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Nephrotic syndrome

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https://www.readbyqxmd.com/read/28643120/the-incidence-of-the-nephrotic-syndrome-in-childhood-in-germany
#1
Ingo Franke, Malik Aydin, Corinna Elke Llamas Lopez, Lisa Kurylowicz, Rainer Ganschow, Michael Lentze, Mark Born
BACKGROUND: The incidence of childhood nephrotic syndrome (NS) in Germany is not well known. METHODS: An ESPED-based nationwide collection of epidemiological data of children in 2005 and 2006. RESULT: The mean age of NS at onset was 5.5 ± 3.7 years. The gender ratio of boys to girls was 1.8:1. The average length of stay was 15.5 ± 11.2 days, with younger children remaining significantly longer in hospital. Steroid-resistance was more common in children ≥8 years (p = 0...
June 22, 2017: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/28642375/acquired-resistance-to-corticotropin-therapy-in-nephrotic-syndrome-role-of-de-novo-neutralizing-antibody
#2
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/28639503/markers-of-disease-and-steroid-responsiveness-in-paediatric-idiopathic-nephrotic-syndrome-whole-transcriptome-sequencing-of-peripheral-blood-mononuclear-cells
#3
Hee Gyung Kang, Heewon Seo, Jae Hyun Lim, Jong Il Kim, Kyoung Hee Han, Hye Won Park, Ja Wook Koo, Kee Hyuck Kim, Ju Han Kim, Hae Il Cheong, Il-Soo Ha
Objective To identify markers of disease and steroid responsiveness in paediatric idiopathic nephrotic syndrome. Methods Whole-transcriptome sequencing was performed of peripheral blood mononuclear cells (PBMCs) from patients with NS. Differentially expressed genes (DEGs) were identified in patients with active NS vs those in remission, and those with steroid-sensitive NS (SSNS) vs steroid-resistant NS (SRNS). Results A total of 1065 DEGs were identified in patients with NS ( n = 10) vs those in remission ( n = 9)...
June 2017: Journal of International Medical Research
https://www.readbyqxmd.com/read/28638602/b-cell-depleting-therapy-with-rituximab-or-ofatumumab-in-immunoglobulin-a-nephropathy-or-vasculitis-with-nephritis
#4
Sigrid Lundberg, Emelie Westergren, Jessica Smolander, Annette Bruchfeld
BACKGROUND: Approximately 30% of adult patients with immunoglobulin A (IgA) nephropathy (IgAN) or IgA vasculitis with nephritis (IgAVN) develop end-stage renal disease during long-term follow-up. In particular, patients with nephritic-nephrotic syndrome have an increased risk of rapid progression. Conventional immunosuppressive therapy with corticosteroids (CSs) may be insufficient for disease control and is associated with a number of side effects. Rituximab (RTX) has been shown to be well tolerated and effective in a range of glomerular diseases, but there is little information on its therapeutic potential in IgAN...
February 2017: Clinical Kidney Journal
https://www.readbyqxmd.com/read/28638601/the-efficacy-of-rituximab-in-adult-frequently-relapsing-minimal-change-disease
#5
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/28637442/membranous-nephropathy-a-retrospective-observational-study-of-membranous-nephropathy-in-north-east-and-central-london
#6
Sanjana Gupta, John Connolly, Ruth J Pepper, Stephen B Walsh, Magdi M Yaqoob, Robert Kleta, Neil Ashman
BACKGROUND: Membranous nephropathy (MN) is the leading cause of nephrotic syndrome in adults. MN is a clinically heterogeneous disease and it is difficult to accurately predict outcomes (including end stage renal failure) at presentation and whom to treat with potentially toxic therapies. We aimed to identify factors predicting outcome in MN in our cohort from two large tertiary London units by undertaking a retrospective data analysis of 148 biopsy-proven MN patients from North East and Central London between 1995 and 2015...
June 21, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28634522/popliteal-arterial-thrombosis-in-nephrotic-syndrome-a-case-report
#7
Niranjan Tachamo, Rashmi Dhital, Bidhya Timilsina, Salik Nazir, Saroj Lohani, Dilli Ram Poudel
Thrombosis is a frequent cause of morbidity and mortality in patients with nephrotic syndrome (NS). Though venous thromboses are common in NS, arterial thromboses are relatively rare. Commonly involved arteries include coronary, iliac, femoral, renal, cerebral, pulmonary, mesenteric, and axillary arteries, and the aorta. Arterial thromboses are associated with poor prognosis; treatment options are limited and patients may not always be amenable to treatment. We present the case of a 39-year-old female with NS who presented with thigh pain and was found to have sub-acute popliteal artery thrombosis...
