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Nephrotic syndrome

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https://www.readbyqxmd.com/read/29034405/immunohistochemical-and-serological-characterization-of-membranous-nephropathy-in-children-and-adolescents
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
https://www.readbyqxmd.com/read/29020719/diagnosis-and-management-of-nephrotic-syndrome
#12
Oonagh McCloskey, Alexander P Maxwell
Nephrotic syndrome is defined by a triad of clinical features: oedema, substantial proteinuria (> 3.5 g/24 hours) and hypoalbuminaemia (< 30 g/L). It is often associated with hyperlipidaemia, thromboembolism and an increased risk of infection. Nephrotic syndrome develops following pathological injury to renal glomeruli. This may be a primary problem, with a disease specific to the kidneys, or secondary to a systemic disorder such as diabetes mellitus. The most common cause in children is minimal change glomerulonephritis...
February 2017: Practitioner
https://www.readbyqxmd.com/read/28994502/pro-protein-subtilisin-kexin-9-pcsk9-inhibition-in-practice-lipid-clinic-experience-in-2-contrasting-uk-centres
#13
Monika Kohli, Kinjal Patel, Zofia MacMahon, Radha Ramachandran, Martin A Crook, Timothy M Reynolds, Anthony S Wierzbicki
BACKGROUND: Prescribing criteria have been suggested for proprotein convertase subtilisin kexin-9 (PCSK-9) inhibitors but few studies exist of their real-world effectiveness. METHODS: This study audited PCSK-9 inhibitor therapy in 105 consecutive patients from two hospital centres-a university hospital (UH; n = 70) and a district general hospital (DGH; n = 35). Baseline characteristics including cardiovascular disease risk factors, NICE qualification criteria, efficacy and side effects were assessed...
October 10, 2017: International Journal of Clinical Practice
https://www.readbyqxmd.com/read/28992850/association-of-single-nucleotide-polymorphism-in-the-fkbp5-gene-with-response-to-steroids-in-pediatric-patients-with-primary-nephrotic-syndrome%C3%A2
#14
Ni Du, Fang Yang, Liangzhi Li, Xiaoxiao Liu, Liangzhong Sun, Sui Zhang, Xianhong He, Yuewu Tang, Jihong Shi, Chunqiu Liu, Xianxiang Zhang
AIM: This study aimed to assess the association of single-nucleotide polymorphism (SNP) of FKBP5 with response to steroids in children with primary nephrotic syndrome (NS). MATERIALS AND METHODS: A total of 66 primary NS patients (cases) and 68 healthy individuals (controls) were enrolled in this study. The FKBP5 polymorphism rs4713916 (T/C) was analyzed by polymerase chain reaction (PCR) and sequencing after amplification of regions that potentially contain the SNP...
October 10, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28990100/low-molecular-weight-heparin-may-benefit-nephrotic-remission-in-steroid%C3%A2-sensitive-nephrotic-syndrome-via-inhibiting-elastase
#15
Songhui Zhai, Lijuan Hu, Lin Zhong, Yuhong Tao, Zheng Wang
Low molecular weight heparin (LMWH) has a structure similar to heparan sulfate, which exerts anti‑inflammatory effects via inhibiting elastase (Ela) activity. Release of Ela along the glomerular capillary wall may induce glomerular injury and proteinuria. The present study aimed to investigate the influence of LMWH on steroid‑sensitive nephrotic syndrome (SSNS) and the potential underlying mechanism. A total of 40 SSNS patients and 20 healthy controls were recruited. SSNS patients were treated with LMWH and prednisone simultaneously (LMWH+pred group) or with prednisone alone (pred group)...
