Database : MEDLINE
Search on : nephrotic and syndrome [Words]
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[PMID]: 29523515
[Au] Autor:Maharaj S; Seegobin K; Chrzanowski S; Chang S
[Ad] Address:Internal Medicine, University of Florida College of Medicine, Jacksonville, Florida, USA.
[Ti] Title:Acute glomerulonephritis secondary to .
[So] Source:BMJ Case Rep;2018, 2018 Mar 09.
[Is] ISSN:1757-790X
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:is a clinically important pathogen that is emerging globally but remains poorly investigated. Here, we report the first case of acute glomerulonephritis resulting from infection with Glomerulonephritis is typically caused by and reports secondary to other strains including and exist. Infection with in this patient was associated with acute nephritis (haematuria, oedema and hypertension), nephrotic syndrome and progressive azotemia. There was activation of the complement system. The presence of low C1q and elevated anti-C1q binding complexes points to a potential pathogenic role. Testing for streptococcal antigens was strongly positive. Emerging nephritogenic strains of present a significant health concern for both developed and developing countries.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180310
[Lr] Last revision date:180310
[St] Status:In-Process

  2 / 20510 MEDLINE  
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[PMID]: 29523091
[Au] Autor:Mrabet S; Aicha NB; Abdessayed N; Mokni M; Achour A
[Ad] Address:Department of Nephrology, Dialysis and transplantation. Sahloul university Hospital, Sousse, Tunisia. snaiida@yahoo.fr.
[Ti] Title:Membranous nephropathy succeeding autologous hematopoietic stem cell transplant: a case report.
[So] Source:BMC Nephrol;19(1):57, 2018 Mar 09.
[Is] ISSN:1471-2369
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:BACKGROUND: Membranous nephropathy (MN), the leading cause of nephrotic syndrome in adults, is characterized by the deposition of subepithelial immune deposits. Most of the cases are primary, while only approximately 25% of the cases are secondary to some known diseases. Recently, MN has been considered to be a possible presentation of chronic graft-versus-host disease (GVHD) of the kidney in allogeneic hematopoietic stem cell transplantation (HSCT) patients. In autologous HSCT populations, there have been scarce reports of associated MN, as a result of immune dysregulation leading to systemic autoimmunity and miming chronic GVHD. CASE PRESENTATION: We report an exceptional case of MN associated to an acute renal failure occurring within days following an autologous HSCT indicated by multiple myeloma. There was no evidence of GVHD or myeloma relapse. A complete remission of nephrotic syndrome with normalization of renal function were rapidly obtained by corticosteroid therapy. CONCLUSION: This is the first published case of acute renal failure due to MN occurring in the acute phase of an autologous HSCT. These findings support the antibodymediated autoimmune glomerular disease.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180310
[Lr] Last revision date:180310
[St] Status:In-Data-Review
[do] DOI:10.1186/s12882-018-0855-z

