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https://www.readbyqxmd.com/read/29149305/rituximab-for-non-responsive-idiopathic-membranous-nephropathy-in-a-chinese-cohort
#1
Xin Wang, Zhao Cui, Yi-Miao Zhang, Zhen Qu, Fang Wang, Li-Qiang Meng, Xu-Yang Cheng, Gang Liu, Fu-de Zhou, Ming-Hui Zhao
Background: Rituximab had been shown to be effective in inducing remission of nephrotic syndrome in patients with idiopathic membranous nephropathy (iMN). This study applied rituximab therapy for 36 non-responsive iMN patients to investigate its effects and safety. Methods: Thirty-six iMN patients who were non-responsive to prior immunosuppression were enrolled. Rituximab was used for B-cell depletion in patients, with a goal of <5 B cells/mm3 in the circulation...
November 14, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/29148523/nephrotic-syndrome-and-duplication-of-the-inferior-vena-cava-a-series-of-unfortunate-events
#2
Katarzyna Klejna, Tomasz Hryszko, Radosław Zawadzki, Beata Naumnik
No abstract text is available yet for this article.
November 17, 2017: Polish Archives of Internal Medicine
https://www.readbyqxmd.com/read/29146214/-treatments-of-steroid-dependent-nephrotic-syndrome-in-children
#3
A Couderc, E Bérard, V Guigonis, I Vrillon, J Hogan, V Audard, V Baudouin, C Dossier, O Boyer
Primary nephrotic syndrome (NS) is the most common glomerular disease in children. It is characterized by massive proteinuria and hypoalbuminemia. It typically has a sudden onset and more than 70% of patients will experience at least one relapse. An immunological origin has long been postulated, although the precise molecular mechanisms underlying the disease remain debated. Steroids are the first-line therapy with cumulative dose and duration of initial treatment varying among countries. Steroid-sparing agents may be indicated in case of steroid-dependency or frequent relapses...
November 13, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/29143203/relapse-of-nephrotic-syndrome-triggered-by-kawasaki-disease
#4
Ryo Maeda, Yukihiko Kawasaki, Shigeo Suzuki, Shinichiro Ohara, Suyama Kazuhide, Mitsuaki Hosoya
Minor infections, allergies, insect bites, and bee stings are commonly reported causes of nephrotic syndrome (NS). Herein, we report, to the best of our knowledge, the first case of NS relapse due to Kawasaki disease (KD). An 8-year-old boy presented with high fever of 4-day duration. He had developed steroid-dependent NS at the age of 4 years and remained in remission after steroid and mizonbin therapy. Renal biopsy, performed at the age of four, showed minimal change (MC) disease. Upon examination, the patient fulfilled 5 of 6 criteria for KD under the Japanese diagnostic guidelines, with positive proteinuria...
November 15, 2017: CEN Case Reports
https://www.readbyqxmd.com/read/29142983/efficacy-and-safety-of-sparsentan-compared-with-irbesartan-in-patients-with-primary-focal-segmental-glomerulosclerosis-randomized-controlled-trial-design-duet
#5
Radko Komers, Debbie S Gipson, Peter Nelson, Sharon Adler, Tarak Srivastava, Vimal K Derebail, Kevin E Meyers, Pablo Pergola, Meghan E MacNally, Jennifer L Hunt, Alvin Shih, Howard Trachtman
Introduction: Primary focal segmental glomerulosclerosis (FSGS) is a leading cause of nephrotic syndrome and end-stage renal disease. There are no US Food and Drug Administration-approved therapies for FSGS, and treatment often fails to reduce proteinuria. Endothelin is an important factor in the pathophysiology of podocyte disorders, including FSGS. Sparsentan is a first-in-class, orally active, dual-acting angiotensin receptor blocker (ARB) and highly selective endothelin Type A receptor antagonist...