October 3, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28979773/efficacy-of-mycophenolate-treatment-in-adults-with-steroid-dependent-frequently-relapsing-idiopathic-nephrotic-syndrome
#16
Diego Sandoval, Rafael Poveda, Juliana Draibe, Laureà Pérez-Oller, Montserrat Díaz, José Ballarín, Anna Saurina, Helena Marco, Josep Bonet, Xoana Barros, Xavier Fulladosa, Joan Torras, Josep M Cruzado
BACKGROUND: This study assessed the efficacy of therapy with mycophenolate (MF) and reduced doses of steroids in adults with steroid-dependent/frequently relapsing idiopathic nephrotic syndrome (SD/FR-INS). METHODS: Twenty-nine nephrotic patients (including 16 males and 13 females; mean age: 40 years, range: 18-74) were treated. Starting doses of MF were 2000 mg/day for mofetil MF (1500 mg/day in one patient) or 1440 mg/day for sodium MF. The initial prednisone (PDN) dose was 10 mg/day in 14 patients, 5 mg/day in two patients and no steroids in one patient...
October 2017: Clinical Kidney Journal
https://www.readbyqxmd.com/read/28979337/investigating-the-use-of-human-albumin-in-a-non-teaching-hospital-in-iran
#17
Farzaneh Foroughinia, Shahriar Mazraie
Albumin is an expensive drug which imposes relatively high cost on the health care system. Doing ABC analysis in Shahid Motahari Hospital, it was revealed that albumin is categorized in class A. Therefore, the present study aimed to evaluate the pattern of albumin use and the physicians' adherence to evidenced-based albumin guidelines in this large general non-teaching hospital in Shiraz, Iran. This study is an observational retrospective research on drug utilization. All patients admitted to Shahid Motahari hospital that had received albumin during the study period of one year (December 2013 to December 2014), were included in the study...
2017: Iranian Journal of Pharmaceutical Research: IJPR
https://www.readbyqxmd.com/read/28975100/impact-of-%C3%AE-h1-and-%C3%AE-h2-cytokines-in-the-progression-of-idiopathic-nephrotic-syndrome-due-to-focal-segmental-glomerulosclerosis-and-minimal-change-disease
#18
Maria Stangou, Μichael Spartalis, Dimitra-Vasilia Daikidou, Theodora Kouloukourgiotou, Erasmia Sampani, Ioanna-Theologia Lambropoulou, Afroditi Pantzaki, Αikaterini Papagianni, George Efstratiadis
BACKGROUND: Differential diagnosis between primary focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD) is sometimes difficult as nephrotic syndrome is the main clinical symptom in both diseases. OBJECTIVES: This study has attempted to evaluate the urinary excretion of Th1 and Th2 cytokines as potential biomarkers in distinguishing the two types of nephrotic syndrome, and predicting outcome of renal function. PATIENTS AND METHODS: Thirty-six patients with FSGS (M/F 22/14, Age; 41...
July 2017: Journal of Nephropathology
https://www.readbyqxmd.com/read/28975099/predictive-risk-factors-of-steroid-dependent-nephrotic-syndrome-in-children
#19
Maher Ahmed Abdel-Hafez, Nagy Mohamed Abou-El-Hana, Adel Ali Erfan, Mohamed El-Gamasy, Hend Abdel-Nabi
BACKGROUND: Development of steroid dependency is one of the difficult problems in the management of children with idiopathic nephrotic syndrome, leading to increased morbidity, complications and cost of treatment. Thus, predicting early in the disease course will be useful in counseling parents and may improve treatment strategy. OBJECTIVES: To determine the clinical characteristics that can predict the development of steroid dependency early in the initial episodes of steroid sensitive nephrotic syndrome (SSNS)...
July 2017: Journal of Nephropathology
https://www.readbyqxmd.com/read/28975093/association-of-endothelin-1-rs5370-g-t-gene-polymorphism-with-the-risk-of-nephrotic-syndrome-in-children
#20
Mohammad Hashemi, Simin Sadeghi-Bojd, Soheib Aryanezhad, Maryam Rezaei
BACKGROUND: Primary nephrotic syndrome (NS) is a common kidney disease in children. Objectives: The present study was aimed to investigate whether rs5370 G>T (lys198Asn) genetic variant of endothelin-1 (ET-1) is involved in the susceptibility to NS. PATIENTS AND METHODS: This case-control study was performed on 138 patients with NS and 150 healthy children. Genomic DNA was extracted from whole blood using salting out method. Polymorphism of the ET-1 rs5370 G>T (lys198Asn) polymorphism detected by T-ARMS-PCR as well as PCR-RFLP method...
July 2017: Journal of Nephropathology
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