January 2017: Journal of Community Hospital Internal Medicine Perspectives
https://www.readbyqxmd.com/read/28631656/brevundimonas-diminuta-infection-in-a-case-of-nephrotic-syndrome
#8
Abhilash Chandra, Anupam Das, Manodeep Sen, Meenakshi Sharma
Brevundimonas has rarely been isolated from clinical specimens. We here report a case of Brevundimonas diminuta infection in an 18-year-old male patient with nephrotic syndrome. B. diminuta was identified by the VITEK 2 Compact system, following isolation from his blood sample. To the best of our knowledge, B. diminuta has not been reported in patients with nephrotic syndrome.
April 2017: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/28630858/pulsed-vincristine-therapy-in-steroid-resistant-nephrotic-syndrome
#9
Shenal Thalgahagoda, Shamali Abeyagunawardena, Heshan Jayaweera, Umeshi Ishanthika Karunadasa, Asiri Samantha Abeyagunawardena
Steroid-resistant nephrotic syndrome (SRNS) poses a therapeutic challenge for the paediatric nephrologist. As relentless progression to renal failure occurs with continued proteinuria, such patients will be treated with different cytotoxic medications with variable success rates and side-effects. We present here our findings on administering the anticancer drug vincristine for SRNS patients at a single centre in Sri Lanka. Methods. Between 2002 and 2007, fifty-four children presenting with steroid and cyclophosphamide resistance were treated with vincristine at 1...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28629718/changes-in-podocyte-trpc-channels-evoked-by-plasma-and-sera-from-patients-with-recurrent-fsgs-and-by-putative-glomerular-permeability-factors
#10
Eun Young Kim, Hila Roshanravan, Stuart E Dryer
Primary forms of focal and segmental glomeruloslerosis (FSGS) are driven by circulating factors that cause dysfunction or loss podocytes. Rare genetic forms of FSGS can be caused by mutations in TRPC6, which encodes a Ca(2+)-permeable cationic channel expressed in mesangial cells and podocytes; and NPHS2, which encodes podocin, a TRPC6-binding protein expressed in podocyte slit diaphragm domains. Here we observed that exposing immortalized mouse podocytes to serum or plasma from recurrent FSGS patients for 24h increased the steady-state cell-surface abundance of TRPC6, accompanied by an increase in currents through endogenous TRPC6 channels evoked by a hypoosmotic stretch stimulus...
June 16, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28628381/monoclonal-immunoglobulin-associated-proliferative-glomerulonephritis-characterized-by-organized-deposits-of-striated-ultra-substructures-a-case-report
#11
Shigeo Hara, Hiroyasu Tsukaguchi, Tastufumi Oka, Makiko Kusabe, Masayuki Mizui, Kensuke Joh
We herein report the case of a 64-year-old male who presented with progressive glomerulonephritis notable for organized and striated ultra-substructures. The patient was diagnosed with hypertension and proteinuria 3 years prior to admission and subsequently developed nephrotic syndrome and impairment of renal function. Laboratory tests did not reveal any evidence of infections or autoimmune diseases. Monoclonal gammopathy was not detected in serum or urine, although a small population of abnormal plasma cell clones was detected by flow cytometry...
