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Minimal change nephrotic syndrome

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https://www.readbyqxmd.com/read/28642375/acquired-resistance-to-corticotropin-therapy-in-nephrotic-syndrome-role-of-de-novo-neutralizing-antibody
#1
Pei Wang, Yan Zhang, Yu Wang, Andrew S Brem, Zhangsuo Liu, Rujun Gong
There is increasing evidence supporting the use of corticotropin as an alternative treatment of refractory proteinuric glomerulopathies. The efficacy of short-acting corticotropin, however, remains unknown and was tested here in an adolescent with steroid-dependent nephrotic syndrome caused by minimal change disease. After developing Cushing syndrome and recently being afflicted with severe cellulitis, the patient was weaned off all immunosuppressants, including corticosteroids. This resulted in a relapse of generalized anasarca, associated with massive proteinuria and hypoalbuminemia...
June 22, 2017: Pediatrics
https://www.readbyqxmd.com/read/28638601/the-efficacy-of-rituximab-in-adult-frequently-relapsing-minimal-change-disease
#2
Catherine King, Sarah Logan, Stuart W Smith, Peter Hewins
BACKGROUND: Corticosteroids are the basis of treatment for nephrotic syndrome due to minimal change disease (MCD), but 25% of patients have frequently relapsing nephrotic syndrome (FRNS) and 30% become steroid dependent. Prolonged use of conventional immunosuppressants causes significant toxicity. Rituximab (RTX) is now included in guidelines for childhood MCD. Evidence for use in adult MCD is limited. We describe a single-centre experience of RTX use in adult MCD. METHODS: Outcomes of all adult MCD patients treated with RTX for FRNS between 2008 and 2015 were retrospectively analysed...
February 2017: Clinical Kidney Journal
https://www.readbyqxmd.com/read/28626447/autoimmune-thyroiditis-and-glomerulopathies
#3
REVIEW
Domenico Santoro, Carmela Vadalà, Rossella Siligato, Michele Buemi, Salvatore Benvenga
Autoimmune thyroiditis (AIT) is generally associated with hypothyroidism. It affects ~2% of the female population and 0.2% of the male population. The evidence of thyroid function- and thyroid autoantibody-unrelated microproteinuria in almost half of patients with AIT and sometimes heavy proteinuria as in the nephrotic syndrome point to a link of AIT with renal disease. The most common renal diseases observed in AIT are membranous nephropathy, membranoproliferative glomerulonephritis, minimal change disease, IgA nephropathy, focal segmental glomerulosclerosis, antineutrophil cytoplasmic autoantibody (ANCA) vasculitis, and amyloidosis...
2017: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/28617070/oral-subchronic-exposure-to-silver-nanoparticles-causes-renal-damage-through-apoptotic-impairment-and-necrotic-cell-death
#4
Ratnakar Tiwari, Radha Dutt Singh, Hafizurrahman Khan, Siddhartha Gangopadhyay, Sandeep Mittal, Vikas Singh, Nidhi Arjaria, Jai Shankar, Somendu Kumar Roy, Dhirendra Singh, Vikas Srivastava
Silver nanoparticles (AgNPs) are one of the most widely used nanomaterials. Following oral exposure, AgNPs can accumulate in various organs including kidneys where they show gender specific accumulation. There is limited information on their effect on renal system following long-term animal exposure especially at the ultramicroscopic and molecular level. In this study, we have assessed the effect of 60 days oral AgNPs treatment on kidneys of female Wistar rats at doses of 50 ppm and 200 ppm that are below previously reported lowest observed adverse effect level (LOAEL)...
June 15, 2017: Nanotoxicology
https://www.readbyqxmd.com/read/28615961/the-role-of-novel-biomarkers-in-childhood-idiopathic-nephrotic-syndrome-a-narrative-review-of-published-evidence
#5
REVIEW
Samuel N Uwaezuoke
Two histological subtypes of idiopathic nephrotic syndrome are commonly recognized in children, namely minimal change nephropathy and focal segmental glomerulosclerosis. Children with minimal change nephropathy (the majority of whom are steroid-sensitive) and focal segmental glomerulosclerosis (the majority of whom are steroid-resistant) require early identification in order to ensure appropriate therapeutic intervention and better outcome. Although renal biopsy and histology remain the ideal diagnostic steps to identify these histological subtypes, reports indicate that serum and urinary biomarkers are now being utilized in the investigation of childhood idiopathic nephrotic syndrome...
