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https://www.readbyqxmd.com/read/29429752/patterns-of-clinical-response-to-eculizumab-in-patients-with-c3-glomerulopathy
#1
Moglie Le Quintrec, Anne-Laure Lapeyraque, Arnaud Lionet, Anne-Laure Sellier-Leclerc, Yahsou Delmas, Véronique Baudouin, Eric Daugas, Stéphane Decramer, Leila Tricot, Mathilde Cailliez, Philippe Dubot, Aude Servais, Catherine Mourey-Epron, Franck Pourcine, Chantal Loirat, Véronique Frémeaux-Bacchi, Fadi Fakhouri
BACKGROUND: Cases reports and small series of patients with C3 glomerulopathy have reported variable efficacy of eculizumab. STUDY DESIGN: Case series of C3 glomerulopathy. SETTING & PARTICIPANTS: Pediatric and adult patients with C3 glomerulopathy treated with eculizumab between 2010 and 2016 were identified through the C3 glomerulopathy French registry database, and a questionnaire was sent to participating French pediatric and adult nephrology centers, as well as one pediatric referral center in Québec, Canada...
February 8, 2018: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/29385997/proteinuric-kidney-disease-in-children-at-queen-elizabeth-central-hospital-malawi
#2
Zondiwe Victor Mwanza, Mignon McCulloch, Mark Drayson, Timothy Plant, David V Milford, Gavin Dreyer
BACKGROUND: There is a paucity of data on paediatric kidney disease in developing countries such as Malawi. Descriptive research on kidney disease is essential to improving patient outcomes. METHODS: We conducted a cross-sectional study at a tertiary hospital in Malawi from 2012 to 2013. Children under 14 years with proteinuric kidney disease were enrolled from paediatric wards and outpatient clinics at Queen Elizabeth Central Hospital (QECH). Demographic, clinical and laboratory data were collected from patients at enrolment and at 3 months review at which point clinical status and disease outcome were ascertained...
January 31, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29325951/shunt-nephritis-an-increasingly-unfamiliar-diagnosis
#3
Tessa A Harland, Ken R Winston, Anna J Jovanovich, Richard J Johnson
BACKGROUND: Shunt nephritis is a rare, reversible immune-complex mediated complication of CSF shunt infection that can progress to end-stage renal disease and even death if diagnosis is delayed. CASE DESCRIPTION: The present case report details the manifestation and clinical course of shunt nephritis in a 50-year old patient who presented with symptoms of nephrotic syndrome 30 years after ventriculo-jugular shunt placement. Diagnosis was delayed due to initial negative CSF and blood cultures but later a CSF culture was positive for Propionibacterium acnes...
January 8, 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29325287/-the-diagnostic-value-and-limits-of-diagnostic-parameters-for-wilson-s-disease
#4
X Yang
Wilson disease (WD) is a rare and treatable genetic disorder. This paper describes the new advances and author's long-term experiences in the diagnosis of WD. The characteristics in clinical and routine tests are: the age of presentation can be quite broad, the WD could not be excluded based on age only; the patients usually have mild digestive symptoms but obvious chronic liver disease signs; liver function tests may reveal normal or a mild elevation in bilirubin, ALT and AST, but quite abnormal in serum albumin and prothrombin time in most patients; Coombs-negative hemolytic anemia, normal or markedly subnormal serum alkaline phosphatase (typically < 40 IU/L) are useful for the diagnosis of fulminant WD...
December 20, 2017: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/29320993/distribution-of-glomerular-diseases-in-taiwan-preliminary-report-of-national-renal-biopsy-registry-publication-on-behalf-of-taiwan-society-of-nephrology
#5
Hsien-Fu Chiu, Hung-Chun Chen, Kuo-Cheng Lu, Kuo-Hsiung Shu
BACKGROUND: Despite the development of biomarkers and noninvasive imaging tools, biopsy remains the only method for correctly diagnosing patients with unexplained hematuria, proteinuria and renal failure. Renal biopsy has been performed for several decades in Taiwan; however, a national data registry is still lacking until 2013. METHODS: The Renal Biopsy Registry Committee was established within the Taiwan Society of Nephrology in January 2013. A biopsy registry format, including basic demographic data, baseline clinical features, laboratory data, and clinical and pathological diagnosis was developed...