  3 / 20510 MEDLINE  
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[PMID]: 29457738
[Au] Autor:Zee J; Hodgin JB; Mariani LH; Gaut JP; Palmer MB; Bagnasco SM; Rosenberg AZ; Hewitt SM; Holzman LB; Gillespie BW; Barisoni L
[Ad] Address:From Biostatistics, Arbor Research Collaborative for Health, Ann Arbor, Michigan (Dr Zee); the Departments of Pathology (Dr Hodgin), Internal Medicine (Dr Mariani), and Biostatistics (Dr Gillespie), University of Michigan, Ann Arbor; Arbor Research Collaborative for Health, Ann Arbor, Michigan (Dr M
[Ti] Title:Reproducibility and Feasibility of Strategies for Morphologic Assessment of Renal Biopsies Using the Nephrotic Syndrome Study Network Digital Pathology Scoring System.
[So] Source:Arch Pathol Lab Med;, 2018 Feb 19.
[Is] ISSN:1543-2165
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:CONTEXT: - Testing reproducibility is critical for the development of methodologies for morphologic assessment. Our previous study using the descriptor-based Nephrotic Syndrome Study Network Digital Pathology Scoring System (NDPSS) on glomerular images revealed variable reproducibility. OBJECTIVE: - To test reproducibility and feasibility of alternative scoring strategies for digital morphologic assessment of glomeruli and explore use of alternative agreement statistics. DESIGN: - The original NDPSS was modified (NDPSS1 and NDPSS2) to evaluate (1) independent scoring of each individual biopsy level, (2) use of continuous measures, (3) groupings of individual descriptors into classes and subclasses prior to scoring, and (4) indication of pathologists' confidence/uncertainty for any given score. Three and 5 pathologists scored 157 and 79 glomeruli using the NDPSS1 and NDPSS2, respectively. Agreement was tested using conventional (Cohen κ) and alternative (Gwet agreement coefficient 1 [AC ]) agreement statistics and compared with previously published data (original NDPSS). RESULTS: - Overall, pathologists' uncertainty was low, favoring application of the Gwet AC . Greater agreement was achieved using the Gwet AC compared with the Cohen κ across all scoring methodologies. Mean (standard deviation) differences in agreement estimates using the NDPSS1 and NDPSS2 compared with the single-level original NDPSS were -0.09 (0.17) and -0.17 (0.17), respectively. Using the Gwet AC , 79% of the original NDPSS descriptors had good or excellent agreement. Pathologist feedback indicated the NDPSS1 and NDPSS2 were time-consuming. CONCLUSIONS: - The NDPSS1 and NDPSS2 increased pathologists' scoring burden without improving reproducibility. Use of alternative agreement statistics was strongly supported. We suggest using the original NDPSS on whole slide images for glomerular morphology assessment and for guiding future automated technologies.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180309
[Lr] Last revision date:180309
[St] Status:Publisher
[do] DOI:10.5858/arpa.2017-0181-OA

  4 / 20510 MEDLINE  
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[PMID]: 29518831
[Au] Autor:Dong YF; Sun LW; Zhang B; Kuang XY; Niu XL; Kang YL; Hao S; Wang P; Li Z; Zhu GH; Huang WY; Wu Y
[Ad] Address:Department of Nephrology, the Children's Hospital of Shanghai Jiao Tong University, Shanghai 200062, China.
[Ti] Title:[Clinical features and expression of PLA(2)R in renal tissue with idiopathic membranous nephropathy in children].
[So] Source:Zhonghua Er Ke Za Zhi;56(3):206-210, 2018 Mar 02.
[Is] ISSN:0578-1310
[Cp] Country of publication:China
[La] Language:chi
[Ab] Abstract:To explore the clinical features and expression of PLA(2)R in renal tissue of children with idiopathic membranous nephropathy. Retrospective study was performed in patients with membranous nephropathy diagnosed through renal biopsy and the follow-up time was at least half a year in ' from January 2010 to February 2017. We compared their clinicopathological and pathological findings of IMN. Indirect immunofluorescence assay was used to detect glomerular PLA(2)R expression. We analyzed the differences of clinical features between the PLA(2)R negative and positive groups. T test, rank-sum test and Fisher exact test were used. Eleven cases had hematuria and proteinuria, 9 cases presented with nephrotic syndrome, and 2 cases showed isolated proteinuria. Of the 22 cases of children with IMN, 16 patients had complete remission (complete remission rate was 72.8%), and 22 patients had partial remission. The renal function of all cases was normal and in all cases the estimated glomerular filtration rate was > 90 ml/(min·1.73m(2)). Of 22 cases with IMN, 7 cases were PLA(2)R-positive in renal tissue and 15 cases were PLA(2)R-negative. The age of positive group (10 years old) was older than the negative group (6 years old)( -2.483, 0.05) and the time of positive group (6 months) for urine protein to return to negative was longer than the negative group (2.5 months) through treatment. These differences were significantly different ( -2.072, 0.05). Hematuria and proteinuria can be found in most children with idiopathic primary membranous nephropathy. Prednisone combined with immunosuppressant was effective. The positive rate of PLA(2)R in renal tissue of children with IMN was about 32%. The age of PLA(2)R positive group was older than the negative group. And the time of urine protein turning to negative in positive group was longer than that in the negative group.
[Pt] Publication type:ENGLISH ABSTRACT; JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180309
[Lr] Last revision date:180309
[St] Status:In-Process
[do] DOI:10.3760/cma.j.issn.0578-1310.2018.03.010