June 19, 2017: Ultrastructural Pathology
https://www.readbyqxmd.com/read/28626447/autoimmune-thyroiditis-and-glomerulopathies
#12
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/28626181/long-term-low-density-lipoprotein-apheresis-in-a-patient-with-refractory-idiopathic-membranous-glomerulonephritis
#13
Junko Yabuuchi, Tatsuya Suwabe, Hiroki Mizuno, Toshiharu Ueno, Junichi Hoshino, Akinari Sekine, Masahiro Kawada, Masayuki Yamanouchi, Noriko Hayami, Rikako Hiramatsu, Eiko Hasegawa, Naoki Sawa, Kenmei Takaichi, Takeshi Fujii, Kenichi Ohashi, Yoshifumi Ubara
A 61-year-old Japanese man developed nephrotic syndrome (NS) due to idiopathic membranous glomerulonephritis (MGN). He received immunosuppressive therapy for two years, including prednisolone, cyclophosphamide, and cyclosporine A, but the NS persisted. Low-density lipoprotein apheresis (LDL-A) was initiated at a frequency of twice a month and continued for 9 years (203 sessions in total). His proteinuria reduced to less than 1 g daily after 9 years. LDL-A was stopped, and the NS has not relapsed for five years...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28617070/oral-subchronic-exposure-to-silver-nanoparticles-causes-renal-damage-through-apoptotic-impairment-and-necrotic-cell-death
#14
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
#15
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/28614268/pulmonary-and-cutaneous-infection-caused-by-nocardia-farcinica-in-a-patient-with-nephrotic-syndrome-a-case-report
#16
Ning Zhu, Yuan Zhu, Yi Wang, Shaoshao Dong
RATIONALE: Nocardia species is known as conditional pathogenic bacteria. Disseminated infection caused by Nocardia species is rare and occurs primarily in immunosuppressed patients. Signs and symptoms of this infection are frequently nonspecific making early diagnosis and treatment difficult. PATIENT CONCERNS: We report a case of subcutaneous and pulmonary nocardiosis due to Nocardia farcinica (N farcinica) in a patient with nephrotic syndrome who is undergoing long-term corticosteroid therapy...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28614261/a-prisma-compliant-meta-analysis-of-mdr1-polymorphisms-and-idiopathic-nephrotic-syndrome-susceptibility-and-steroid-responsiveness
#17
Shi-Sheng Han, Yan-Qiu Xu, Yan Lu, Xiang-Chen Gu, Yi Wang
BACKGROUND: Studies have investigated rs1128503, rs1045642, and rs2032582 in multidrug resistance protein 1 (MDR1) for association with susceptibility to idiopathic nephrotic syndrome (INS) and steroid resistance. However, because these findings were inconsistent, we performed a meta-analysis to determine whether there was evidence of a role of these MDR1 variants in INS. METHODS: The PubMed, Embase, and Web of Science databases were systematically searched to identify studies that examined MDR1 polymorphisms with susceptibility to INS and/or to steroid resistance...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28614243/different-types-of-glomerulonephritis-associated-with-the-dysregulation-of-the-complement-alternative-pathway-in-2-brothers-a-case-report
#18
Pei Chen, Li Zhu, Feng Yu, Sha-Sha Han, Si-Jun Meng, Wei-Yi Guo, Hong Zhang, Yan Song
RATIONALE: C3 glomerulonephritis (C3GN) and complement-mediated hemolytic uremic syndrome (HUS) both result from the abnormal regulation of the complement system. A significant number of patients with C3GN or complement-mediated HUS have mutations of more than 1 complement protein. This discovery has had a major impact on identifying the underlying cause of familial C3GN or complement-mediated HUS. PATIENT CONCERNS: We report the cases of 2 brothers (herein referred to as patient II-1 and patient II-9), both with complement disorders that differed in their clinical and genetic features...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28613279/nox-mediated-impairment-of-pdgf-induced-dna-synthesis-in-peripheral-blood-lymphocytes-of-children-with-idiopathic-nephrotic-syndrome
#19
Amal Al-Eisa, Gursev S Dhaunsi
BackgroundCellular oxidative stress, inflammatory responses, and immunogenic events are involved in pathogenesis of idiopathic nephrotic syndrome (INS); however, the exact mechanism remains unknown. We examined NADPH oxidase (NOX) activity and platelet-derived growth factor (PDGF)-induced DNA synthesis in peripheral blood lymphocytes (PBL) of patients with INS.MethodsPBL from 15 patients with INS and 15 age- and gender-matched controls were isolated, and enzyme activities of NOX, catalase, and superoxide dismutase (SOD) were measured along with the assay of malondialdehyde levels and bromo-deoxyuridine incorporation...
June 14, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28611949/pseudomalabsorption-of-levothyroxine-a-challenge-for-the-endocrinologist-in-the-treatment-of-hypothyroidism
#20
Nancy Van Wilder, Bert Bravenboer, Sarah Herremans, Nathalie Vanderbruggen, Brigitte Velkeniers
BACKGROUND: Hypothyroidism due to non-compliance with levothyroxine therapy (pseudomalabsorption) is rare. The diagnosis is considered in patients with persistent severe hypothyroidism despite treatment with large doses of levothyroxine. Intestinal malabsorption, drug and dietary interference with levothyroxine absorption and nephrotic syndrome should be excluded. The diagnosis of pseudomalabsorption can be demonstrated by using "an oral 1,000 µg of levothyroxine test" showing a rapid decrease in thyroid-stimulating hormone and increase in thyroxine...
February 2017: European Thyroid Journal
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