January 10, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29301131/safety-of-outpatient-kidney-biopsies
#6
Bojana Šimunov, Mihaela Gunjača, Branislav Čingel, Dinko Škegro, Mladen Knotek
BACKGROUND: Kidney biopsy is frequently performed in our centre as an outpatient procedure. The aim of this study was to evaluate the safety of biopsy in the outpatient setting. METHODS: We analysed kidney biopsies performed from March 2013 to February 2017. Seven hundred twenty-five biopsies performed in the outpatient setting were identified: There were 592 transplant and 133 native biopsies including 3 solitary kidney biopsies. All were performed under ultrasound guidance using a 16G or 18G needle, with freehand technique...
January 4, 2018: Nephron
https://www.readbyqxmd.com/read/29274588/%C3%AE-asarone-reduce-proteinuria-by-restoring-antioxidant-enzymes-activities-and-regulating-necrosis-factor-%C3%AE%C2%BAb-signaling-pathway-in-doxorubicin-induced-nephrotic-syndrome
#7
Brijesh Sutariya, Madhusudan Saraf
α-asarone is natural bioactive compound that has been reported to have many benefits and medicinal properties. The present study aimed to assess the protective effect of α-asarone against doxorubicin (DOX) induced nephrotic syndrome in rats. An experimental nephrotic syndrome was induced by single intravenous injection of DOX (7 mg/kg) in rats. Animals were orally administered α-asarone (10 and 20 mg kg-1 d-1) for 4 weeks. Blood, urine and kidney tissues were collected for analyses at the end of the study...
December 20, 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/29098818/-advanced-progress-of-main-pharmacology-activities-of-triptolide
#8
REVIEW
Jin Cui, Xiao Chen, Jia-Can Su
Triptolide is an active component in traditional Chinese medicine Tripterygium wilfordii. Currently, triptolide has been used to treat various diseases, including lupus, cancer, rheumatoid arthritis and nephritic syndrome. Its main pharmacology efficacies include anti-inflammation, anti-tumor, and immunity suppression. Recent studies have also demonstrated triptolide's protective effect on cardiovascular diseases and osteoporosis. This paper summarizes the pharmacological efficacy of triptolide based on the advance in studies of triptolide...
July 2017: Zhongguo Zhong Yao za Zhi, Zhongguo Zhongyao Zazhi, China Journal of Chinese Materia Medica
https://www.readbyqxmd.com/read/29045960/pharmacokinetics-of-once-daily-prolonged-release-formulation-of-tacrolimus-in-children-with-primary-nephrotic-syndrome
#9
Y Han, S Q DU, H J Xiao, Y Zhou, J Ding, J J Ding, Y M Cui
OBJECTIVE: Tacrolimus prolonged-release(PR) formulation is a new once-daily formulation of the calcineurin inhibitor tacrolimus, which is currently used in adult liver or kidney transplant patients,and is also gradually widely used in children with nephrotic syndrome.The present study was undertaken to preliminarily investigate the pharmacokinetic characteristics of tacrolimus PR in pediatric nephrotic syndrome recipients. METHODS: This single-center open-label prospective study was performed in pediatric nephrotic syndrome recipients...
October 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/28990057/id3-may-protect-mice-from-anti%C3%A2-gbm-glomerulonephritis-by-regulating-the-differentiation-of-th17-and-treg-cells
#10
Huan Zhou, Le Wang, Qing Xu, Qingquan Liu, Hui Liu, Wenhui Qiu, Tingyang Hu, Yongman Lv, Qian Zhang
Anti‑glomerular basement membrane glomerulonephritis (anti‑GBM GN) is an autoimmune disease that leads to severe and rapidly progressive renal injury. Inhibition of DNA‑binding factor 3 (ID3) serves a key role in autoimmune diseases, such as asthma and Sjögren's syndrome, and in experimental allergic encephalitis models. However, the role of ID3 in the progression of anti‑GBM GN remains unknown. In the present study, ID3 mRNA expression increased between 3‑ and 20‑fold in the renal tissues of anti‑GBM GN mice compared with the Control group, with a peak at day 14 post‑induction...
December 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28950764/unusual-presentation-of-secondary-syphilis-membranoproliferative-glomerulonephritis-andmuco-cutaneous-lesions
#11
Linda Tognetti, Elisa Cinotti, Sergio Tripodi, Guido Garosi, Pietro Rubegni
The worldwide re-emergence of secondary syphilis which happened in the last decade, has led to an increase in primary and secondary syphilis cases, along with the presentation of atypical forms. Nevertheless, reports of renal syphilis with mucosal and/or cutaneous manifestations are nowadays increasing. Typically, secondary syphilis infection in adults causes nephrotic syndrome due to a membranous glomerulonephritis. Here, we report a case of a 30-year-old immunocompetent man presenting with skin rash, oral and perianal erosions and nephritic syndrome...