  5 / 20510 MEDLINE  
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[PMID]: 29378770
[Au] Autor:Bomback AS
[Ad] Address:Division of Nephrology, Department of Medicine, Columbia University College of Physicians and Surgeons, New York, New York asb68@columbia.edu.
[Ti] Title:Management of Membranous Nephropathy in the PLA R Era.
[So] Source:Clin J Am Soc Nephrol;, 2018 Jan 29.
[Is] ISSN:1555-905X
[Cp] Country of publication:United States
[La] Language:eng
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1801
[Cu] Class update date: 180309
[Lr] Last revision date:180309
[St] Status:Publisher

  6 / 20510 MEDLINE  
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[PMID]: 29517874
[Au] Autor:Scheen AJ; Paquot N; Lancellotti P; Krzesinski JM
[Ad] Address:Service de Diabétologie, Nutrition et Maladies métaboliques et Unité de Pharmacologie clinique, CHU Sart Tilman, Liège, Belgique.
[Ti] Title:Vignette diagnostique de l'étudiant. Oedème des membres inférieurs chez un patient diabétique de type 2. [Lower limb oedema in a patient with type 2 diabetes].
[So] Source:Rev Med Liege;73(2):101-106, 2018 Feb.
[Is] ISSN:0370-629X
[Cp] Country of publication:Belgium
[La] Language:fre
[Ab] Abstract:Differential diagnosis of lower limb oedema is a common exercise in clinical practice. Taking the example of a patient presenting with such clinical picture in the presence of type 2 diabetes and arterial hypertension with coronary heart disease and chronic kidney disease as comorbidities, we discuss here the respective contributions of congestive heart failure, renal impairment (and possibly nephrotic syndrome) and liver disease in the development of lower limb oedema. The focus is made on a careful patient history and a meticulous clinical examination, two crucial steps that should allow prescribing well selected simple complementary procedures and rapidly make the final diagnosis.
[Pt] Publication type:ENGLISH ABSTRACT; PRACTICE GUIDELINE
[Em] Entry month:1803
[Cu] Class update date: 180308
[Lr] Last revision date:180308
[St] Status:In-Data-Review

  7 / 20510 MEDLINE  
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[PMID]: 29512444
[Au] Autor:Du X; Nyagblordzro M; An L; Gao X; Du L; Wang Y; Ondieki G; Kikete S; He X
[Ad] Address:School of Chinese Materia Medica, Tianjin University of Traditional Chinese Medicine, Nankai District, Tianjin 300193. China.
[Ti] Title:Pharmacokinetic and Toxicological Characteristics of Tripterigium Glycosides and Their Derivatives.
[So] Source:Curr Drug Metab;, 2018 Mar 02.
[Is] ISSN:1875-5453
[Cp] Country of publication:Netherlands
[La] Language:eng
[Ab] Abstract:Tripterigium wilfordii glycosides (TWG) demonstrate paramount bioactive effectiveness in the management of many autoimmune diseases, such as rheumatoid arthritis, psoriasis, and nephrotic syndrome. However, its side effects on the hepatic, nephrotic, reproductive, and cardiovascular systems have limited its immense therapeutic potentials. Triptolide (TP) and Celastrol (CL), the leading bioactive as well as toxic constituents of TWG, have been widely studied. These studies have documented the key mechanisms that trigger the toxic reactions and the precautionary measures that could prevent and reduce such reactions. It has been reported that reactive oxygen species (ROS) generation, mitochondrial respiratory chain inhibition, cauda epididymis sperm structure disruption, and metabolizing enzyme inhibition are the leading factors of the toxic reactions. The bioactive effects and toxicities of TWG are closely related to its metabolic profiles. It has been confirmed that TP and CL inhibit Cytochrome P450 (CYP) and the transporters. This paper reviews and summarizes the pharmacokinetic and toxicological parameters of TWG, some mitigative interventions and several implications of its pharmacokinetic characteristics. Antioxidants, polymeric micelle formulations, topical nanoparticle formulations have exhibited potentials in toxicity circumvention. A thorough understanding of the pharmacokinetic and toxicological characteristics of TWG combined with further in-depth study of its efficacy and toxicity will enhance the efficacy and safety in using TWG, which would augment and improve its clinical application in the future.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180307
[Lr] Last revision date:180307
[St] Status:Publisher
[do] DOI:10.2174/1389200219666180302152752

