired alternative pathway abnormalities are also observed in IC-MPGN. Here, we explored the presence of distinct disease entities characterized by specific pathophysiologic mechanisms. We performed unsupervised hierarchical clustering, a data-driven statistical approach, on histologic, genetic, and clinical data and data regarding serum/plasma complement parameters from 173 patients with C3G/IC-MPGN...
October 13, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/29022109/interaction-of-cd80-with-neph1-a-potential-mechanism-of-podocyte-injury
#17
Bhavya Khullar, Renu Balyan, Neelam Oswal, Nidhi Jain, Amita Sharma, Malik Z Abdin, Arvind Bagga, Shinjini Bhatnagar, Nitya Wadhwa, Uma Chandra Mouli Natchu, Anna George, Satyajit Rath, Vineeta Bal, Shailaja Sopory
BACKGROUND: The induction of CD80 on podocytes has been shown in animal models of podocyte injury and in certain cases of nephrotic syndrome. In a lipopolysaccharide (LPS)-induced mouse model of albuminuria, we have recently shown a signalling axis of LPS-myeloid cell activation-TNFα production-podocyte CD80 induction-albuminuria. Therefore, in this report, we investigated the cellular and molecular consequences of TNFα addition and CD80 expression on cultured podocytes. METHODS: A murine podocyte cell line was used for TNFα treatment and for over-expressing CD80...
October 11, 2017: Clinical and Experimental Nephrology
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
#18
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/29022080/urinary-potassium-to-urinary-potassium-plus-sodium-ratio-can-accurately-identify-hypovolemia-in-nephrotic-syndrome-a-provisional-study
#19
Werner Keenswijk, Mohamad Ikram Ilias, Ann Raes, Raymond Donckerwolcke, Johan Vande Walle
There is evidence pointing to a decrease of the glomerular filtration rate (GFR) in a subgroup of nephrotic children, likely secondary to hypovolemia. The aim of this study is to validate the use of urinary potassium to the sum of potassium plus sodium ratio (UK/UK+UNa) as an indicator of hypovolemia in nephrotic syndrome, enabling detection of those patients who will benefit from albumin infusion. We prospectively studied 44 nephrotic children and compared different parameters to a control group (36 children)...
October 11, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/29021446/nephrotic-syndrome-and-a-retroperitoneal-mass-a-case-report-of-a-patient-with-recurrent-invasive-thymoma
#20
Hiroaki Myoga, Tetsu Akimoto, Naoko Mato, Takakiyo Nakaya, Takuya Murakami, Hiromichi Yoshizawa, Saki Nakagawa, Atsushi Miki, Takahiro Masuda, Takahisa Kobayashi, Yuko Ono, Osamu Saito, Yoshihiko Ueda, Shigeaki Muto, Daisuke Nagata
A 68-year-old man was admitted to our hospital to undergo an examination for nephrotic syndrome while concurrently complicated with recurrent thymoma in the parietal pleura and retroperitoneum. He had been diagnosed with invasive thymoma and had undergone thymo-thymectomy seven years previously. Based on the renal biopsy findings, his nephrotic syndrome was ascribed to minimal change disease. He was treated with corticosteroid monotherapy, which resulted in complete remission six months later, despite the fact that the recurrent thymoma remained...
October 11, 2017: Internal Medicine
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