January 1, 2017: International Journal of STD & AIDS
https://www.readbyqxmd.com/read/28929695/-effects-of-mongolia-astragali-radix-in-protecting-early-cardiac-and-nephritic-functions-of-patients-of-hypertension-with-metabolic-syndrome
#12
Ning-Yin Li, Xiu-Li Li, Xiao-Ping Zhai, Qiong-Ying Wang, Xiao-Wei Zhang, Feng Zhao, Xiao-Feng Wang, Jian-Yun Fan, Feng Bai, Jing Yu
To explore the effect of Mongolia Astragali Radix produced in Longxi of Gansu province in protecting cardiac and nephritic functions of patients of essential hypertension(EH) with metabolic syndrome(MetS). A total of two hundred and twenty-six EH patients with MetS aged above 18 were selected. Patients were randomly divided to control group(adopted conventional medical treatment), Astragali Radix group 1(added Astragali Radix capsules 10 g•d⁻¹ besides conventional medical treatment) and Astragali Radix group 2(added Astragali Radix capsules 5 g•d⁻¹ besides conventional medical treatment)...
November 2016: Zhongguo Zhong Yao za Zhi, Zhongguo Zhongyao Zazhi, China Journal of Chinese Materia Medica
https://www.readbyqxmd.com/read/28918421/macrophage-activation-syndrome-at-the-onset-of-glucocorticoid-resistant-systemic-lupus-erythematosus-a-case-report
#13
Delia Tulbă, Marius Balea, Cristian Băicuș
INTRODUCTION: Macrophage activation syndrome (MAS) is a life-threatening hyperinflammatory state mediated by uncontrolled cytokine storm and haemophagocytosis. Although rarely reported, MAS might occur in systemic lupus erythematosus (SLE), notably as an inaugural manifestation. Glucocorticoids (GCs) are the cornerstone of SLE therapy. However, in some cases high doses of GCs are required to achieve remission (i.e. glucocorticoid-resistance), leading to significant side effects. CASE REPORT: A 28-year-old Romani male was admitted to our hospital for polyarthralgia, polyserositis and fatigability...
September 16, 2017: Romanian Journal of Internal Medicine, Revue Roumaine de Médecine Interne
https://www.readbyqxmd.com/read/28904427/micrornas-involvement-in-renal-pathophysiology-a-bird-s-eye-view
#14
REVIEW
P Jaswani, S Prakash, A Dhar, R K Sharma, N Prasad, S Agrawal
MicroRNAs (miRNAs) are known to suppress gene expression by binding to messenger RNAs and in turn regulate different pathophysiological processes. Transforming growth factor-β, mitogen-activated protein kinase signaling, and Wnt signaling-like major pathways associated with miRNAs are involved with kidney diseases. The discovery of miRNAs has provided new insights into kidney pathologies and may provide effective therapeutic strategies. Research has demonstrated the role of miRNAs in a variety of kidney diseases including diabetic nephropathy, lupus nephritis, hypertension, nephritic syndrome, acute kidney injury, renal cell carcinoma, and renal fibrosis...
September 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/28901387/triptolide-blocks-the-stat3-signaling-pathway-through-induction-of-protein-tyrosine-phosphatase-shp-1-in-multiple-myeloma-cells
#15
Ji-Hun Kim, Byoungduck Park
Triptolide, an active component extracted from the medicinal plant Tripterygium wilfordii Hook F., has been used to treat various diseases, including lupus, cancer, rheumatoid arthritis and nephritic syndrome. The present study investigated the effects of triptolide on multiple myeloma using western blotting and an electrophoretic mobility shift assay. Triptolide was found to suppress the inducible and constitutive activation of signal transducer and activator of transcription 3 (STAT3), which is closely associated with inflammation and tumorigenesis...