  8 / 20510 MEDLINE  
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[PMID]: 29501407
[Au] Autor:Bamborschke D; Pergande M; Becker K; Körber F; Dötsch J; Vierzig A; Weber LT; Cirak S
[Ad] Address:Center for Molecular Medicine Cologne, Cologne, Germany; Department of Pediatrics, University Hospital of Cologne, Cologne, Germany.
[Ti] Title:A novel mutation in sphingosine-1-phosphate lyase causing congenital brain malformation.
[So] Source:Brain Dev;, 2018 Mar 02.
[Is] ISSN:1872-7131
[Cp] Country of publication:Netherlands
[La] Language:eng
[Ab] Abstract:INTRODUCTION: Recently recessive mutations in sphingosine-1-phosphate lyase (SGPL1) have been published as a cause of syndromic congenital nephrotic syndrome with adrenal insufficiency. We have identified a case with fetal hydrops and brain malformations due to a mutation in SGPL1. CASE REPORT: We report a patient presenting with severe fetal hydrops, congenital nephrotic syndrome and adrenal calcifications. MRI imaging showed generalized cortical atrophy with simplified gyral pattern and hypoplastic temporal lobes as well as cerebellar hypoplasia and hyperintensity in the pons. The boy deceased at 6 weeks of age. Via whole exome sequencing, we identified a novel homozygous frameshift mutation c.1233delC (p.Phe411Leufs 56) in SGPL1. CONCLUSION: In our patient, we describe a novel mutation in sphingosine-1-phosphate lyase (SGPL1) leading to severe brain malformation. Neurodevelopmental phenotypes have been reported earlier, but not described in detail. To this end, we present a review on all published SGPL1-mutations and genotype-phenotype correlations focusing on neurodevelopmental outcomes. We hypothesized on the severe neurological phenotypes, which might be due to disruption of neuronal autophagy. Mutations in SGPL1 shall be considered in the differential diagnosis of fetal hydrops as well as congenital brain malformations and neuropathies.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180307
[Lr] Last revision date:180307
[St] Status:Publisher