2017: International Journal of Nephrology and Renovascular Disease
https://www.readbyqxmd.com/read/28608180/montelukast-improves-the-changes-of-cytoskeletal-and-adaptor-proteins-of-human-podocytes-by-interleukin-13
#6
Tae-Sun Ha, Ja Ae Nam, Su-Bin Seong, Moin A Saleem, Se Jin Park, Jae Il Shin
OBJECTIVE AND DESIGN: Interleukin-13 (IL-13) has recently been reported to be a potential cytokine in the pathogenesis of minimal-change nephrotic syndrome (MCNS). However, the mechanistic insights associated with podocyte dysfunction mediated by IL-13-induced changes in various slit diaphragm (SD) and cytoskeletal molecules have not yet been shown in cultured human podocytes in vitro. MATERIALS: Human conditionally immortalized podocytes were used. TREATMENT: Podocytes were incubated with various concentrations of IL-13 during the indicated time periods (6, 12, and 24 h) and montelukast was administered with the dose of 0...
June 12, 2017: Inflammation Research: Official Journal of the European Histamine Research Society ... [et Al.]
https://www.readbyqxmd.com/read/28566477/long-term-outcome-of-steroid-resistant-nephrotic-syndrome-in-children
#7
Agnes Trautmann, Sven Schnaidt, Beata S Lipska-Ziętkiewicz, Monica Bodria, Fatih Ozaltin, Francesco Emma, Ali Anarat, Anette Melk, Marta Azocar, Jun Oh, Bassam Saeed, Alaleh Gheisari, Salim Caliskan, Jutta Gellermann, Lina Maria Serna Higuita, Augustina Jankauskiene, Dorota Drozdz, Sevgi Mir, Ayse Balat, Maria Szczepanska, Dusan Paripovic, Alexandra Zurowska, Radovan Bogdanovic, Alev Yilmaz, Bruno Ranchin, Esra Baskin, Ozlem Erdogan, Giuseppe Remuzzi, Agnieszka Firszt-Adamczyk, Elzbieta Kuzma-Mroczkowska, Mieczyslaw Litwin, Luisa Murer, Marcin Tkaczyk, Helena Jardim, Anna Wasilewska, Nikoleta Printza, Kibriya Fidan, Eva Simkova, Halina Borzecka, Hagen Staude, Katharina Hees, Franz Schaefer
We investigated the value of genetic, histopathologic, and early treatment response information in prognosing long-term renal outcome in children with primary steroid-resistant nephrotic syndrome. From the PodoNet Registry, we obtained longitudinal clinical information for 1354 patients (disease onset at >3 months and <20 years of age): 612 had documented responsiveness to intensified immunosuppression (IIS), 1155 had kidney biopsy results, and 212 had an established genetic diagnosis. We assessed risk factors for ESRD using multivariate Cox regression models...
May 31, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28553650/recent-treatment-advances-and-new-trials-in-adult-nephrotic-syndrome
#8
REVIEW
Eva Königshausen, Lorenz Sellin
The etiology of nephrotic syndrome is complex and ranges from primary glomerulonephritis to secondary forms. Patients with nephrotic syndrome often need immunosuppressive treatment with its side effects and may progress to end stage renal disease. This review focuses on recent advances in the treatment of primary causes of nephrotic syndrome (idiopathic membranous nephropathy (iMN), minimal change disease (MCD), and focal segmental glomerulosclerosis (FSGS)) since the publication of the KDIGO guidelines in 2012...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28540445/indications-for-kidney-biopsy-in-idiopathic-childhood-nephrotic-syndrome
#9
Alanoud Alshami, Abishek Roshan, Marisa Catapang, Jasper J Jöbsis, Trevor Kwok, Nonnie Polderman, Jennifer Sibley, Matt Sibley, Cherry Mammen, Douglas G Matsell
BACKGROUND: Most cases of childhood nephrotic syndrome (NS) are due to minimal change disease (MCD), while a minority of children have focal segmental glomerulosclerosis (FSGS) and an unfavorable clinical course, requiring a kidney biopsy to confirm diagnosis. We hypothesized that clinical characteristics at diagnosis and initial response to corticosteroid treatment accurately predict FSGS and can be used to guide consistent practice in the indications for kidney biopsy. METHODS: This was a case control study (1990-2012)...
May 24, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28540057/rituximab-in-minimal-change-disease-mechanisms-of-action-and-hypotheses-for-future-studies
#10
REVIEW
Nima Madanchi, Martin Bitzan, Tomoko Takano
Treatment with rituximab, a monoclonal antibody against the B-lymphocyte surface protein CD20, leads to the depletion of B cells. Recently, rituximab was reported to effectively prevent relapses of glucocorticoid-dependent or frequently relapsing minimal change disease (MCD). MCD is thought to be T-cell mediated; how rituximab controls MCD is not understood. In this review, we summarize key clinical studies demonstrating the efficacy of rituximab in idiopathic nephrotic syndrome, mainly MCD. We then discuss immunological features of this disease and potential mechanisms of action of rituximab in its treatment based on what is known about the therapeutic action of rituximab in other immune-mediated disorders...