November 2017: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/28885376/clinicopathological-features-of-diabetic-and-nondiabetic-renal-diseases-in-type-2-diabetic-patients-with-nephrotic-range-proteinuria
#16
Yu Ho Lee, Ki-Pyo Kim, Yang Gyun Kim, Ju-Young Moon, Su Woong Jung, Eunji Park, Jin Sug Kim, Kyung-Hwan Jeong, Tae Won Lee, Chun-Gyoo Ihm, Young-Il Jo, Hoon-Young Choi, Hyeong-Cheon Park, So-Young Lee, Dong-Ho Yang, Joo-Hark Yi, Sang-Woong Han, Sang-Ho Lee
Heavy proteinuria with or without features of nephrotic syndrome is associated with many primary and systemic diseases. For diabetic patients, distinguishing nondiabetic renal disease (NDRD) from diabetic nephropathy (DN) is important in choosing treatment modalities and determining renal prognosis. However, clinical relevance of heavy proteinuria is inconsistent with clinical DN assessments. This study investigated the clinicopathological features and renal outcomes of DN and NDRD in type 2 diabetic patients with nephrotic-range proteinuria...
September 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28846194/spectrum-of-biopsy-proven-renal-disease-in-south-asian-children-two-decades-at-a-tropical-tertiary-care-centre
#17
Anjali Mohapatra, Shailesh Kakde, Vellaichamy M Annapandian, Anna T Valson, Neelaveni Duhli, Anila Korula, Smita Mary Matthai, Anna B Pulimood, Vinoi G David, Suceena Alexander, Shibu Jacob, Santosh Varughese, Basu Gopal, Tamilarasi Veerasamy, George T John
AIM: We report findings from a large single centre paediatric renal biopsy cohort in South Asia. METHODS: We analyzed all renal biopsies performed on children aged ≤18 years between 1996 and 2015 at our centre. The clinical characteristics and histological diagnosis pertaining to each case, distribution of renal diseases in children with various clinical presentations, and changes in the pattern of kidney disease during the study period were analyzed. RESULTS: A total of 1740 paediatric kidney biopsies were performed during the study period...
August 28, 2017: Nephrology
https://www.readbyqxmd.com/read/28838767/c4-nephritic-factors-in-c3-glomerulopathy-a-case-series
#18
Yuzhou Zhang, Nicole C Meyer, Fernando C Fervenza, Winnie Lau, Adam Keenan, Gabriel Cara-Fuentes, Dingwu Shao, Aalia Akber, Veronique Fremeaux-Bacchi, Sanjeev Sethi, Carla M Nester, Richard J H Smith
BACKGROUND: C3 glomerulopathy (C3G) defines a group of rare complement-mediated kidney diseases with a shared underlying pathophysiology: dysregulation of complement in the fluid phase and glomerular microenvironment. Dysregulation can be driven by autoantibodies to C3 and C5 convertases. STUDY DESIGN: Case series. SETTING & PARTICIPANTS: 168 patients with C3G (dense deposit disease, 68; C3 glumerulonephritis, 100) selected from our C3G biobank...
December 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/28717937/clinical-presentation-and-outcomes-of-childhood-onset-membranous-lupus-nephritis
#19
Maria Pereira, Eyal Muscal, Karen Eldin, M John Hicks, Anna Carmela P Sagcal-Gironella, Marietta DeGuzman, Scott E Wenderfer
BACKGROUND: Best practices for managing childhood-onset membranous lupus nephritis (MLN) are not yet established. Most studies involve primarily or exclusively adult cohorts or pediatric cohorts with combinations of pure or mixed membranous and proliferative nephritis. METHODS: We performed a single-center cohort study of consecutively diagnosed children with pure MLN from 1990 and 2016. Patients received care in Houston, Texas, one of the most diverse metropolitan areas in North America...
December 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28685717/variants-in-the-promoter-region-of-hla-dqa1-were-associated-with-idiopathic-membranous-nephropathy-in-a-chinese-han-population
#20
Xiao-Song Qin, Jian-Hua Liu, Guan-Ting Lyu, Meng-Le Peng, Fu-Ning Yang, Dong-Chun Qin, Yong-Zhe Li, Yong Liu
BACKGROUND: Idiopathic membranous nephropathy (IMN) is an autoimmune disease and the leading cause of adult nephritic syndrome. HLA-DQA1 had been identified to be associated with IMN in Europeans and the result was replicated in Chinese Han population. In this study, six single nucleotide polymorphisms (SNPs) in the promoter of HLA-DQA1 and other two SNPs with IgA nephropathy were included for the association analysis. METHODS: The SNPs were genotyped in 509 patients and 601 controls by the MassArray iPLEX...
July 20, 2017: Chinese Medical Journal
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