  9 / 20510 MEDLINE  
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[PMID]: 29490294
[Au] Autor:Wang S; Pan Q; Xu C; Li JJ; Tang HX; Zou T; Jing KP; Ye L; Wu HL; Liu WJ; Liu HF
[Ti] Title:Massive Proteinuria-Induced Injury of Tubular Epithelial Cells in Nephrotic Syndrome is Not Exacerbated by Furosemide.
[So] Source:Cell Physiol Biochem;45(4):1700-1706, 2018.
[Is] ISSN:1421-9778
[Cp] Country of publication:Switzerland
[La] Language:eng
[Ab] Abstract:BACKGROUND/AIMS: Massive proteinuria, a significant sign of nephrotic syndrome (NS), has the potential to injure tubular epithelial cells (TECs). Furosemide is widely used for the treatment of edema, a common manifestation of NS. However, whether furosemide treatment affects massive proteinuria-induced TEC injury in patients with NS is unknown. METHODS: The effect of furosemide on TEC damage was investigated in vitro. In addition, a clinical study was conducted to study whether the short-term treatment of nephrotic edema with furosemide could exacerbate TEC injury. RESULTS: The proliferation of in vitro human kidney-2 (HK-2) cells exposed to massive urinary protein (8 mg/mL) significantly decreased (P<0.05), while the levels of kidney injury molecule-1 (Kim-1) and neutrophil gelatinase associated lipocalin (NGAL) in the supernatants significantly increased (P<0.05). Importantly, furosemide treatment did not further increase the expression of Kim-1 and NGAL in HK-2 cells upregulated by massive proteinuria. For the clinical study, 26 patients with NS, all prescribed the recommended dosage of prednisone (1 mg/kg/day), were randomly assigned to two groups. One group (n=13) received furosemide (60-120 mg/day, intravenously) for 1 week; the remaining participants (control group) did not receive furosemide or any other diuretics. The results showed that the 24-h urine volume in the furosemide-treated group was slightly, but not significantly, higher than that in the control group (P>0.05). In addition, serum levels of BUN, Scr, Cys C, and urinary Kim-1 and NGAL were not significantly different between the two groups (all P>0.05). Twenty-three patients underwent a renal biopsy. Of these, 22 patients exhibited vacuolar degeneration of the TECs; 8 patients showed brush border membrane shedding of the TECs; and 12 patients showed protein casts. However, there were no significant differences between the two groups (all P>0.05). CONCLUSION: In summary, massive proteinuria induced the injury of TECs in patients with NS, and furosemide treatment did not aggravate this injury.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180306
[Lr] Last revision date:180306
[St] Status:In-Process
[do] DOI:10.1159/000487776

  10 / 20510 MEDLINE  
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[PMID]: 29264866
[Au] Autor:Lambe T; Frew E; Ives NJ; Woolley RL; Cummins C; Brettell EA; Barsoum EN; Webb NJA; PREDNOS Trial Team
[Ad] Address:Health Economics Unit, Institute of Applied Health Research, University of Birmingham, Edgbaston, Birmingham, UK.
[Ti] Title:Mapping the Paediatric Quality of Life Inventory (PedsQL™) Generic Core Scales onto the Child Health Utility Index-9 Dimension (CHU-9D) Score for Economic Evaluation in Children.
[So] Source:Pharmacoeconomics;36(4):451-465, 2018 Apr.
[Is] ISSN:1179-2027
[Cp] Country of publication:New Zealand
[La] Language:eng
[Ab] Abstract:BACKGROUND: The Paediatric Quality of Life Inventory (PedsQL™) questionnaire is a widely used, generic instrument designed for measuring health-related quality of life (HRQoL); however, it is not preference-based and therefore not suitable for cost-utility analysis. The Child Health Utility Index-9 Dimension (CHU-9D), however, is a preference-based instrument that has been primarily developed to support cost-utility analysis. OBJECTIVE: This paper presents a method for estimating CHU-9D index scores from responses to the PedsQL™ using data from a randomised controlled trial of prednisolone therapy for treatment of childhood corticosteroid-sensitive nephrotic syndrome. METHODS: HRQoL data were collected from children at randomisation, week 16, and months 12, 18, 24, 36 and 48. Observations on children aged 5 years and older were pooled across all data collection timepoints and were then randomised into an estimation (n = 279) and validation (n = 284) sample. A number of models were developed using the estimation data before internal validation. The best model was chosen using multi-stage selection criteria. RESULTS: Most of the models developed accurately predicted the CHU-9D mean index score. The best performing model was a generalised linear model (mean absolute error = 0.0408; mean square error = 0.0035). The proportion of index scores deviating from the observed scores by <  0.03 was 53%. CONCLUSIONS: The mapping algorithm provides an empirical tool for estimating CHU-9D index scores and for conducting cost-utility analyses within clinical studies that have only collected PedsQL™ data. It is valid for children aged 5 years or older. Caution should be exercised when using this with children younger than 5 years, older adolescents (>  13 years) or patient groups with particularly poor quality of life. ISRCTN REGISTRY NO: 16645249.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1712
[Cu] Class update date: 180307
[Lr] Last revision date:180307
[St] Status:In-Data-Review
[do] DOI:10.1007/s40273-017-0600-7


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