July 2017: KI Reports
https://www.readbyqxmd.com/read/29141789/a-case-of-seropositive-neuromyelitis-optica-in-a-paediatric-patient-with-co-existing-acute-nephrotic-syndrome
#6
Thomas Volkman, Cheryl Hemingway
Neuromyelitis optica (NMO) and NMO spectrum disorder (NMOSD) is a rare relapsing autoimmune disease of the central nervous system constituting less than 1% of demyelinating diseases (Jeffery and Buncic, 1996). It preferentially affects the optic nerves and spinal cord, with the brain parenchyma generally spared. Demyelinating lesions are characterised by longitudinally extensive transverse myelitis (LETM) and often longitudinally extensive optic neuritis. Following the discovery of a novel pathogenic antibody, Aquaporin 4 in 2004 (Lennon et al...
November 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/29141309/-interpretation-of-guidelines-for-the-diagnosis-and-treatment-of-steroid-resistant-nephrotic-syndrome
#7
C L Gao, Z K Xia
No abstract text is available yet for this article.
November 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29141308/-guidelines-for-the-diagnosis-and-treatment-of-steroid-resistant-nephrotic-syndrome
#8
(no author information available yet)
No abstract text is available yet for this article.
November 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29131245/expression-of-the-wnt-%C3%AE-catenin-signal-pathway-in-patients-with-acute-renal-injury
#9
S-Y Wang, S-X Yang, X-X Zhao, F Chen, J Shi
OBJECTIVE: The purpose of this investigation is to determine if the protein expression of the Wnt/β-catenin signal pathway is induced after acute renal injury in patients with acute tubular necrosis. PATIENTS AND METHODS: Sixty patients in who met inclusion criteria underwent renal biopsy. Based on the result of the biopsy, patients were divided into two groups: one with nephrotic syndrome with mini-change disease (MCD group, 30 patients), the other one with minor lesions and acute tubular necrosis (ATN, 30 patients)...
October 2017: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/29130775/results-of-native-and-transplant-kidney-biopsies-of-children-in-a-single-center-over-a-15-years-period
#10
Emel Isiyel, Kibriya Fidan, Bahar Buyukkaragoz, Meltem Akcaboy, Yasar Kandur, Ipek Isik Gonul, Necla Buyan, Sevcan Bakkaloglu, Oguz Soylemezoglu
Renal biopsy is an important diagnostic procedure in pediatric nephrology. This study retrospectively investigates the indications, results and complications in a single tertiary children's hospital in Turkey. We evaluated the native and transplant kidney biopsies in Gazi University Pediatric Nephrology Department between 2001 and 2015. A total of 196 biopsies (144 natives and 52 transplants) were included into the study. The mean age of the patients was respectively 10.8 ± 3.5, 13.9 ± 1.5 years...
November 2017: Renal Failure
https://www.readbyqxmd.com/read/29129927/nephrotic-syndrome-avil-mutations-reduce-podocyte-migration-rate-in-srns
#11
Jack M Heintze
No abstract text is available yet for this article.
November 13, 2017: Nature Reviews. Nephrology
https://www.readbyqxmd.com/read/29127259/whole-exome-sequencing-of-patients-with-steroid-resistant-nephrotic-syndrome
#12
Jillian K Warejko, Weizhen Tan, Ankana Daga, David Schapiro, Jennifer A Lawson, Shirlee Shril, Svjetlana Lovric, Shazia Ashraf, Jia Rao, Tobias Hermle, Tilman Jobst-Schwan, Eugen Widmeier, Amar J Majmundar, Ronen Schneider, Heon Yung Gee, J Magdalena Schmidt, Asaf Vivante, Amelie T van der Ven, Hadas Ityel, Jing Chen, Carolin E Sadowski, Stefan Kohl, Werner L Pabst, Makiko Nakayama, Michael J G Somers, Nancy M Rodig, Ghaleb Daouk, Michelle Baum, Deborah R Stein, Michael A Ferguson, Avram Z Traum, Neveen A Soliman, Jameela A Kari, Sherif El Desoky, Hanan Fathy, Martin Zenker, Sevcan A Bakkaloglu, Dominik Müller, Aytul Noyan, Fatih Ozaltin, Melissa A Cadnapaphornchai, Seema Hashmi, Jeffrey Hopcian, Jeffrey B Kopp, Nadine Benador, Detlef Bockenhauer, Radovan Bogdanovic, Nataša Stajić, Gil Chernin, Robert Ettenger, Henry Fehrenbach, Markus Kemper, Reyner Loza Munarriz, Ludmila Podracka, Rainer Büscher, Erkin Serdaroglu, Velibor Tasic, Shrikant Mane, Richard P Lifton, Daniela A Braun, Friedhelm Hildebrandt
BACKGROUND AND OBJECTIVES: Steroid-resistant nephrotic syndrome overwhelmingly progresses to ESRD. More than 30 monogenic genes have been identified to cause steroid-resistant nephrotic syndrome. We previously detected causative mutations using targeted panel sequencing in 30% of patients with steroid-resistant nephrotic syndrome. Panel sequencing has a number of limitations when compared with whole exome sequencing. We employed whole exome sequencing to detect monogenic causes of steroid-resistant nephrotic syndrome in an international cohort of 300 families...