2017: Canadian Journal of Kidney Health and Disease
https://www.readbyqxmd.com/read/28530079/-kidney-diseases-in-north-israel-according-to-kidney-biopsies-bnai-zion-medical-center-14-years-experience
#11
Shimon Storch, Nadav Willner, Aurora Toubi, Simona Croitoru, Vladimir Wolfson, Ibrahim Matar, Elinor Grushka, Majed Odeh, Efrat Wolfovits, Elad Schiff, Yitzhak Rosner, Elias Toubi, Aharon Kessel, Ofer Ben Izhak, Boaz Moskovitz, Ofer Nativ
INTRODUCTION: Little is known about the prevalence of kidney diseases according to renal biopsy in Israel. Since updated literature worldwide emphasizes changing etiologies of chronic kidney disease, it is crucial to research and define the epidemiology and pathology of kidney disease in Israel. Hereby, we introduce an original review of the prevalence of kidney diseases in our study population, which we believe reflects the prevalence of kidney diseases in the population of Israel. AIMS: To investigate the prevalence of kidney diseases diagnosed by renal biopsy, according to age, gender, race and clinical symptoms...
September 2016: Harefuah
https://www.readbyqxmd.com/read/28522940/potential-urine-proteomics-biomarkers-for-primary-nephrotic-syndrome
#12
Young Wook Choi, Yang Gyun Kim, Min-Young Song, Ju-Young Moon, Kyung-Hwan Jeong, Tae-Won Lee, Chun-Gyoo Ihm, Kang-Sik Park, Sang-Ho Lee
BACKGROUND: Nephrotic syndrome (NS) is a nonspecific kidney disorder, commonly caused by minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS), and membranous nephropathy (MN). Here we analyzed urinary protein profiles, aiming to discover disease-specific biomarkers of these three common diseases in NS. METHODS: Sixteen urine samples were collected from patients with biopsy-proven NS and healthy controls. After removal of high-abundance proteins, the urinary protein profile was analyzed by LC-MS/MS to generate a discovery set...
2017: Clinical Proteomics
https://www.readbyqxmd.com/read/28483380/rac1-activation-in-podocytes-induces-the-spectrum-of-nephrotic-syndrome
#13
Richard Robins, Cindy Baldwin, Lamine Aoudjit, Jean-François Côté, Indra R Gupta, Tomoko Takano
Hyper-activation of Rac1, a small GTPase, in glomerular podocytes has been implicated in the pathogenesis of familial proteinuric kidney diseases. However, the role of Rac1 in acquired nephrotic syndrome is unknown. To gain direct insights into this, we generated a transgenic mouse model expressing a doxycycline-inducible constitutively active form of Rac1 (CA-Rac1) in podocytes. Regardless of the copy number, proteinuria occurred rapidly within five days, and the histology resembled minimal change disease...
May 5, 2017: Kidney International
https://www.readbyqxmd.com/read/28477260/atypical-thymic-carcinoid-manifesting-with-nephrotic-range-proteinuria-in-a-7-year-old-boy
#14
Jolanta Soltysiak, Danuta Ostalska-Nowicka, Katarzyna Zaorska, Karolina Sterzyńska, Katarzyna Derwich, Jacek Zachwieja
BACKGROUND: Nephrotic-range proteinuria as a paraneoplastic syndrome (PNS) is an exceptional presentation, especially in children. It is usually associated with hematologic malignancies. Solid tumors are very rare causes of proteinuria. CASE-DIAGNOSIS/TREATMENT: We present the case of a 7-year-old boy with an extremely rare atypical thymic carcinoid accompanied by nephrotic-range proteinuria as PNS. The kidney biopsy was consistent with minimal change disease (MCD)...
May 5, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28441404/angiopoietin-like-4-and-minimal-change-disease
#15
Gabriel Cara-Fuentes, Alfons Segarra, Cecilia Silva-Sanchez, Heiman Wang, Miguel A Lanaspa, Richard J Johnson, Eduardo H Garin
BACKGROUND: Minimal Change Disease (MCD) is the most common type of nephrotic syndrome in children. Angiopoietin-like-4 (Angplt4) has been proposed as mediator of proteinuria in MCD. The aim of this study was to evaluate the role of Angptl4 as a biomarker in MCD. METHODS: Patients with biopsy-proven primary MCD, focal segmental glomerulosclerosis, membranous nephropathy (60, 52 and 52 respectively) and 18 control subjects had urinary and serum Angptl4 measured by Elisa...