November 10, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/29127133/nephrotic-syndrome-and-acute-kidney-injury-induced-by-malathion-toxicity
#13
Kei Yokota, Masamichi Fukuda, Ritsuko Katafuchi, Tadashi Okamoto
We treated a case of acute kidney injury and nephrotic syndrome after malathion inhalation. A 69-year-old Japanese man presented with oedema 15 days after inhalation of malathion, a widely used pesticide. Serum albumin was 2.4 g/dL, urinary protein 8.6 g/gCr and serum creatinine 2.5 mg/dL. Kidney biopsy revealed tubular cell damage, epithelial cell damage in glomeruli and diffuse foot process effacement in electron microscopy. Acute kidney injury progressed to treatment with dialysis. Renal function recovered after corticosteroid administration from the 43rd day after admission...
November 9, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29124323/-goodpasture-syndrome-ocular-manifestation-in-a-young-man
#14
C Lommatzsch, A Lommatzsch, C Heinz, A Heiligenhaus
Goodpasture syndrome (GS) is a rare organ-specific autoimmune disease that may consist of glomerulonephritis and pulmonary hemorrhage. Only few case reports have been published on ocular symptoms in the context of GS. This is a case report of a 22-year-old male with GS who was referred to our practice because of visual disturbances. The anamnesis revealed pulmonary hemorrhage, acute renal failure and nephrotic syndrome, which was treated by immunosuppressive therapy with additional plasmapheresis. Ophthalmoscopically, bilateral cotton wool spots were found along the vessels and bilateral retinal hemorrhages predominantly at the posterior pole...
November 9, 2017: Der Ophthalmologe: Zeitschrift der Deutschen Ophthalmologischen Gesellschaft
https://www.readbyqxmd.com/read/29120340/mycophenolate-sodium-for-children-with-frequently-relapsing-or-steroid-dependent-nephrotic-syndrome
#15
Kanika Kapoor, Abhijeet Saha, Manpreet Kaur, Nand Kishore Dubey, Ashish Datt Upadhyay
In this retrospective study, patients with idiopathic frequently-relapsing nephrotic syndrome (FRNS) (n=27) and steroid dependent nephrotic syndrome (SDNS) (n=13) who received enteric coated mycophenolate sodium (ECMS) for at least 6 months, were included for analysis. Primary outcome was response to ECMS, which was defined as complete if there were no relapses, partial response if there was 1 relapse and no response if there were 2 or more relapses within 6 months of initiation. The mean (SD) dose of ECMS was 985...
October 15, 2017: Indian Pediatrics
https://www.readbyqxmd.com/read/29119640/il-23-actived-%C3%AE-%C3%AE-t-cells-affect-th17-cells-and-regulatory-t-cells-by-secreting-il-21-in-children-with-primary-nephrotic-syndrome
#16
Li Zhang, Junli Yan, Baohui Yang, Gaofu Zhang, Mo Wang, Shifang Dong, Wei Liu, Haiping Yang, Qiu Li
OBJECTIVE: This study (1) analyzed the percentage of γδ T cells, γδ T cell subsets, Th17 cells, and regulatory T cells (Treg cells), and (2) determined the role of IL-23 in primary nephrotic syndrome (PNS) patients with active disease and in remission. MATERIALS AND METHODS: Eighty-four PNS patients and 51 healthy age-matched controls were included in this study. The percentage of γδ T cells, γδ T cell subsets, Th17 cells, and Treg cells in peripheral blood mononuclear cells (PBMCs) were analyzed by fluorescence-activated cell sorting...
November 9, 2017: Scandinavian Journal of Immunology
https://www.readbyqxmd.com/read/29110886/the-incidence-of-primary-vs-secondary-focal-segmental-glomerulosclerosis-a-clinicopathologic-study
#17
Musab S Hommos, An S De Vriese, Mariam P Alexander, Sanjeev Sethi, Lisa Vaughan, Ladan Zand, Kharmen Bharucha, Nicola Lepori, Andrew D Rule, Fernando C Fervenza
OBJECTIVES: To describe the change in the incidence rates of primary and secondary focal segmental glomerulosclerosis (FSGS) from 1994 through 2013 in Olmsted County, Minnesota, and to identify the clinical and biopsy characteristics that distinguish primary from secondary FSGS. PATIENTS AND METHODS: Olmsted County adult residents with native kidney biopsy from January 1, 1994, through December 31, 2013, and FSGS as the only glomerulopathy were identified. The clinical and pathologic characterstics of primary and secondary FSGS were described and compared, and incidence rates were calculated...
October 27, 2017: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/29104670/expression-of-foxp3-in-renal-tissue-of-patients-with-hbv-associated-glomerulonephritis-and-their-clinical-and-pathological-characteristics
#18
Wenge Zhao, Yanli Ma, Mingmin Wang, Changhe Shi, Jianping Sun, Kaiqiu Chu, Chuansheng Liu
Our study retrospectively investigated the expression of forkhead/winged helix transcription factor (Foxp3) in renal tissue and clinical features of patients with hepatitis B virus (HBV)-associated glomerulonephritis (HBV-GN). A total of 58 patients with HBV-GN were assigned to group A; 45 serum and renal tissue HBsAg-negative patients with nephritis were group B; 24 serum HBsAg-positive and renal tissue HBsAg-negative patients with slightly increased serum creatinine without nephritis were group C. Clinical manifestations, laboratory indices and renal biopsies were recorded...
November 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29100540/isolated-right-ventricular-thrombus-in-an-adult-patient-with-nephrotic-syndrome-a-case-report
#19
Severin Lempp, Vedat Schwenger
BACKGROUND: Venous thrombosis in nephrotic syndrome is a well-described phenomenon. We report a case of an adult patient with an isolated thrombus in the right ventricle due to nephrotic syndrome, which was initially suspected to be a myxoma. CASE PRESENTATION: A 28-year-old white woman presented to our emergency department with signs of fluid overload. On further evaluation, a right ventricular mass was detected, which was resected and was found to be a thrombus...
November 4, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29098438/acute-kidney-injury-in-patients-with-nephrotic-syndrome-undergoing-contrast-enhanced-ct-for-suspected-venous-thromboembolism-a-propensity-score-matched-retrospective-cohort-study
#20
Shu Min Tao, Xiang Kong, U Joseph Schoepf, Julian L Wichmann, Darby C Shuler, Chang Sheng Zhou, Guang Ming Lu, Long Jiang Zhang
OBJECTIVES: To determine whether intravenous iodinated contrast material administration increases the risk of acute kidney injury (AKI) in patients with nephrotic syndrome undergoing contrast-enhanced CT. METHODS: Patients with nephrotic syndrome undergoing contrast-enhanced CT were retrospectively identified (n = 701). Control group consisted of patients with nephrotic syndrome receiving non-contrast CT (n = 1053). Two different 1:1 propensity score matching models using three or 10 variables were developed for each estimated glomerular filtration (eGFR) subgroup...
November 2, 2017: European Radiology
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