2017: PloS One
https://www.readbyqxmd.com/read/28425160/causes-of-nephrotic-syndrome-and-nephrotic-range-proteinuria-are-different-in-adult-chinese-patients-a-single-centre-study-over-33%C3%A2-years
#16
Jack Kit-Chung Ng, Terry King-Wing Ma, Fernand Mac-Moune Lai, Kai Ming Chow, Bonnie Ching-Ha Kwan, Chi Bon Leung, Philip Kam-To Li, Cheuk Chun Szeto
BACKGROUND: The reported causes of nephrotic syndrome (NS) varied between different countries. Less is known about the causes of nephrotic-range proteinuria (NPU). METHODS: In this single-centre, retrospective study of adult patients who underwent renal biopsy between 1983-2015 in a tertiary referral hospital in Hong Kong, we aimed to evaluate the underlying causes of NS and NPU. We also determined the distribution of histopathological diagnoses with regard to the age subgroups and time periods RESULTS: Among 7,456 patients who underwent renal biopsy, 982 and 838 patients had NS and NPU, respectively...
April 20, 2017: Nephrology
https://www.readbyqxmd.com/read/28416778/steroid-pulse-therapy-for-de-novo-minimal-change-disease-during-pregnancy
#17
Hiroshi Sato, Yuna Asami, Reona Shiro, Masato Aoki, Miki Yasuda, Saeko Imai, Rie Sakai, Kenji Oida, Kanako Kawaharamura, Hiroko Yano, Nao Taguchi, Takako Suzuki, Masaya Hirose
BACKGROUND Nephrotic syndrome occurs very rarely, in only about 0.01%-0.02% of all pregnancies, and de novo minimal change disease during pregnancy is especially rare. Nephrotic syndrome and, especially, minimal change disease are highly responsive to steroids, and preterm labor may be avoidable if the maternal condition is improved with steroid therapy. Therefore, prompt diagnosis and proper management are critical to maternal and fetal outcome when severe proteinuria occurs during pregnancy. CASE REPORT A 30-year-old pregnant Japanese woman presented with systemic edema, oliguria, and severe proteinuria and hypoalbuminemia at 25 weeks of gestation, although she was normotensive...
April 18, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28396739/nephrotic-syndrome-due-to-minimal-change-disease-secondary-to-spider-bite-clinico-pathological-case-of-a-non-described-complication-of-latrodectism
#18
Gonzalo P Méndez, Daniel Enos, José Luis Moreira, Fátima Alvaredo, David Oddó
The patient was an 18-year-old man who developed nephrotic syndrome after a 'wheat spider' bite (Latrodectus mactans). Due to this atypical manifestation of latrodectism, a renal biopsy was performed showing minimal change disease. The nephrotic syndrome subsided after 1 week without specific treatment. This self-limited evolution suggests that the mechanism of podocyte damage was temporary and potentially mediated by a secondary mechanism of hypersensitivity or direct effect of the α-latrotoxin. The patient did not show signs of relapse in subsequent checkup...
April 2017: Clinical Kidney Journal
https://www.readbyqxmd.com/read/28352022/analysis-of-renal-diseases-detected-in-renal-biopsies-of-adult-patients-a-single-center-experience
#19
Salman Imtiaz, Murtaza F Drohlia, Kiran Nasir, Beena Salman, Aasim Ahmad
Renal biopsy is crucial while evaluating for the diagnosis of glomerular, vascular, tubulointerstitial, and genetic diseases. It gives vital information which helps in estimating the disease prognosis, progression, and management. This is the retrospective analysis of all adult patients aged above 18 years, who underwent percutaneous renal biopsy at The Kidney Center Post Graduate Training Institute, Karachi, over a duration of 18 years, i.e., January 1, 1996, to December 2013. Renal graft biopsies and those which were inadequate were excluded from analysis...
March 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28339906/interstitial-fibrosis-scored-on-whole-slide-digital-imaging-of-kidney-biopsies-is-a-predictor-of-outcome-in-proteinuric-glomerulopathies
#20
Laura H Mariani, Sebastian Martini, Laura Barisoni, Pietro A Canetta, Jonathan P Troost, Jeffrey B Hodgin, Matthew Palmer, Avi Z Rosenberg, Kevin V Lemley, Hui-Ping Chien, Jarcy Zee, Abigail Smith, Gerald B Appel, Howard Trachtman, Stephen M Hewitt, Matthias Kretzler, Serena M Bagnasco
Background.: Interstitial fibrosis (IF), tubular atrophy (TA) and interstitial inflammation (II) are known determinants of progression of renal disease. Standardized quantification of these features could add value to current classification of glomerulopathies. Methods.: We studied 315 participants in the Nephrotic Syndrome Study Network (NEPTUNE) study, including biopsy-proven minimal change disease (MCD = 98), focal segmental glomerulosclerosis (FSGS = 121), membranous nephropathy (MN = 59) and IgA nephropathy (IgAN = 37)...
February 27, 2017: Nephrology, Dialysis, Transplantation
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